_id
stringlengths 7
16
| description
stringlengths 55
95.2k
|
|---|---|
pmc-6029167-4 | A 74-year-old man presented with right exophthalmos which suddenly developed 15 days ago. On examination, his best corrected visual acuities were 20/30 OD and 20/100 OS. Automatic refraction showed + 1.00 Dsph − 0.50 Dcyl x 180A OD and − 2.00 Dsph − 0.50 Dcyl x 180A OS. Slit lamp examination showed left posterior capsular opacity. He had orthotropia at distance and at near in the primary position with the alternate prism and cover test (Fig. ). Ductions and versions were full (Fig. ). Exophthalmometry showed 23 mm OD and 18.5 mm OS.
Orbit MR imaging showed an infiltrating mass involving both orbits, especially the lacrimal gland and both lateral rectus muscles, foramen rotundum, infraobital groove and foramen (Figs. ), trigeminal nerve, and midline anterior skullbase. Multiple enlarged lymph nodes were found in bilateral parotid glands, level I/II, and mediastinum with peribronchial infiltration in the right upper lung. Serum IgG4 level was 13.3 mg/dL. Right anterior orbitotomy and lacrimal gland biopsy showed lymphoplasmacytic infiltration with increased IgG4-positive cells (> 50–100 cells/HPF, IgG4/IgG ratio > 80%), consistent with IgG4-ROD.
Three years later, he presented with right facial edema (Fig. ), itching, right visual decrease, and vertical diplopia. On examination, his best corrected visual acuities were 20/50 OD and 20/100 OS. He had 10 Δ of XT and 35 Δ of RHT in the primary position, XT 4 Δ and RHT 25 Δ in right gaze, XT 10 Δ and RHT 20 Δ in left gaze, XT 10 Δ and RHT 35 Δ in upgaze, and XT 10 Δ and RHT 25 Δ in downgaze. With right or left head tilt, he showed XT 10 Δ and RHT 35 Δ. Ductions and versions showed − 3 limitation of downgaze, − 2 limitation of adduction, and − 1 limitation of abduction in the right eye (Fig. ). Serum IgG4 level was elevated to 788.0 mg/dL.
One month later after taking oral prednisolone 20 mg a day, diplopia resolved and he showed orthotropia at distance and at near in the primary position with alternate prism and cover test (Fig. ). Ductions and versions were full (Fig. ). Orbit MR imaging showed a decreased extent of infiltrative mass-forming lesions in both orbits, especially in both lacrimal glands. However, significant remnant mass lesions were found in the right inferior extraconal space involving the right medial, lateral, and inferior rectus muscles, left lateral, and inferior rectus muscles, with perineural spread along both foramen rotundum and infraorbital foramen. Three months later after taking oral prednisolone, serum IgG4 level decreased to 249.9 mg/dL. |
pmc-6029168-1 | Patient 5 was a 50 year old male, had pulmonary tuberculosis in the past with destroyed left lung, and this patient also had a bronchial artery embolization in the past with gel foam only, which was performed 4 years before. The patient was admitted with massive hemoptysis. A large bronchial feeder was identified in the left upper lobe, and embolization was performed with PVA particles and followed by gel foam. At the end of the procedure a stent was placed (endeavor 2.5 mm × 18 mm) at 8 atm, and final closure was performed with PVA particles followed by gel foam with good results (Additional file : Patient 5a Additional file : Patient 5b, Additional file : Patient 5c, Additional file : Patient 5d, Additional file : Patient 5e). The patient had severe chest pain and mild hemoptysis at the end of the procedure. The patient is being followed up for 3 months, and there are no episodes of hemoptysis. |
pmc-6029170-1 | A 76-year-old woman had an 8-year history of atrial fibrillation (AF) and severe TR. Her history included mild hypothyroidism and right upper lobectomy for lung carcinoma 11 years previously. For 2 years, she had complained of shortness of breath when lying in the left lateral decubitus position. She had felt dyspnea after mild exercise for 9 months. Recently, she had a sense of abdominal fullness. Although administration of diuretics was started, her symptoms did not completely improve, and she was referred to our department for surgical treatment. The follow-up chest X-ray showed a gradually protruding right-side shadow of the cardiac silhouette, and the cardiothoracic ratio on the chest X-ray reached 88% (Fig. ). The electrocardiogram showed AF with low fibrillatory wave amplitude. Echocardiography showed an enlarged right ventricular (RV) cavity and mild paradoxical motion of the ventricular septum. The tricuspid valve had no findings of an organic and constructive abnormality, with no severe tethering. The annular size of the tricuspid valve was 50 mm, and the tricuspid annular plane systolic excursion was 21 mm (Table ). Repeated preoperative cardiac catheterization showed slightly elevated wedge pressure with mild pulmonary hypertension, although LV function was preserved with a cardiac index of 4.0 (Table ). There was no L-R shunt disease. Computed tomography (CT) findings showed that the maximum size of the RA reached 121 mm (Fig. ). The change in dimension of the RA by CT showed that the size of the RA increased with time (Fig. ). Blood tests showed no liver dysfunction.
Surgery was performed via median sternotomy. The pericardium was extremely thin on the RA side without any defect. There was no adherence in the pericardial cavity. Cardiopulmonary bypass was established by ascending aorta cannulation with bicaval drainage. The RA was extremely thin and the tricuspid valve annulus was enlarged, with a diameter of 55 mm, but there was no tricuspid structural abnormality. Tricuspid annuloplasty was performed on the beating heart using a 28-mm Carpentier-Edwards Physio tricuspid annuloplasty ring (Edwards Lifesciences, Irvine, CA, USA). Plication of the enlarged RA was performed, mainly at the interatrial septum, the free RA wall, including the appendage, and the space between the inferior vena cava and the tricuspid ring, in addition to the free RA wall, including the appendage (Fig. ). Additionally, closure of the left atrial appendage from the outside was performed to prevent left atrial thrombus formation. All procedures were performed on the beating heart. The postoperative course was uneventful. The pathological findings of the RA wall demonstrated thinning of the myocardium, inflammatory cell infiltrate, and few cardiomyocytes (Figs. , ). Postoperative X-ray and CT showed reduced cardiac silhouette and RA volume (Figs. , ). Postoperative echocardiography showed an ejection fraction of 58% and mild TR with a pressure gradient of 29 mmHg (Table ). The postoperative value of tricuspid annular plane systolic excursion (TAPSE) decreased after the operation. However, the patient’s symptoms were completely resolved, and she was discharged 3 weeks after surgery. The patient is doing well 2 years after surgery. |
pmc-6029171-1 | The deceased donor was a 67-year-old man with a kidney Doppler ultrasound (DUS) that was negative for any nodular lesion. As part of the routine postoperative follow-up management, the recipient underwent DUS to assess the patency of the graft on postoperative day 1. The DUS finding was suspicious for an acute arterial thrombosis but did not reveal any focal irregularities. Consequently, a computed tomography (CT) scan was urgently obtained but it did not show any arterial complications. However, it serendipitously revealed a 2.4-cm lesion on the upper pole of the renal allograft which was not detected during the back-table or ultrasonography monitoring. A biopsy of the lesion was performed, and its histology revealed an epithelial proliferation of large cells with finely granular cytoplasm and medium round nucleus vesicular acidophilus, arranged tubules, and alveoli and cords immersed in a connective tissue stroma. This picture was consistent with oncocytoma. However, because the eosinophilic variant of chromophobe renal cell carcinoma (RCC) may morphologically resemble renal oncocytoma, immunohistochemical staining was performed using Ki-67 antibodies and RCC antigens. The results were negative, ruling out chromophobe RCC. The therapeutic options and potential related outcomes were clearly discussed with the patient. Given the low risk of malignant transformation in an oncocytoma [], we found no reason for resection of the lesion or an allograft nephrectomy. Consequently, we opted for active surveillance of the benign tumor with ultrasonography, every 2 months, for the first year and, then, with magnetic resonance imaging (MRI), every year (Fig. ). The patient received mycophenolate-mofetil, tacrolimus, and prednisone throughout the 5-year follow-up period and the regimen for immunosuppression was not changed despite the presence of the renal mass. After 60 months of active surveillance, we report that radiological studies have shown no growth, regression, or any other interim morphological changes to the lesion, and the patient is alive and well (Fig. ). |
pmc-6029341-1 | The 40-year-old male patient was enrolled in a study of anti-PD-1 therapy for Hodgkin lymphoma (HL) after a 3-year history of classical HL that was refractory to classical chemotherapeutic agents. The patient began intravenous administration of an anti-PD-1 antibody (SHR-1210, 200 mg) every 2 weeks. Urine protein was negative prior to the initiation of treatment. After the third dose of the anti-PD1 antibody (30 days from initial treatment), the patient developed massive proteinuria (5.47 g/day) with normal serum albumin and creatinine levels (35.3 g/L and 68 μmol/L, respectively). The treatment was suspended and proteinuria was monitored regularly. His urine protein excretion decreased to 0.47 g/day and further to 0.1 g/day on days 30 and 37, respectively, following the final dose of anti-PD1 antibody. However, 2 weeks later, urine protein excretion increased to 3.21 g/day, and to 30 g/day following an additional 14 days. The patient denied receiving administration of any additional drugs during this period and was admitted for further evaluation. Upon admission, his blood pressure was 110/75 mmHg with moderate pitting edema of both lower limbs. Laboratory tests revealed hypoalbuminemia (21 g/L), normal serum creatinine (80 μmol/L), and elevated total serum cholesterol (6.58 mmol/L). A positron emission tomography/computed tomography scan showed complete metabolic remission of HL (Fig. ).
A kidney biopsy was performed. Upon light microscopy, there were 20 glomeruli with no obvious changes. The tubulointerstitium and small arterioles showed no remarkable changes. Immunofluorescence showed the specimen was negative for immunoglobulin G, M, and A, C3, C1q, and κ and λ light chains. Electron microscopy demonstrated diffuse podocyte foot process effacement. The final diagnosis was MCD (Fig. ). We further screened the secondary causes of MCD. A panel of viral antibodies including hepatitis B virus, hepatitis C virus, human immunodeficiency virus were screened and showed no significant positive results.
The patient was prescribed prednisone (1 mg/kg/day). Proteinuria improved within 2 weeks (protein excretion decreased to 1.7 g/day, and serum albumin increased to 31.3 g/L). One month following the initiation of prednisone, proteinuria was fully remitted with serum albumin of 37 g/L. Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers were not used. Prednisone was tapered after 8 weeks. Figure shows the changes in serum albumin and 24-h urine protein excretion over the course of treatment. |
pmc-6029345-1 | A 75-year-old Caucasian man was admitted for bicytopenia in May 2013. His blood cell count was as follows: hemoglobin 8 g/dl, platelets 87 × 109/l, and leukocytes 6.1 × 109/L (Fig. ). His medical history included T2D treated with biphasic insulin aspart, 50 units at breakfast and 30 units at bedtime, and the dose of rapid-acting insulin was adjusted according to his blood glucose level at lunch. He was also followed for triple-bypass surgery for coronary disease in 2010. No family history of hematological malignancies was noted. Initially, he was hospitalized in an intensive care unit for grade IV anemia at 5.5 g/dl. After blood transfusions, he was admitted in our hematology department. Bone marrow aspiration showed multilineage dysplasia with 8% of blasts (Fig. ), consistent with a diagnosis of MDS with excess blasts: refractory anemia with excess blasts-1 (RAEB-1). A karyotype analysis identified trisomy 13. Immunophenotypic evaluation showed the presence of CD34+, CD117+, cytoplasmic myeloperoxidase (CMPO+), and CD33− cells. Plasma folate, vitamin B12, iron, and thyroid stimulating hormone levels were normal. No inflammatory syndrome was found. He was initially treated with darbepoetin 300 μg per week, without response.
Two months later, because of a worsening of cytopenia, a second bone marrow aspiration was performed. It showed 29% of blasts, consistent with the diagnosis of AML progression (Fig. ). He consented to participate in a phase I–II clinical research study assessing the combination of 5-azacitidine with idarubicin in July 2013. He showed a partial response with 8% of bone marrow blasts after six cycles, and transfusion independence. After four cycles of 5-azacitidine, he reported higher blood glucose levels 2–3 days after the start of each 5-azacitidine cycle, requiring an increased dose of self-administered insulin. No change in diet or in physical activities or other causes of glycemic dysregulation such as corticosteroids were noted. Further monitoring confirmed higher blood glucose levels from days 4 to 12 of each 5-azacitidine cycle (Fig. ). Our patient experienced such dysregulation during all the additional 15 cycles until a fatal progression of AML. |
pmc-6029347-1 | A previously healthy full term 15 day old Caucasian male with an unremarkable antenatal, perinatal, and family history presented on Day 2 of illness with a 24 h history of poor feeding, irritability, fever, and rash. Examination revealed fever (39.6 C), tachycardia (HR 180–210), tachypnea (RR 68), extreme irritability, acrocyanosis, and a generalized maculopapular rash, but otherwise was normal. Admission blood work revealed a normal complete blood count, slightly elevated serum transaminase levels, mild hypoalbuminemia (30 g/L), and a normal c-reactive protein (CRP) of 2.6 mg/L. Empiric treatment with intravenous ampicillin, cefotaxime, and acyclovir was given for presumed neonatal sepsis and possible encephalitis. On day 4 of illness, in addition to ongoing fever, he developed recurrent apnea and required supplemental oxygen and transfer to the tertiary care hospital pediatric intensive care unit. His blood work showed mild neutropenia, elevation of serum transaminase levels (alanine transaminase 86 U/L, aspartate transaminase 220 U/L), a normal ESR (1 mm/hr) and hypoalbuminemia. An infectious etiology was considered unlikely given negative bacterial cultures from blood, cerebral spinal fluid, and urine, and negative viral studies for herpes simplex virus 1 and 2, Respiratory Syncytial Virus, Influenza A and B, cytomegalovirus, Epstein Barr virus, rubella, adenovirus, and rotavirus. The urine and blood cultures were collected before the start of antibiotics. His chest radiograph was normal. On day 6 of illness, he developed bilateral non-purulent conjunctivitis, palmar erythema, striking bilateral non-pitting edema and erythema of his feet, and erythema and swelling of several proximal interphalangeal joints (Fig. ). He continued to require supplemental oxygen for suspected pneumonitis. On Day 7 of illness, after 5 days of fever and meeting 3 out of 5 criteria for KD, with no other obvious diagnosis, he was given IVIG (2 g/kg) for suspected incomplete KD. Over the next 48 h, his symptoms including extremity edema resolved, he no longer required supplemental oxygen, and fever did not recur. His CRP remained normal throughout his illness. Laboratory values improved and his platelet count progressively increased from 246,000 on Day 7 to 852,000 on Day 13 of illness. In the subacute phase, he was treated with low dose aspirin. He had persistent thrombocytosis followed by typical extremity desquamation. Echocardiograms on weeks 1, 2, and 6, and at 12 months were normal with no carditis or coronary artery abnormalities. |
pmc-6029359-1 | A 52-year-old male presented with severe headache and elevated blood pressure (180/120 mmHg). Color Doppler echocardiography revealed a large, solid right intraventricular mass of 7.1 × 2.2 × 4.7 cm, originating from the middle-inferior ventricular septum and extending into the right atrium, with systo-diastolic fluttering (Fig. ). A cardiac magnetic resonance imaging (MRI) study confirmed the presence of the solid, intracavitary mass.
A cardiac biopsy via catheterization was not conclusive regarding the precise nature of the lesion. After informed consent, the patient underwent surgery. Intraoperatively, a whitish mass, 3 × 4 cm, non-adherent to the wall, was found in the right atrium after median sternotomy and atriotomy (Fig. ). Through the tricuspid valve, the mass extended without discontinuity into the right ventricle, adhering to the septal surface of the leaflet and infiltrating the adjacent interventricular septum, in its medial and superior portion. The exophytic intraatrial and intraventricular components were removed. Since intraoperative frozen-section on tissue from the infiltrated septum confirmed the clinical suspect of malignancy, no further surgery was attempted. Definitive histological examination showed a malignant neoplasm characterized by epithelial cells, positive for cytokeratin, forming glandular structures, admixed with a spindle cell component (Fig. -). A final diagnosis of biphasic synovial sarcoma was also confirmed by molecular demonstration of an SYT-SSX1 transcript.
Computed tomography scan (CT) and cardiac MRI, performed one month after surgery, prompted administration of chemotherapy (ifosfamide/mesna, 4 cycles/3 months) due to increase of the residual ventricular tumor. However, since at the end of this treatment a (18) F-fluorodeoxyglucose positron emission tomography/CT revealed an increased ventricular mass, a SynCardia total artificial heart was implanted in the patient. Unfortunately, waiting for transplantation, the patient developed multiple pulmonary metastases and rapidly died, one and half year after initial surgery. |
pmc-6029359-2 | A 39-year-old man presented with signs of cardiac tamponade. The echocardiography revealed abundant pericardial effusion with a large intrapericardial mass. The patient underwent surgery after informed consent. At surgery, a pericardial tumor (9.8 × 6.1 × 3.2 cm) adherent to the anterior wall of the aorta, superior vena cava and right atrium, was extensively removed. No deep infiltration of the myocardium was apparent and aortic reconstruction was unnecessary. Since intraoperative histological examination did not reach a conclusive diagnosis, further surgery was not performed. Definitive histology revealed a monophasic (spindle cell) synovial sarcoma carrying an SYT-SSX2 transcript.
Postoperatively, echocardiography, cardiac MRI and CT did not show residual tumor. Owing to a diagnosis of synovial sarcoma, the patient underwent chemotherapy (epirubicin/ifosfamide, 6 cycles/4 months), remaining in healthy conditions for the following 8 months, before the onset of tachycardia and dyspnea. Echocardiography, cardiac MRI and CT revealed a recurrent pericardial tumor extensively involving both atria and left pulmonary veins. Because further surgery was considered unfeasible, an additional course of chemotherapy was started (cisplatinum/docexatel, 6 cycles/4 months), followed by intensity-modulated radiotherapy (DT 54 Gy/25 sessions/2 months). After a temporary improvement of the clinical conditions, CT documented an extensive regrowth of the tumor, with involvement of the heart, great vessels, left bronchus and esophagus, with mediastinal lymphadenopathy. The patient died 32 months after surgery. |
pmc-6029361-1 | A 32-year old white man presented to our department with a 1 month history of eye redness (left eye). He had already been observed by a general practitioner who advised the patient to seek ophthalmological advice if the lesion would not resolve within 3 weeks. In the first ophthalmic evaluation, the patient presented with 2 ocular lesions. These were best observed in biomicroscopy. There was an enlarged violet-coloured mass on the right superior eyelid which had evolved over the course of 1 week (Fig. ). There was also a mobile bulbar conjunctival lesion with a bright red colour, approximately 5 mm × 5 mm, in the superior temporal quadrant of his left eye (Figs. and ). The patient did not mention any pain or visual changes. The lesions looked like a chalazion and a subconjunctival haemorrhage, respectively (Fig. ). Best corrected visual acuity was 20/20 in both eyes (Snellen chart) and subsequent fundoscopic exam was normal. Patient denied previous trauma history or drug abuse. Other systemic features included facial seborrheic dermatitis (Fig. ), a characteristic of HIV-AIDS. Past medical history was unremarkable with no serological evaluation for HIV. The main differential diagnosis at the time of presentation was blood dyscrasia, due to the duplicity of lesions. We also considered KS as part of an immunodeficiency syndrome unknown to the patient. Blood cell count and differential analysis were normal and coagulation disorders were excluded. However the HIV-1 test was found positive and the presumed diagnosis of ocular KS was established. The patient elected for an excisional biopsy of the conjunctival mass and for an incisional biopsy of the right superior eyelid. In addition, HIV-AIDS staging was performed. A CD4 cell count was determined to be 23/mm3 and the viral RNA load of 427,000/ml. Pathologic examination confirmed KS diagnosis of both lesions. Postoperative evaluations were uneventful and no signs of recurrence were noticed during the 6-month follow-up.
Further multiple disciplinary assessments showed a diffuse KS with skin and supraglottis involvement. The patient was offered treatment for both AIDS and diffused KS. In order to achieve disease control, patient initiated systemic antiretroviral therapy – HAART - and systemic chemotherapy under medical supervision with regression of the tumour. |
pmc-6029363-1 | A two-year-old Vietnamese female child had fever and cough 20 days before admission to our hospital. She visited a local hospital and was diagnosed with pneumonia. She was treated with meropenem and vancomycin for 15 days. Despite treatment, she developed increased fever (40 °C), persistent cough and general malaise. Her respiratory condition deteriorated and she was transferred to our hospital. Prior to hospitalization, she had been healthy and had developed normally. She had no immunodeficiency and no history of contact with anyone suffering from TB.
Physical examination on admission showed a child with a weight of 10 kg, body temperature of 38.5 °C, blood pressure of 79/41 mmHg, and a heart rate of 157 beats/min. Her weight was reduced from 11 kg (25th percentile) to 10 kg (10th percentile) over the past month alone. Immediately after admission, tracheal intubation was performed because of severe dyspnea, and she was transferred to a pediatric intensive care unit (PICU). Arterial blood gas analysis in the PICU showed PaO2 of 60 mmHg, PaCO2 of 59 mmHg, and pH 7.27 under mechanical ventilation with FiO2 of 1.0, which resulted in a PaO2/FiO2 (P/F) ratio of 60 mmHg and an oxygenation index (OI) of 29 (Table ). A chest X-ray showed bilateral infiltration without findings of heart failure (Fig. ). Laboratory findings were as follows: white blood cell (WBC) count, 7000 cells/μl; red blood cell (RBC) count, 430 × 103 cells/μl; platelet (PLT) count, 223 × 103 cells/μl; aspartate aminotransferase (AST), 60 U/L; alanine aminotransferase (ALT), 13 U/L; blood urea nitrogen (BUN), 18.0 mg/dL; creatinine (Cre), 32 μmol/L; c-reactive protein (CRP), 7.3 mg/dL; fibrinogen, 2.79 g/L; and prothrombin time (PT), 16.1 s (Table ). The results of a rapid influenza test were negative. Genomes of mycobacterium TB and cytomegalovirus (CMV) were detected in tracheal lavage fluid (TLF) by real-time polymerase chain reaction (rPCR) assay. Acid fast bacilli smear and culture were both positive. She was diagnosed as having severe ARDS that developed from TB pneumonia, and the standard anti-tuberculous drugs: rifampicin, 15 mg/kg/day; isoniazid, 10 mg/kg/day; pyrazinamide, 30 mg/kg/day; ethambutol, 20 mg/kg/day were administered. We also used colistin and levofloxacin for not only nosocomial infection, but also mycoplasma infection, which we could not rule out. However, her respiratory and circulatory conditions deteriorated despite high-frequency oscillating ventilation (HFO), vasopressor support (noradrenaline at 0.2 μg/kg/min), and administration of 1 g/kg of immunoglobulin. On the third day after admission, the patient’s International Society on Thrombosis and Hemostasis DIC score had increased to 5 (PLT, 153 × 103 cells/μl; fibrinogen, 2.11 g/L; D-dimer, 5659 ng/mL; and PT, 23.3 s). For the treatment of DIC, 380 U/kg of rTM was administered as an intravenous drip infusion for 6 consecutive days. After administration of rTM was completed, OI gradually decreased, and the mechanical ventilation mode was changed from HFO to synchronized intermittent mandatory ventilation (Fig. ). DIC score also gradually decreased. We measured plasma levels of soluble receptor for advanced glycan end products (sRAGE) and high mobility group box 1 (HMGB-1) before and after rTM administration, because increased levels of sRAGE and HMGB-1 are associated with death in patients with ARDS []. After administration of rTM, HMGB-1 and sRAGE decreased (Fig. ), and inflammatory biomarkers, including interferon-gamma (IFN-γ) and interleukin-6 (IL-6), also decreased (Fig. ). Throughout the administration of rTM, there was no bleeding-related and other adverse event. Although severe ARDS (P/F ratio ≦ 100 mmHg) was sustained for 19 days, the patient’s OI and P/F ratio improved gradually (Fig. ), and she was extubated on the 27th day after admission. Although there was still a diffuse pattern in the chest X-ray just after extubation (Fig. ), her fever went down and her respiratory condition was stable (P/F ratio: 325, OI: 3). Therefore, she was moved from the PICU to the infectious disease department. The severe ARDS with DIC was successfully treated, and she was discharged from hospital on the 33rd day after admission. |
pmc-6029377-1 | A 42-year-old Cameroonian (black African) man with no relevant past medical history presented to our hospital with a 1-week history of fever and a 4-day history of no bowel movements. The fever was intermittent and worse in the evenings, and associated with headache, joint pains, and anorexia. He self-medicated an undocumented orally administered antimalarial medication but symptoms persisted. Three days later, he developed generalized abdominal pain, colicky in character, with a severity of 5/10 on a visual analogue scale. This was associated with progressive abdominal distension and an altered bowel pattern initially characterized by an inability to pass stool and flatus over 4 days but he denied any vomiting. A worsening of his symptoms prompted his visit to our hospital.
On examination, his mucous membranes were dry, his conjunctivae were pink, and his vital signs were normal. His abdomen was mildly distended with mild diffuse tenderness. There was neither guarding nor rebound tenderness. Percussion note was tympanic and bowel sounds were hyperactive. His rectum was empty per digital rectal examination and his prostate was not enlarged. The rest of the physical examination was unremarkable.
A rapid diagnostic (Alere™ Malaria Ag P.f, Abbott Rapid Diagnostics, USA) test for malaria was positive. Also, his complete blood count values were within normal ranges: white blood cell count of 6200 cells/uL, hemoglobin of 12.5 g/dL, and platelet count of 222,000 cells/uL. His metabolic panel was within normal range: serum sodium ion (Na+) of 140 mmol/L, serum potassium ion (K+) of 4.0 mmol/L, serum chloride ion (Cl−) of 98 mmol/L, serum creatinine of 0.9 mg/dL, alanine aminotransferase was 41.2 U/L, aspartate aminotransferase was 33.0 U/L, total bilirubin was 0.81 mg/dL, and alkaline phosphatase was 55.9 U/L. His human immunodeficiency virus serology was non-reactive. His rapid antibody test for typhoid fever was also non-reactive. There was no significant air fluid level or increased bowel gas on a plain abdominal radiograph and an abdominal ultrasound revealed mild free intra-abdominal fluid in all four quadrants.
We concluded on a working diagnosis of functional bowel obstruction secondary to malaria. He received three doses of intravenously administered quinine base infusion (600 mg in 500 ml of dextrose 5% to flow for 4 hours every 8 hours for 24 hours) and intravenously administered crystalloids (0.9% normal saline at rate of 1 liter 8 hourly for 48 hours) and was placed on “nothing by mouth.” He passed a small quantity of feces 48 hours after initiation of antimalarial medication, with complete resolution of the abdominal pain, distension, and bowel movements on the fourth day of hospital stay. A stool examination was done with no organisms identified. We then concluded on a final diagnosis of sub-acute intestinal obstruction secondary to malaria. He was then discharged on an orally administered artemisinin-based antimalarial combination (artemether/lumefantrine 20/120 mg, 4 tablets 12 hourly for 3 days). His 2-week follow up was unremarkable. |
pmc-6029390-1 | A 20-year-old woman, who had an unremarkable medical history and was immunocompetent, was admitted to another hospital due to fever, fatigue, and anorexia, and she was administered acetaminophen and antibiotics. She also experienced vomiting, as well as systemic myalgia 5 days after admission causing an inability to move. Her condition was worsening, and she was transferred to our hospital 7 days after her initial admission. Upon admission, her liver enzyme and creatine phosphokinase (CPK or CK) levels were high. She had no history of jaundice, pruritus, clay stools, melena, hematemesis, abdominal distension, or altered sensorium. She reported only an occasional small amount of ethanol intake and had not had sexual intercourse. The patient denied intake of indigenous medicine or intoxication. The patient did not report any past major surgeries, blood transfusions, or intravenously injected drug abuse prior to onset of the disease. Additionally, she did not report any history of diabetes, hypertension, tuberculosis, thyroid disease, trauma, exposure to industrial toxins or radiation, blood or blood component therapy, bleeding disorders, promiscuity, or similar complaints in the family or neighborhood.
Upon admission, her vital signs were as follows: body temperature, 37.2 °C; blood pressure, 110/72 mmHg; pulse, 75 beats/min; respiratory rate, 20 breaths/min; and oxygen saturation, 98% on room air. A physical examination revealed mild enlargement of the liver, no pitting edema in both legs, and no coarse crackles over the lung fields. Laboratory findings are presented in Table .
Additionally, a chest radiography showed absence of pulmonary congestion, pleural effusion, and cardiomegaly. Electrocardiography was not performed on admission. Computed tomography of the chest (Fig. ) and abdomen revealed minimal pericardial effusion, diffuse swelling, and an internal heterogeneous image in the liver. These findings were compatible with acute hepatitis; therefore, we did not examine for cardiac function despite the presentation of pericardial effusion.
On the second day of hospitalization, we performed a liver biopsy, which showed parenchymal necrosis and lymphocytic infiltration (Fig. ). On that same night, the patient was hypotensive, with a blood pressure of 80/65 mmHg, and her heart rate was elevated (113 beats/min). Physical examination revealed cyanosis of the lips, distended external jugular veins, pretibial edema in both legs, and coarse crackles over the lower bilateral lung fields.
Electrocardiography showed diffuse ST-segment elevation (Fig. ). Her troponin I, CK level in the blood (CK-MB), and brain natriuretic peptide (BNP) were elevated (troponin T, 0.879 ng/mL [normal value: ≤0.016 ng/mL]; CK-MB, 253 U/L [normal value: ≤5 U/L]; BNP, 1513 pg/mL [normal value: ≤18.4 pg/mL]). Chest radiography showed a normal cardiac size, pulmonary congestion, and pleural effusion in the right lung only (Fig. ). Echocardiography showed a dilated, spherical ventricle with reduced systolic function (left ventricle ejection fraction [LVEF], 17%) and pericardial effusion (Fig. ).
Cardiac catheterization on day 3 revealed high pulmonary capillary wedge pressure (22 mmHg) and a low cardiac index (2.0 L/min/m2). Coronary angiography showed no abnormalities. Endomyocardial biopsy (EMB) was performed via the right internal jugular vein, and five specimens were obtained from the right ventricle side of the interventricular septum. Endomyocardial biopsy findings showed lymphocyte infiltration of the myocardium and intranuclear inclusions, which was confirmed as acute myocarditis (Fig. ). In response, intravenous administration of dobutamine (4 μg/kg/min) was started on day 3. The patient’s systolic blood pressure increased to approximately 100 mmHg and stabilized, and dobutamine administration was gradually tapered off. On day 10, echocardiography showed normalization of LVEF to 71%, dobutamine was stopped, and laboratory findings were almost normalized as follows: CPK, 143 U/L (standard values: 40–150 U/L); aspartate aminotransferase (AST), 36 U/L (standard values: 11–30 U/L); and alanine aminotransferase (ALT), 278 U/L (standard values: 4–30 U/L). Laboratory findings on day 18 were normalized as follows: CPK, 123 U/L; AST, 18 U/L; ALT, 21 U/L, and BNP, 18 pg/mL. She was eventually discharged on day 19 of her hospital stay.
One serum sample for viral serological testing was collected before the EMBs were obtained. Follow-up serum samples were collected between 2 weeks, 1 month, and 6 months after the initial serum sample. The presence of HSV-specific immunoglobulin M (IgM) was detected upon admission to our hospital, increased at 2 weeks, and returned to normal 6 months later. Additionally, HSV-specific immunoglobulin G (IgG) increased from hospital admission to 2 weeks (Table ). Other viral serologic tests, including HIV, were negative (Table ). Based on this finding, we diagnosed the patient with acute myocarditis combined with hepatitis arising from HSV infection. |
pmc-6029403-1 | An asymptomatic 70-year-old female known to have surgically treated squamous cell carcinoma of the nose underwent a routine screening colonoscopy that revealed an uncomplicated diverticulosis and a cecal submucosal mass (Fig. ). The appearance was most likely correlated with a submucosal tumor and less likely to be an extrinsic compression. At pathology, colonoscopic biopsies of the mass showed normal colonic mucosa. Laboratory examination showed no anemia (hb 13.2 g/dL) was negative for CEA tumor marker (CEA 2.2 μg/L). Abdominal computed tomography scan (CT scan) revealed a well-circumscribed hypervascular anterior cecal wall mass (Fig. ) with no liver metastases and no other distant lesions. The mass had no metabolic activity on either FDG-PET scan or on Octreo-PET (Fig. , ).
After multidisciplinary team discussion, a differential diagnosis of mesenchymal tumor of the colon (GIST, leiomyoma, and leiomyosarcoma) was suggested and we decided to perform an exploratory surgery. The patient was consented for open exploration by mini-laparotomy and possible right hemi-colectomy. The right colon was mobilized at the white line of Toldt, the 3 cm white cecal mass was well circumscribed, and a wedge resection, including the appendix, using GIA 75 (Ethicon Endo-Surgery GIA; 75 mm; Guaynabo, Puerto Rico 00969 USA) was performed. The operative specimen was sent for frozen section at pathology. The temporary diagnosis was a benign spindle cell tumor. The intra-operative decision was to wait for the definitive histopathologic examination report in order to try to avoid a right hemicolectomy. The final pathology report revealed a benign spindle cell tumor that stained negative for CD117 and DOG-1 and was diagnosed as cecal schwannoma with a reactive lymph node (Fig. ).
The post-operative course was uneventful and the patient started oral feeding the same night and was discharged on day 5 with pain killers. The final multidisciplinary committee decision was follow-up without further treatment needed. At 1-year follow-up, the patient is disease free. |
pmc-6029437-1 | A 39-year-old man fell and injured himself whilst walking on the road. Upon physical examination, he complained of severe pain in his right elbow, which showed swelling and tenderness. Because of the pain, the range of motion of the elbow joint was limited to 30°–50°. Radiographs revealed a fracture of the ulna coronoid process, which was displaced by about 4 mm (). He was diagnosed with a coronoid process fracture, Regan-Morrey type 2. He was young, highly active, and wanted an early return to normal life; therefore, we performed a minimally invasive arthroscopic osteosynthesis 7 days after the injury.
Surgery was performed under general anaesthesia with the patient in the prone position. We confirmed the fracture site through the anterolateral portal (). A shaver and radiofrequency probe were used to remove any clots and debris. The radiofrequency probe allowed us to reduce the amount of bone fragment entering the fracture bed. Under fluoroscopic control, a guide pin for a cannulated cancellous screw (CCS) was inserted from the posterior ulnar shaft into the coronoid fragment (). We fixed the fracture with a 3.5 mm CCS and two 1.5 mm K-wires, whilst confirming the anatomical reduction arthroscopically (). After fixation, we confirmed a good reduction position and sufficient stability upon examination. We also confirmed a reduction by radiographs (). Postoperatively, he wore an immobilizing splint for 1 week and then began physical therapy to increase the range of motion. After 3 weeks, the K-wires were removed, and bone union was achieved after 4 months (). At 1 year postoperatively, he had no pain and a physical examination revealed a complete range of motion. |
pmc-6029437-2 | A 29-year-old woman fell and injured herself whilst walking down the stairs. She was also diagnosed with a coronoid process fracture, Regan-Morrey type 2. We performed arthroscopic osteosynthesis 5 days after the injury in almost the same way as described in Case 1, using two 3.5 mm CCSs (). Postoperatively, she also had a good clinical course; she had no pain and had full range of motion of the elbow joint 1 year after surgery. |
pmc-6029437-3 | A 40-year-old man fell and injured himself whilst walking. He was also diagnosed with a coronoid process fracture, Regan-Morrey type 2 (). We performed arthroscopic osteosynthesis 10 days after the injury in almost the same way as described in Case 1. However, the fracture was too comminuted to be fixed by CCS, so we performed osteosynthesis using three K-wires (). Postoperatively, he wore an immobilizing splint for 2 weeks and then began physical therapy to improve the elbow range of motion. After 4 months, bone union was confirmed, and after 8 months, the K-wires were removed (). A follow-up at 10 months showed that he had no pain, and a physical examination revealed complete range of motion, indicating a good clinical course. |
pmc-6029438-1 | A 62-year-old man presented with 2 weeks of progressive dyspnea and chest pain. He was previously diagnosed with high-grade invasive urothelial carcinoma (UC) of the bladder. He underwent neoadjuvant chemotherapy followed by radical cystectomy, resulting in complete pathologic remission. On examination, patient was afebrile with a heart rate of 136 bpm, blood pressure of 122/74 mm Hg, respiratory rate of 18 breaths/min, and O2 saturation of 98% on room air. Heart sounds were muffled. Heart rhythm was irregularly irregular. Distention of the jugular veins was observed and Kussmaul's sign was present. Although pulsus paradoxus was not observed, other physical findings were extremely concerning for cardiac tamponade. Bibasilar crackles were present on lung auscultation. Laboratory data was remarkable for a creatinine of 1.42 (baseline of 1.08) and international normalized ratio (INR) of 3.6 (on warfarin for a history of pulmonary embolism). His complete blood counts and electrolytes were normal. His electrocardiogram revealed atrial fibrillation with a rapid ventricular rate (RVR) and electrical alternans. Chest radiograph showed enlargement of the cardiac silhouette.
Due to high clinical suspicion for cardiac tamponade, a bedside transthoracic echocardiogram (TTE) was obtained which showed a large pericardial effusion with diastolic compression of the right ventricle, suggestive of cardiac tamponade, as well as mild left ventricular systolic dysfunction (see Figures and ). Patient's INR was reversed to <1.5 and he underwent emergent subxiphoid pericardial window with the removal of 700 ml of turbid, dark, bloody pericardial fluid. He had significant improvement in hemodynamics and respiratory distress. The pericardial fluid cytology and pericardial biopsy were both positive for malignancy, consistent with metastatic UC (see ).
One week after the pericardial window, the patient underwent a computerized tomography (CT) scan of the chest, abdomen, and pelvis which did not show evidence of metastatic disease elsewhere. Given the recurrence of UC with metastasis to the pericardium, he was started on atezolizumab. Subsequently, he was noted to have recurrent pericardial effusion suggesting failure of treatment (see ). He was then started on pemetrexed, a folate antimetabolite chemotherapy drug, for metastatic UC with plans to complete 6 cycles. |
pmc-6029455-1 | A 21-year-old man with no known past medical history presented from prison with abdominal pain in the past five days. He explained that five days prior to presentation, he began experiencing squeezing abdominal pain, rated 4/10 localized to his RUQ with no radiation. He reported associated nausea, vomiting, diarrhea, and intermittent fever. The patient reported that he had initially thought he had the “stomach flu.” He had tried oral rehydration and Tylenol with no improvement in symptoms since onset. He reported continual progression of symptoms with minimal resolution in abdominal pain, episodes of vomiting, or diarrhea. He denied any change in diet, change in eating habits, recent travel, jaundice, tea-colored urine, itching, recent skin, and soft tissue infection. He stated that he was a previous smoker but quit since being incarcerated two years ago. He denied any alcohol or illicit drug use. He denied having any current sexual partner. He also denied any family history of illnesses or malignancy. On physical examination, patient was normotensive, tachycardic, and febrile on presentation with temperature recorded as 103.7F. Patient appeared weak and diaphoretic and had moderate right upper quadrant tenderness with no rebound tenderness or mass palpated and no other pertinent abdominal findings. Examination of the other systems revealed no abnormalities. The patient was admitted for further work-up of his condition given presentation from prison.
Initial laboratory results showed normocytic anemia (hemoglobin of 9.8, MCV 81.1), white blood cell count of 15.6 with significant left shift, sodium of 137 mmol/L (reference value 136-145 mmol/L), and potassium of 3.4 mmol/L (3.5-5.1 mmol/L) with normal renal function. Other significant labs on presentation included alanine aminotransferase (ALT) of 30 U/L (12-78 U/L), aspartate aminotransferase (AST) of 43 U/L (15-37 U/L), alkaline phosphatase (ALP) of 240 U/L (45-117 U/L), total bilirubin of 1.5 mg/dL (0.2-1.0 mg/dL), albumin of 1.4 g/dL (3.4-5.0 g/dL), and total protein 5.8 g/dL (6.3-9.0 g/dL). Rapid influenza testing was negative. Urine drug screen resulted as negative. Blood cultures from two peripheral sites were collected. Chest X-ray was significant for bibasilar infiltrates with bilateral pleural effusions noted. Given the clinical condition with concern for intra-abdominal source of infection, a CT scan of the abdomen/pelvis with IV contrast was performed. This showed a large multiloculated right hepatic mass, measuring 8.4cm with soft tissue mass extension into the upper pole of the right kidney (), unremarkable findings in the gallbladder, bowel, and appendix.
In the Emergency department, the patient was initially started on aggressive hydration with 0.9% normal saline given initial presentation prior to work-up. Ceftriaxone 1g Intravenous (IV) route and Metronidazole 500mg Intravenous (IV) route were administered in the emergency department. Blood cultures obtained from two peripheral sites resulted as gram-positive cocci with time to positivity about 15 hours. Patient was started on intravenous broad spectrum antibiotics with Vancomycin 1g IV q12 hours and Meropenem 1g IV q8 hours, the initial choice of therapy. Antibiotic sensitivity resulted in day two of admission as MSSA. Antibiotic therapy was narrowed down to Intravenous Nafcillin on day two of admission. Interventional radiology department was consulted for image guided percutaneous drainage of the liver abscess on day one of admission. Two abdominal drains were placed with initial return of 150ml of serosanguinous fluid. Fluid obtained from the CT-guided aspiration was sent to the laboratory for gram stain and culture. The culture obtained from percutaneous catheter drainage resulted as MSSA on day two of admission. The source of MSSA bacteremia was confirmed as pyogenic liver abscess and Nafcillin IV therapy was continued with no change in antibiotic management. Abdominal drains were removed on day six of admission as output remained minimal.
The patient was found to have bilateral pleural effusions. Given significant right sided pleural effusion, thoracentesis was performed on day two of admission with 1.2L of pleural fluid removed. Fluid was not sent for studies. Patient also developed nonoliguric acute kidney injury with creatinine rise from 0.7mg/dl (reference value 0.7-1.3 mg/dL) on admission to 1.7 mg/dL on day four of admission. Work-up was consistent with an intrinsic etiology of the acute kidney injury. The etiology was clinically suspected to be secondary to acute tubular injury due to sepsis. The patient was encouraged to continue per oral intake and intravenous fluids were given. Creatinine trended back to patient's baseline while on Nafcillin IV therapy. Repeat blood cultures obtained on day four of admission were consistently growing MSSA. Transthoracic echocardiogram was performed and it showed no evidence of intracardiac vegetations and no abnormalities. Repeat blood cultures on day six of admission were negative. A mid-line catheter was inserted in the patient's right upper extremity for vascular access on discharge for continued antibiotic therapy. The patient was discharged after 10 days with continued Nafcillin IV therapy for four weeks postdate of negative blood cultures.
The patient was followed up in the general surgery clinic one month after discharge. At this time, he had completed IV antibiotic therapy. Follow-up revealed stable clinical condition as patient reported resolution of symptoms and return to baseline functional status. Laboratory findings indicated normal renal function. Repeat CT imaging of the abdomen with IV contrast on follow-up visit showed resolution of the pyogenic liver abscess (). |
pmc-6029456-1 | A 35-year-old para 0+6 Black African lady presented with a history of five first and one second trimester recurrent pregnancy losses. In addition, she had a nine-year history of irregular heavy bleeding associated with dysmenorrhea.
Her menarche was at 17 years of age with regular painful cycles that lasted 10 days. She was not on any contraceptive method and did not report any dyspareunia or urinary symptoms. Her first miscarriage occurred 11 years and was surgically managed by dilatation and curettage. Subsequently she noted changes in her menstrual cycle. Her menstrual cycle became irregular with a heavy flow for 10 days associated with severe dysmenorrhea and bowel symptoms of bloating and diarrhea. She used tranexamic acid one gram three times a day during her menses for the heavy prolonged periods and Mefenamic Acid 500 milligrams three times a day for the dysmenorrhea with reported relief of the symptoms.
The physical examination was unremarkable except for mild tenderness in the suprapubic region; she had grossly normal external genitalia and normal looking cervix.
Her initial hormonal profile was as follows:FSH: 4.5 IU/m (3.1-7.9 IU/L) LH: 10 IU/L (1-18 IU/L)
She had a recent pap smear one year ago that was normal.
Transabdominal and transvaginal (TVS) scans had shown separate right and left cornu with multiple cysts in the peripheral ovarian parenchyma features suggestive of bicornuate uterus and polycystic ovaries (). She also had a hysterosalpingogram (HSG) that was reported to have a uterus opacified with banana configuration oriented to the right with no delineation of fallopian tubes, findings suggestive of a unicornuate uterus.
Her past medical history was not significant. She was recently divorced, a condition she attributed to her history of several miscarriages in the context of an African cultural expectation of siring children.
She was subsequently scheduled her for hysteroscopy and diagnostic laparoscopy. She also gave consent for possible open Strassmans metroplasty.
On hysteroscopy, a normal looking endometrium was visualized with a right sided tubal ostia and a small tubal ostia on the left that did not appear patent (). Laparoscopically, a bicornuate uterus was found () with associated endometriosis at the vesicouterine fold. Open metroplasty was done (Figures and ). A fundal transverse incision was made and dissection done to the level of the endometrium after injection of subserosal vasopressin. Apposition of the two horns was done and the uterus sutured in layers (Figures and ). A copper intrauterine device was left in situ to separate the uterine walls and possibly reduce the chances of uterine synechiae though the evidence for this practice is still not conclusive. In addition, endometriotic deposits discovered at the vesico uterine fold were ablated.
She had an unremarkable follow-up after surgery with no complications reported.
A HSG 6 months after surgery revealed unification of the uterine horns but both tubes were not delineated.
Patient was lost to follow-up but two years later she presented having conceived spontaneously and a TVS revealed a single intrauterine pregnancy at 13 weeks. Her antenatal workup was as highlighted in .
She was subsequently followed up at the antenatal clinic and had no concerns till 33 weeks when she presented with a history of lower abdominal pain worsening over the past few weeks and right sided fundal tenderness pain score of seven out of ten on the pain assessment scale. The main concern at the time of assessment was possible uterine rupture. She did not have any features suggestive of bowel obstruction as she was passing stool and bowel sounds were present. She had a normal NST and an obstetric ultrasound that was done showed a single intrauterine pregnancy with normal biophysical profile and no features of placental abruption or slower uterine segment site dehiscence. She had no other investigations done at this time. The pain was significant enough to warrant an admission for which she subsequently received antenatal steroids for lung maturity and opioid analgesia for pain relief. However, the pain did not completely resolve and she underwent a caesarean delivery at 33 weeks and 5 days due to persistent lower abdominal pain. The outcome was a live male infant with a birth weight of 1950 grams and APGAR of 9, 10, 10. There was no evidence of uterine rupture although she had venous congestion and massive varicose veins bilaterally at the cornual areas. The previous metroplasty scar was intact.
She had a resolution of the abdominal pain postoperatively and had an unremarkable postoperative recovery. She was discharged on the fifth postoperative day. |
pmc-6029461-1 | Case 1 is a 40-year-old male, presenting with general malaise, arthralgia in his extremities, facial cyanosis, and fever (39°C). In the medical history, a post-traumatic splenectomy was reported. Empirical antibiotic therapy started with ceftriaxone (2 g every 12h) and vancomycin (500 mg every 6h). After several bacteriological analysis, secondary sepsis caused by meningitis from Streptococcus pneumoniae was diagnosed. The patient was transferred to the intensive care unit (ICU) where his clinical status rapidly deteriorated, showing a severe hemodynamic instability with need for vasopressor support (norepinephrine 0.4 μg/kg/min), persistent hypotension, and a pronounced inflammatory state with C-reactive protein (CRP) at 176.3 mg/l and procalcitonin (PCT) at 485.88 ng/ml. The patient also exhibited metabolic abnormalities, with lactate levels of 7.8 mmol/l, and severe coagulative disorders.
In face of a persistent anuria, continuous renal replacement therapy (CRRT), hemodiafiltration mode (CVVHDF, Prismaflex, M150, Baxter, USA) was started, together with Cytosorb cartridge, for a total of 68 hours (start of treatment <24h after ICU admission).
A general improvement was obtained already during the first treatment. PCT could be reduced to 6 ng/ml at the end of the treatments. Similarly, CRP also dropped to 136.41 mg/l during the course of the treatments. On the metabolic level, the patient showed a marked improvement in lactate acidosis, with lactate levels decreasing to 1.9 mmol/dl. From a hemodynamic point of view, a MAP stabilization could be achieved paralleled with a reduction in the need for norepinephrine. The patient could be stopped from CRRT after 12 days with a recovery of diuresis. The course for norepinephrine demand during Cytosorb treatment is presented in , whereas the course of lactate and PCT is shown in .
The patient was still hospitalized after 2 months, however in an alert, collaborative, tracheotomised, spontaneously breathing state, waiting for transfer to a rehabilitation facility. |
pmc-6029461-2 | Case 2 is a 66-year-old female, presenting with general illness, fever (40°C), and hypotension. Prior medical history included beta-blocker therapy for tachyarrhythmia, appendectomy, colon cancer, and splenectomy for colonic metastases. On admission, she exhibited skin ischemic lesions with rapid deterioration to whole body cyanosis and metabolic and lactic acidosis. Empirical antibiotic therapy started with ceftriaxone (2 g every 12h) and levofloxacin (500 mg every 12h). After 24h, blood cultures confirmed the diagnosis of sepsis from Streptococcus pneumoniae paralleled with severe purpura fulminans. Empirical antibiotic therapy continued for 11 and 7 days, respectively, with adjustments due to renal function changes. In the further course, she became oligoanuric, severely hypotonic with hemodynamic instability (epinephrine 0.4 μg/kg/min), and thrombocytopenic, presenting severe bleeding.
A CVVHDF treatment (Multifiltrate, AV1000, Fresenius Medical Care, Germany) was started in combination with one 24-hour session of Cytosorb hemoadsorption immediately after the ICU admission.
This combined treatment resulted in a clear and progressive hemodynamic stabilization accompanied by a marked reduction of epinephrine to 0.1 μg/kg/min () and a reduction of CRP, from 17.5 mg/dl to 13 mg/dl. Initially diuresis started to work again; however this went back to anuria in the first 12 hours (probably due to sepsis-associated tubular necrosis) and fully recovered later on day 10. Lactacidemia, elevated at the admission (15 mmol/l), decreased until 4 in the first 24h. At the same time, PCT decreased from 18.35 ng/ml to 2 ng/ml after 6 days ().
Subsequent to this first acute event, the patient showed a recrudescence of the septic state. Blood cultures were positive for Enterobacter aerogenes, which colonise the body through the injured intestine wall, resulting from purpura fulminans. Because of her severe injuries at the extremities, the patient needed amputations and 2 months later, the reoccurrence of the second septic episode caused the death of the patient. |
pmc-6029461-3 | Case 3 is a 47-year-old male showing fever (38°C), asthenia, and lumbar pain with signs of diffuse petechiae. In the further course, he became severely hypovolemic and tachycardic, showing also metabolic acidosis (lactate 12 mmol/l). Blood cultures were taken and empirical antibiotic therapy was started with meropenem (2 g every 8h) and amikacin (1 g every 24h). Diagnoses confirmed by blood cultures were septic shock secondary to Neisseria meningitidis infection (Serotype C) and antibiotic therapy was replaced with ceftriaxone (2 g every 12h). After transfer to the ICU, norepinephrine infusion was started at 0.2 μg/kg/min and rapidly increased to 0.5 μg/kg/min, in combination with epinephrine infusion at 0.2 μg/kg/min. Inflammation was controlled with PCT, which was extremely elevated, 121.7 ng/ml. In the following hours, a worsening of general conditions of the patient was reported, with extension of petechiae and severe bleeding from the insertion points of the catheters and severe hypotension with the increase of norepinephrine up to 0.75 μg/kg/min. In face of a persistent condition of oligoanuria, it was necessary starting a hemofiltration treatment (CVVH, Prismaflex, M150, Baxter, USA) and Cytosorb was additionally installed into the CVVH circuit the next day (start of treatment: 15h after ICU admission).
Within the course of 72-hour Cytosorb treatments, the patient witnessed a hemodynamic improvement with norepinephrine and epinephrine being tapered off after the third session () as well as a stabilization of lactate values and inflammation, with PCT decreased until 16.52 ng/ml ().
The patient also showed an attenuation of disseminated intravascular coagulopathy, a demarcation of skin necrosis areas, and a recovery of vital functions. The patient could be extubated 10 days and weaned from CVVH 12 days after the ICU admission. He was transferred to rehabilitation medicine after 23 days from the recovery, waiting for plastic surgeon consultant for the amputation of some phalanges. |
pmc-6029461-4 | Case 5 is a 36-year-old female, showing a persistent fever, severe polymyalgia, and diffuse petechiae in expansion to the limbs and arms. Antibiotic therapy was started with levofloxacin (750 mg every 24h) and ceftriaxone (2 g every 12h). The patient was immediately transferred to ICU with a suspected diagnosis of sepsis secondary to meningitis. The patient presented hypotension, requiring the administration of norepinephrine at 0.5 μg/kg/min and dobutamine in the next day. Lab tests confirmed the diagnosis of bacterial meningitis infection caused by Neisseria meningitidis (Serotype C) and underlined a condition of thrombocytopenia, acute renal failure, and inflammation, initially monitored with CRP (17.5 mg/l). Lactate level was at 3.6 mmol/l. Immediately after the ICU admission, a CVVHDF treatment (Prismaflex, Oxiris, Baxter, USA) was started in combination with Cytosorb (start of treatment: 8h after ICU admission).
Within the course of hemoadsorption treatments, performed for a total of 96 hours, a hemodynamic stabilization was observed, associated with a reduction in need of inotrope drugs. Indeed, dobutamine could be stopped after 48h and norepinephrine after 78h. The course of norepinephrine is shown in . The patient showed a stabilization lactate level, reduced at 1.7 mmol/l, as presented in . Moreover, the inflammatory status was also monitored during Cytosorb treatment dosing IL-6 levels (), directly adsorbed by the sorbent. After 12h of hemoperfusion, IL-6 values were 4.326 ng/ml and decreased dramatically during the course of treatments until 60.1 ng/ml after 60h and 35 ng/ml after 72h. Petechiae and skin lesions were stable already after the first day of treatment. A complete recovery of renal function and diuresis was observed after 9 days from the admission and the patient could be transferred to medicine after 7 days. The patient was followed by dermatologists and vascular surgeons for the outcome of the septic embolism complication, evaluating the possible amputation of one toe. |
pmc-6029463-1 | This case describes a 23-year-old male who was struck by a motor vehicle. Upon arrival at our hospital, the patient had a GCS of 8. FAST exam, chest radiograph, and computed topography (CT) of the head and cervical spine were obtained and were negative.
Exam of the lower extremities revealed abrasions over the left knee and tenderness over the lateral joint line with an effusion. The right knee was diffusely tender to palpation without effusion. The patient had palpable pulses in both feet with well-perfused extremities. Ankle brachial indices were performed and found to be >0.9. He demonstrated guarding and pain with the attempted Lachman maneuver of the left knee and slight opening of the left knee joint with varus stress. Radiographs were obtained and revealed a left knee Segond fracture ().
Magnetic resonance imaging (MRI) of both knees was performed to evaluate for ligamentous injury. Left knee imaging demonstrated the Segond fracture along with a grade III lateral collateral ligament (LCL) tear with retraction (), a grade II tear of the popliteus tendon and anterior cruciate ligament (ACL) (), and a grade I medial collateral ligament (MCL) injury (), as well as partial thickness tears of the biceps femoris and vastus medialis. Right knee imaging revealed a grade III tear of the ACL and MCL (), grade II tears of the posterior cruciate ligament (PCL) (), LCL, and popliteus tendon, and a medial meniscus tear. The patient was placed in bilateral hinged braces with the left knee unlocked and the right knee in locked extension to aid with transfers from a bed to a wheelchair. The patient was also given a left foot drop boot for a foot drop discovered during a secondary exam. On hospital day three, the patient was discharged home.
Nine days after the accident, the patient presented to the clinic. He noted that the left-sided foot drop was improving. On that side, he had 5/5 strength of his extensor hallucis longus and tibialis anterior (TA), without any sensory deficits in the peroneal nerve distributions. On the physical exam of the left knee, the Lachman maneuver was grade 2B (ACL injury with 5–10 mm translation without an endpoint), the varus stress test grade 3 (complete LCL tear with >10 mm opening of the lateral joint), and the valgus stress test grade 2 (MCL injury with 6–10 mm opening of the medial joint). The right lower extremity was also neurovascularly intact, and the right knee exam revealed a grade 2A Lachman maneuver (ACL injury with 5–10 mm translation and a firm endpoint), a grade 3 posterior drawer test (complete tear of PCL with >10 mm posterior tibial translation), and a grade 3 valgus stress test (MCL injury with 11–15 mm opening of the medial joint), with a presumptive positive dial maneuver on the right side at 30 and 90 degrees (consistent with PCL and posterolateral corner (PLC) injury). However, given that the patient had bilateral PLC injuries, this physical exam finding was somewhat subjective without a reference point on the contralateral side. Subtle gapping with varus stress was also documented.
Multiligamentous reconstructions of both knees were recommended (). The left knee was addressed first in order to explore and decompress the common peroneal nerve. In regard to the right knee, preoperative physical therapy was performed to restore range of motion (ROM) before undergoing surgery.
Intraoperative findings of the left knee included a positive lateral gutter drive-through sign indicative of a PLC injury. The LCL was avulsed off the fibula, and the anterior lateral ligament (ALL) was also avulsed off the tibia. A greater than 50% disruption of the ACL was observed. Exam under anesthesia demonstrated a grade 2A Lachman maneuver, a grade 2 pivot shift, grade 3 varus instability, and instability on external rotation. The procedure included ACL reconstruction with a hamstring autograft augmented with an allograft, PLC reconstruction utilizing a TA allograft, and repair of the native avulsed LCL and ALL with suture anchors (). First, the hamstrings were harvested and augmented with an allograft, and the tunnels for the ACL reconstruction were drilled. The PLC was then reconstructed using the anatomic technique described by Malanga et al. []. The native LCL was repaired using suture anchors with the overlying allograft reconstruction used to supplement it. The posterolateral capsule was then reefed into the LCL allograft reconstruction. Finally, the ACL graft was passed and fixed. Postoperatively, the patient was placed in a hinged brace locked in extension and was made toe-touch weight bearing.
The decision was made to proceed with the right knee reconstruction one week later. Exam under anesthesia revealed a grade 2A Lachman maneuver, grade 3 posterior drawer test, a grade 3 varus stress test, and a grade 2 valgus stress test. Surgery included ACL reconstruction with a bone-tendon-bone (BTB) autograft, PCL reconstruction with an Achilles allograft, MCL primary repair with additional Achilles allograft reconstruction, PLC reconstruction with TA allograft, and repair of the posterior horn of the medial meniscus (). The lateral exposure for the PLC reconstruction was performed first, and the blind-ended sockets and fibular tunnel were drilled. An open approach to the MCL was then performed, and the injured MCL was found and tagged for later repair. The BTB autograft was then harvested for the ACL reconstruction. At this point, the posterior horn of the medial meniscus was confirmed to be torn from its root so this was repaired using sutures passed through a tibial tunnel. Guide pins were then passed for the ACL and PCL tunnels to ensure there was no convergence. Both tunnels were then reamed and the grafts passed. The PCL was fixed first while the leg was flexed, and the ACL was then fixed with the leg in extension. The MCL repair and reconstruction with an allograft were completed using the surgical technique described by Sekiya et al. [], followed by the PLC reconstruction which was carried out utilizing the same method noted previously. Postoperative immobilization and weight bearing status were the same as those in the contralateral side.
Range of motion and physical therapy rehabilitation began at 1 week postoperatively. Early exercises included isometric activities to strengthen the quadriceps and patella mobilization exercises. Both knees were kept in a brace locked in extension with minimal weight bearing the first six weeks following surgery. Six weeks after the initial reconstruction, the patient was instructed to begin weight bearing with crutch assistance, starting in extension and then unlocking the straight leg brace to 90° of flexion. At eight weeks post-op, the patient was transitioned out of knee braces and then given clearance to return to work ten weeks after surgery.
At six months post-op, the patient completed physical therapy. On the physical exam, the patient's knees demonstrated full ROM bilaterally with a grade 1A Lachman maneuver in both knees. The right knee also had a grade 2A posterior drawer test without sag. At that time, clearance was given to begin straight running. At one year post-op, the patient had returned to jogging and playing basketball recreationally and was able to participate in strenuous work (, ). |
pmc-6029475-1 | A 70-year-old woman presented in November 2017 to the Emergency Department at Skåne University Hospital, Sweden, due to the rapid onset of fever, shivers, and a suspected skin infection. She had a previous medical history of left-sided ductal breast cancer with lymph node involvement in 1999, which was treated chronologically with neoadjuvant chemotherapy, partial mastectomy, axillary lymph node dissection, and radiation therapy. In addition, in 2001, a right-sided localised ductal breast cancer in situ was identified and was treated surgically with a partial mastectomy. Secondary to her lymph node dissection, she developed lymphoedema of her left arm, which had been continuously treated with compression stockings. The patient was on treatment with an ACE inhibitor and a beta-blocker due to hypertension, and in addition, she had a known systolic murmur, characterized as physiological, as transthoracic echocardiographs in 2011 and 2017 were normal. Since her surgery in 1999, on a total of six occasions prior to her last and seventh visit, of which the first episode occurred in 2008, she had been treated for erysipelas in her left upper arm. The presentation had always been sudden with spiking fever and erythema spreading in approximately the same localisation. Interestingly, on all three out of the three occasions where a blood culture has been drawn on presentation with erysipelas, the cultures have shown growth of a bacterium belonging to the S. mitis group. These first two isolates also had similar MIC values for penicillin of 0.064 and 0.125 mg/L, for vancomycin of 0.25 and 0.5 mg/L, and for gentamicin of 2 and 2 mg/L (). In addition, they were both sensitive to clindamycin.
On the present visit, she once again had a sharply demarcated, warm, swollen, and painful erythema measuring approximately 7 × 15 cm in the lymphoedematous area on her left upper arm. No local portal of bacterial entry was found. Vital parameters showed a temperature of 38.0°C, respiratory rate of 16 breaths/min, O2 saturation of 96% on room air, heart rate of 80 beats/min, and blood pressure of 120/70 mmHg. On physical examination, a grade II systolic murmur was heard with punctum maximum I2 dexter. She had no signs of septic emboli, oral examination showed no signs of infection, and examination of lymph nodes was normal. Possibly due to her quick presentation, that is, less than 6 hours from the onset of symptoms, her laboratory results were normal with a white blood cell count of 8.4 ∗ 109/L, platelets of 263 ∗ 109/L, and hemoglobin of 147 g/L. Her CRP was 12 mg/L. She was clinically diagnosed with erysipelas, and due to previous bacteraemia with the S. mitis group in relation to erysipelas and the presence of a systolic murmur, blood cultures were drawn and she was treated with one dose of intravenous penicillin (3g≈5 million IU) followed by an oral penicillin (1g≈1.6 million IU) three times daily, for seven days. Once again, now for the third time, the two blood cultures showed growth of a bacterium belonging to the S. mitis group. The MIC value for penicillin was 0.125 mg/L, for vancomycin 1 mg/L, and for gentamicin 16 mg/L (). Similar to the two previous isolates, it was also sensitive to clindamycin. Her treatment was prolonged for 10 days, and a follow-up visit was arranged. Repeat blood cultures were drawn 14 days after discontinuation of antibiotics and they were negative. To prevent further infections, she has once again been referred to the lymphoedema outpatient clinic as well as to the dentist office. On follow-up, thereafter, the patient had no sequelae to her infection, and she gave informed consent for this case report to be published.
The three blood isolates, one analysed in 2015 and two in 2017 (15 and 8 months apart), were initially subgrouped to S. mitis/S. oralis/S. pseudopneumoniae of the S. mitis group by combining the MALDI-TOF MS results (MALDI Biotyper, Bruker) with the information that the three stains were resistant to optochin. To allow a more detailed comparison, the three stored isolates were reanalysed and now ethanol/formic acid extractions were performed on the strains, and the updated and improved Bruker MALDI Biotyper database (DB-7311 MSP Library) was used for the MALDI Biotyper analysis. In addition to the standard log (score), weighted list (scores) was also calculated []. S. mitis was the best match for both the first and second isolates when both log (score) and list (score) were calculated. For the third isolate, the best match was S. oralis for both types of scores (). Next, the mass spectra of the three isolates were inspected manually. All three strains showed the specific peak 6839.1 m/z which is associated with S mitis and S. oralis strains, but only the third isolate showed the specific peak 5822.5 m/z which is associated with S. oralis () []. In addition, no peak profiles typical for S. pneumoniae and S. pseudopneumoniae could be detected in the three isolates [, ]. These results further support that the first two isolates are S. mitis and the third isolate is S. oralis. Many differences were seen in the mass spectra of the third isolate (S. oralis) compared to the first two (S. mitis). On the other hand, no clear differences in the spectra between the first and second isolate could be seen, and one can therefore not exclude that they belong to the same clone. |
pmc-6029490-1 | A 79-year-old man with a past medical history significant for hypertension, chronic kidney disease stage IV, dementia, and chronic obstructive pulmonary disease presented to the Emergency Department after being transferred from a correctional facility due to low hemoglobin found on routine labs. On arrival, his vital signs included a temperature of 36.5°C, heart rate of 88 bpm, respiratory rate of 22/min, blood pressure of 159/70 mmHg, and saturation of 99% on room air. Physical examination was notable for dry oral mucosa and poor dentition, a 2/6 systolic murmur best heard at the left sternal border, and hematomas on both posterior shoulders, bilateral upper arms, and the right medial forearm. The only medication the patient was taking at the time was amlodipine 10 mg daily for hypertension. Upon admission, the patient was agitated and hostile to interview and was thus treated with Haldol 5 mg IM x1. Laboratory studies were performed and showed a hemoglobin level of 6.9 g/dL (decreased from his baseline: 10 g/dL), elevated BUN at 99 mmol/L, and creatinine of 3.23 mg/dL (increased from his baseline creatinine: 2.7 mg/dL).
He denied any symptoms associated with anemia such as lightheadedness, dizziness, shortness of breath, hemoptysis, or hematemesis. He stated that he was unsure if there was melena because he does not routinely inspect his stool. He was initially started on intravenous fluids and given red blood cell transfusion. Immediately after transfusion, his hemoglobin increased to 7.5 g/dL; however, hemoglobin levels continued dropping on subsequent days with the lowest level at 4.6 g/dL. As a result, the patient required a total of 6 packed red blood cell transfusions. After continued intravenous hydration and transfusions, BUN and creatinine decreased to 57 mmol/L and 2.3 mg/dL, respectively. Occult gastrointestinal (GI) bleeding was suspected due to consistent downtrending hemoglobin refractory to blood transfusions. The patient became more cooperative on subsequent days and complained of lower left quadrant abdominal pain as well as chronic bilateral leg pain. The fecal occult blood test was performed and was positive both times. CT abdomen () was performed to rule out intra-abdominal causes of acute anemia and showed right perinephric and right paracolic gutter fat stranding surrounding the right iliopsoas muscle which was asymmetrically enlarged without evidence of definite hyperdense intramuscular hematoma, but possible intramuscular hemorrhage or myositis.
The gastroenterology team was consulted, and esophagogastroduodenoscopy (EGD) and colonoscopy were performed. EGD did not yield any abnormal results, but colonoscopy revealed terminal ileum containing hematin (altered blood/coffee-ground-like material) without a clear source of bleeding (). Coagulation studies were subsequently performed which showed an elevated activated partial thromboplastin time (aPTT) at 101.5 seconds. Hematology service was consulted and a mixing study was ordered, which showed only partial correction of the aPTT, suggesting the possibility of a coagulation factor-specific antibody. Levels of factors VIII, IX, XI, and XII were ordered and showed factor VIII activity of <0.01 unit/mL, factor IX activity of 0.80 unit/mL, factor XI activity of 0.40 unit/mL, and factor XII activity of 0.34 unit/mL. Subsequently, the Bethesda assay was performed to measure autoantibody levels against any of these factors and showed an elevated factor VIII inhibitor level at 390 U/mL, which confirmed the diagnosis of acquired hemophilia A. The patient was started on cyclophosphamide 50 mg QD and dexamethasone 20 mg QD to inhibit the body's immune response against factor VIII. Inhibitor levels decreased to 280 U/mL after 10 days of therapy. Etiology of the patient's acquired factor VIII inhibitor was unclear, so tumor markers (PSA, CEA, CA 19-9) and CT scans (thorax and abdominopelvic) were ordered in order to rule out the underlying malignancy. All markers and imaging were within normal limits. Hemoglobin remained stable at 8 g/dL after starting appropriate treatment. Because throughout the next few weeks, the inhibitor level did not decrease greatly in response to cyclophosphamide therapy, the patient was started on weekly infusions of rituximab. The most recent factor VIII inhibitor level was down to 70.4 U/mL with stable hemoglobin at 13.2 g/dL. Hematology continues to closely monitor the quantity of the inhibitor levels and his response to treatment. In addition, the patient is advised to avoid any activity in which he may sustain trauma or fall due to his increased risk of bleeding. |
pmc-6029492-1 | A 13-month-old female presented to the emergency department for urinary retention. She was born at 35 weeks gestation in Mexico with an unknown postnatal hospital course. She was known to have mild gross motor developmental delay diagnosed several months before; caregivers stated that she pulled to stand but did not cruise or walk and babbled but did not have any words. She had no additional past medical history or previous surgeries and no known previous trauma. Upon presentation, the adoptive parents with whom she has lived since very early in her life provided the history. Adoptive parents reported about 2 weeks of fussiness and decreased ability to bear weight on her lower extremities. Parents denied any known trauma at that time. Her weight was <3rd percentile with a Z score of −3.36. She was thin appearing but without signs of dehydration or malnourishment. Examination was notable for 0/5 strength, hypotonia, and areflexia in the bilateral lower extremities as well as hypotonia of the trunk. In addition, she was found to have significant head lag and very severe thoracic kyphosis or gibbus deformity. The patient was first evaluated by outpatient urology after referral from the primary care office for urinary retention. A renal ultrasound was performed and was notable for bilateral moderate hydronephrosis and bladder distension. She was sent to the emergency department for further workup. Laboratory studies in the emergency department yielded normal creatinine for age at 0.3 mg/dl, and urine culture obtained by in and out bladder catheterization grew >100,000 CFU/mL of Enterococcus faecalis. Complete blood count and basic metabolic panel were unremarkable, and thyroid stimulating hormone was within normal range. The patient's creatinine kinase was >1200 U/L, six times the upper limit of normal. Spinal CT demonstrated complete retrospondylolisthesis of T12 vertebra (). MRI was also performed and showed marked compression of the spinal cord resulting in inability to visualize the cord at the level to T12 with surrounding cord edema (). Brain MRI was without abnormality. This imaging also revealed multiple rib fractures in different stages of healing. Dedicated osseous survey showed numerous right and left posterior rib fractures. Liver function tests, lipase, amylase, phosphorous, parathyroid hormone, and vitamin D were obtained to rule out congenital or acquired reasons for predisposition for bony fractures, all of which were found to be within normal range. The patient's findings were consistent with traumatic spinal spondyloptosis with additional findings concerning for physical child abuse. The initial history provided to the medical team included a confusing timeline and described a progressive decline in motor function. That along with a physical examination notable for head lag made this diagnosis initially challenging. Alternative diagnoses of systemic neurodegenerative disorders such as spinal muscular atrophy or other diseases of motor function such as botulism were considered. However, after the imaging, the diagnosis became clear, and concerns for NAT were raised. After confirmation of her diagnosis with imaging, the hospital's child protection team and the state of North Carolina's child protective services were notified. The patient underwent posterior spinal fusion from T11 to L1 to stabilize her spine. She also received intensive rehabilitation after surgery. Unfortunately, she did not regain lower extremity function and remains paraplegic. Her adoptive mother later admitted to shaking the child aggressively by holding her at the level of mid abdomen/lower thoracic back (the area of injury) and throwing her on the floor several weeks prior to presentation. |
pmc-6029504-1 | Case 1. In a 56-year-old woman with diagnosed ductal carcinoma in situ, the following imaging parameters were used: voltage, 30 kV; tube current, 61; thickness, 46 mm; AGD, 1.75 (15 projections). |
pmc-6029504-2 | Case 2. In a 62-year-old woman with a diagnosis of scirrhous, the following parameters were used: voltage, 29 kV; tube current, 47; thickness, 39 mm; AGD, 1.32 (15 projections). |
pmc-6029504-3 | Case 3. In an 81-year-old woman with a diagnosis of solid tubular carcinoma, the following parameters were set: voltage, 29 kV; tube current, 48; thickness, 41 mm; AGD 1.29 (15 projections). |
pmc-6029576-1 | A 38-year-old woman was referred to Kennedy Krieger Institute a year after being officially diagnosed with TSC at the National Institutes of Health (NIH) in October 2012. Before her diagnosis, the patient had several TSC-related manifestations that should have solidified diagnosis much earlier in her life. She had TSC-associated skin lesions, such as forehead plaques that were apparent as an infant and facial angiofibromas that developed later in her childhood. In her teenage years, she developed periungual fibromas on her foot that would only be removed by a podiatrist in 2003 when she was an adult.
In October 2009, at age 34, the patient had been put on hemodialysis; however, incomplete records received by our institute did not identify the indication for this. At least 1 large (3.8-cm) renal angiomyolipoma in the left kidney and multiple bilateral cysts were identified in 2011. These disease manifestations led the patient to undergo a bilateral nephrectomy later that year at age 36, and renal transplantation was considered as an option in the following year. She had also developed cardiac issues such as hypokinesis and low ejection fraction (40%), likely the result of renal complications. In addition to renal and cardiac issues, lung deterioration was observed as early as 2011. In October 2011, the patient presented with interstitial and alveolar infiltrates with bilateral pleural effusion. Diffuse lung cysts were observed in September 2012.
In October 2012, she was evaluated and diagnosed with TSC at the NIH. Her diagnosis was based on multiple manifestations of TSC identified during her evaluation. Numerous types of TSC-associated skin lesions were identified, including facial angiofibromas, cephalic plaques, oral fibromas, shagreen patches, ungual fibromas, and confetti skin lesions. Dental pitting was also observed. Brain magnetic resonance imaging (MRI) revealed evidence of bilateral abnormal gray/white matter and bilateral subependymal nodules. Two retinal hamartomas were found in her left eye.
The patient’s lung function subsequently declined, and in 2013, while being treated at Johns Hopkins Hospital, she was indicated for lung transplantation. However, there was a decision to wait for further decline because of the possibility of recurrence. She was also switched from hemodialysis to peritoneal dialysis to lessen fatigue.
The patient’s first visit to our clinic occurred in September 2013, at which point she underwent an initial evaluation for comprehensive management of TSC. After examination and reviewing her past medical history, we found that there were no concerns regarding epilepsy or neurodevelopmental delay. Upon lung examination, decreased breath sounds were noted, and it was recommended to start everolimus 5 mg daily to slow the progression of LAM and potentially improve lung function; however, she elected not to initiate treatment at that time. She was listed for renal transplantation pending results of pulmonary testing. A full dental consultation with follow-ups every 6 months was also recommended, in particular for sealant of dental pits.
In a subsequent visit in February 2014, abdominal imaging was recommended to monitor a previously-detected liver angiomyolipoma. After pulmonary tests, follow-up with other disciplines was recommended, including nephrology, neurology, pulmonology, ophthalmology, dentistry, dermatology, and cardiology. At this time, everolimus was recommended at 10 mg daily for treating pulmonary LAM, and the patient agreed to initiate treatment after this visit.
After 1 day of taking everolimus 10 mg, the patient experienced dizziness and hypotension and self-discontinued treatment. Upon her visit in July 2014, it was decided to restart everolimus at 5 mg once daily, and midodrine was initiated for hypotension. The patient did not experience dizziness at the 5-mg dose.
The patient’s next visit at our clinic would not be until March 2015. However, before this visit, she was scheduled for renal transplantation surgery that was stopped because of difficulties with anesthesia. As a result of depressed mood after her canceled renal transplantation, she self-discontinued everolimus again. As a result, pulmonary nodules and cysts throughout the lungs were observed with computed tomography, accompanied with worsening pulmonary function, and were likely attributable to the interruption of everolimus. We recommended she restart everolimus 5 mg once daily, and at her next visit focus on counseling and medication for depressed mood. As of July 2015, her mood has improved, and she is continuing everolimus 5 mg, with amenorrhea reported as the only noticed side effect. |
pmc-6029729-1 | A 58-year-old woman with a four-year history of hypertension-diabetes comorbidity presented to the outpatient department of the Nyakach County Hospital with edematous swelling of the face and upper and lower lips of 11-hour duration (Figure ).
There was associated dysphagia with stridor and hoarseness of voice. She did not have any pruritus, urticaria, or rashes. The tongue was swollen and was reported as hard in consistency. It was wedged between her teeth which prevented her from closing her mouth. Saliva was pooling and dribbling from her mouth. The patient had no history of smoking, angiotensin-converting enzyme (ACE) inhibitor-induced cough, atopy, or any recent use of aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs). Additionally, there was no prior history of a similar episode. The outpatient card indicated that the patient had tolerated a twice daily peroral dose of metformin 500 mg, a once daily peroral dose of glibenclamide 5 mg, a once daily peroral dose of hydrochlorothiazide 50 mg, and a once daily per oral dose of enalapril 5 mg for four years. Other aspects of her medical history were unremarkable. On examination, she had a pulse rate of 75 beats per minute, respiratory rate of 26 breaths per minute, and blood pressure of 140/72 mmHg. Pulse oximetry was not done. Her systemic examination was unremarkable. Review of her medication profile prompted us to suspect enalapril as the cause of the angioedema. Thus, we made use of the Naranjo probability scale to evaluate the likelihood that the observed effect was enalapril induced. Specific responses on this scale were one, two, one, zero, two, zero, zero, zero, zero, one for a cumulative score of seven, which ranks as probable. Therefore, a preliminary diagnosis of enalapril-induced angioedema was made and the enalapril was immediately discontinued. A 200 mg stat intravenous (IV) dose of hydrocortisone and a 20 mg stat dose of intravenous chlorpheniramine were administered and the patient was observed for one hour. The edema was noted to subside (Figure ) and a further three hour period of observation was allowed. The patient was then discharged on a once daily peroral dose of nifedipine 20 mg, a thrice daily peroral dose of chlorpheniramine 8 mg, and a tapered peroral dose of prednisolone as follows: 40 mg thrice daily for five days, 20 mg thrice daily for five days, 10 mg thrice daily for five days, and 5 mg thrice daily for five days. In addition, she was advised to continue with her usual doses of metformin, glibenclamide, and hydrochlorothiazide and to report to the hospital immediately if the swelling was to re-occur. On follow-up after one week, there was a marked change in facial appearance (Figure ), and at three weeks, the patient was symptom-free with a blood pressure (BP) of 142/78 mmHg (Figure ). |
pmc-6029729-2 | A 55-year-old male with a six-year history of hypertension presented to the Nyakach County Hospital with swelling of the upper lip of 10-hours duration (Figure ). The patient complained of restlessness and insomnia. There was no history of atopy or food or drug allergies. On examination, vitals were normal. Moreover, cardiovascular, respiratory, and abdominal examinations were unremarkable. The outpatient card indicated that he had tolerated nifedipine 20 mg, enalapril 5 mg, and hydrochlorothiazide 25 mg for six years. We suspected enalapril to be responsible for the edema and thus made use of the Naranjo probability scale to evaluate whether the observed edema was enalapril induced. The responses on this scale were one, two, one, zero, two, zero, zero, zero, zero, one for a total score of seven, which ranks as probable. Therefore, a preliminary diagnosis of enalapril-induced angioedema was made, enalapril withheld, and the patient immediately put on 4 mg intravenous dexamethasone injection and monitored for one hour. A gradual decrease in the swelling was observed about two hours after the intravenous dose of dexamethasone. The patient was discharged on a peroral dose of prednisolone tapered as follows: a thrice daily 20 mg dose for five days, a thrice daily 10 mg dose for five days, and a thrice daily 5 mg dose for five days. A twice daily peroral dose of cetirizine 10 mg was also given for 10 days, and the patient was advised to continue taking the nifedipine and hydrochlorothiazide, but to report immediately to the hospital if the swelling re-occured. After one week, the patient was reviewed and it was observed that there was a significant resolution of the swelling. On subsequent follow-up after three weeks, it was observed that the patient was symptom-free (Figure ). |
pmc-6029732-1 | A physically healthy 19-year-old schizophrenic girl on oral olanzapine 10 mg nightly for the past two years complained of bilateral progressive blurring of vision for four months. She denied any history of eye trauma, red eye, or eye pain. Besides that, she also had polydipsia and nocturia.
On examination, visual acuity was counting fingers at 1 m distance in both eyes. Anterior segment examination revealed bilateral diffuse cortical cataract (Figure ) precluding fundus examination. Ultrasound B-scans of both eyes showed normal posterior segments.
Systemic workup revealed random blood sugar of 21 mmol/L and an HbA1C of 17.1 %. She was referred to an endocrinologist for initiation of treatment to stabilise her blood sugar. The psychiatrist in charge was alerted as well. Both disciplines agreed to substitute oral olanzapine for intra-muscular flupentixol. The patient was also started on oral anti-diabetic medication.
She underwent uneventful bilateral lens aspiration with intraocular lens implantation under general anaesthesia. After the surgery, her best corrected visual acuity was 6/6 in both eyes. She responded well to the new antipsychotic treatment and her blood sugar was well controlled on two types of oral anti-diabetic agents. On her last review, her blood sugar levels had normalised. The fundi were normal. |
pmc-6029733-1 | A 54-year-old male with a history of non-ischemic cardiomyopathy (left ventricular ejection fraction of 20%), atrial fibrillation, hypertension, and insulin-dependent diabetes mellitus presented with lethargy and confusion. His home medications included aspirin, glimepiride, duloxetine, gabapentin, insulin glargine, prasugrel, furosemide, sotalol, rivaroxaban, and spironolactone. He also did not have a history of tobacco, alcohol, or illicit drug use.
He had a prior hospitalization for orthostatic hypotension and was eventually discharged to a skilled nursing facility but returned to the hospital three days later with an acute change in his mental status. On presentation, he was noted to have a respiratory rate of 26 breaths per minute, a heart rate of 62 beats per minute, blood pressure of 121/73 mmHg, a temperature of 37°C, and oxygen saturation of 97% on room air. The laboratory investigation revealed thrombocytopenia of 35 k/mcL as compared to 137 k/mcL three days earlier. Further workup was significant for an anion gap of 40 and LA of 18.7 mmol/L (reference range (ref) 0.5 - 2.0 mmol/L). He had also developed acute renal injury with creatinine of 1.9 mg/dL (ref 0.70 - 1.50 mg/dL). Liver enzymes were also elevated with aspartate aminotransferase (AST) of 254 U/L (ref 17 - 59 U/L), alanine aminotransferase (ALT) of 180 U/L (ref 21 - 72 U/L), and total bilirubin of 1.9 mg/dL (ref 0.2 - 1.2 mg/dL). Sepsis bundle was initiated with intravenous (IV) fluids and broad-spectrum antibiotics as well as microbial cultures, including the collection of two sets of blood cultures.
Additional investigation showed that lactate dehydrogenase was 1472 U/L (ref 110 - 216 U/L) but haptoglobin was normal: 85.9 mg/dL (ref 16 - 200 mg/dL). His hemoglobin was 6.4 g/dL (ref 14 - 17.4 g/dL), requiring blood transfusions while he also developed leukopenia; absolute neutrophil count (ANC) was 670. Due to his change in mental status associated with thrombocytopenia, anemia, and renal injury, there was a concern for thrombotic thrombocytopenic purpura (TTP). The peripheral blood smear demonstrated decreased platelets and an absence of schistocytes or blasts, but it did show numerous smudge cells and a few nucleated blood cells. ADAMTS-13 was 98 (ref >66 %) ruling out TTP. A computed tomography (CT) scan of the chest, abdomen, and pelvis only revealed an enlarged liver and moderate splenomegaly but did not show any lymphadenopathy.
Although his presentation was considered likely from sepsis, as his condition continued to deteriorate, hemophagocytic lymphohistiocytosis (HLH) was also considered. He met four out of five criteria for HLH with a ferritin of 9374 ng/mL (ref 20 - 300 ng/mL) cytopenia, fever, and splenomegaly. However, his serum triglyceride was 244 mg/dL, which is lower than 265 mg/dL, the criteria needed for HLH. As the patient continued to deteriorate with worsening lactic acidosis (Figure ) despite broad-spectrum antibiotics, thiamine administration, and continuous renal replacement therapy (CRRT), a bone marrow biopsy was performed to evaluate for marrow erythrocytic phagocytosis. He was also started on the HLH treatment protocol, receiving dexamethasone. Cyclosporine was initiated the next day, followed by etoposide. He developed multiorgan failure with persistent encephalopathy, despite discontinuing all sedation, coagulopathy, worsening liver enzymes, and cytopenia. Serum LA remained elevated with a minimum value of 14.9 mmol/L during his seven-day hospital course (Figure ) and arterial blood pH varied from 7.15 to 7.25.
A bone marrow biopsy showed that hemophagocytosis was not identified. The bone marrow contained a monomorphic cellular infiltrate with areas of necrosis and cellular degeneration. Less than 10% of the marrow appeared fully viable and contained a monomorphous infiltrate of cells with irregular nuclear contours and pale cytoplasm. The infiltrate was positive for CD45, CD20, and CD79a, and negative for CD34, with patchy non-specific staining for TdT. The Ki67 (MIB-1) proliferation rate was up to 60%. The absence of CD34 and a convincing TdT expression favored large B-cell lymphoma. The disease was classified as Ann Arbor stage IV due to its presence in the extra-lymphatic organs (liver and bone marrow). The age-adjusted international prognostic index (IPI) score was calculated to be 3, conferring high risk.
Chemotherapy regimen “R-CHOP” for this diagnosis of diffuse large B-cell lymphoma (DLBCL) was planned with a few variations. As rituximab is dialyzed, it was not administered because he required CRRT and vincristine was held due to its hepatotoxicity on already deranged liver enzymes. Steroids were changed to IV methylprednisolone. Doxorubicin was also initiated followed by cyclophosphamide and etoposide while cyclosporine was discontinued. Unfortunately, his condition continued to deteriorate, and he became bradycardic and hypotensive, eventually requiring multiple vasopressors. After discussions with the family, comfort measures were pursued. |
pmc-6029734-1 | An anuric 60-year-old woman with diabetic nephropathy had been treated for four months by continuous ambulatory peritoneal dialysis (CAPD) with four daily exchanges and 2.0 L fill volume. She had a history of coronary artery disease with an anterior myocardial infarction seven months prior to the start of CAPD, followed by severe congestive heart failure with an ejection fraction of 23%. Her dry weight, documented one month after the start of CAPD by absence of both edema and hypertension, was 41.2 kg. Her height was 149 cm. She had not been seen by her CAPD providers for three months because of transportation difficulties when she was admitted with grand mal seizures followed by coma. One week prior to this admission, she communicated by telephone call with one provider. At that time, she reported total body swelling, moderate dyspnea and a body weight exceeding 70 kg. She was advised to use exclusively hypertonic dextrose (4.25%) CAPD dialysate and add two exchanges per day with short (1 hour) dwell times. Information obtained from relatives revealed that in the two days prior to this admission she had omitted her insulin dose because of anorexia and greatly decreased food intake.
On admission, she was comatose with a blood pressure of 180/74 mm Hg, regular pulse rhythm with a rate of 110 beats per minute, and temperature of 36.5 degrees Celsius. Her weight was 63.6 kg. Remarkable findings on physical examination included anasarca detected even in her scalp, a ventricular gallop, and crackles in both lung fields. Chest X-ray confirmed the presence of pulmonary edema and electrocardiogram was unchanged from recent tracings, which were consistent with old transmural myocardial infarction. Laboratory tests on admission revealed extreme hyperglycemia, severe hypertonicity, hypokalemia and respiratory acidosis.
Over the next 30 hours, she received insulin boluses and had frequent (every 2-4 hours) determinations of serum glucose and potassium concentrations. Serum glucose levels decreased progressively, while serum potassium levels remained between 3.1 and 3.4 mmol/L without administration of potassium salts. Blood pressure progressively decreased during treatment. At the end of treatment, she developed frank hypotension (blood pressure 80/60 mm Hg) and increased tachycardia to a pulse rate of 125 mm Hg, while remaining comatose. Chest X-ray revealed worsening pulmonary edema, while electrocardiogram was consistent with a new myocardial infarction and serum levels of cardiac enzymes (creatinine phosphokinase, troponin) were substantially elevated. Subsequently, she developed worsening hypotension and expired within three hours. Table shows laboratory tests at presentation and at the end of insulin treatment. |
pmc-6029735-1 | A 41-year-old woman with no significant past medical history presented to the emergency department with a one-day history of nausea, vomiting, and diarrhea (six loose bowel movements within the previous 24 hours). Five days prior to her presentation, she presented to an urgent care clinic with body aches, chills, and fever. She tested positive for influenza type A and was prescribed Tamiflu. Her husband and several other family members also tested positive for influenza. On admission to the emergency department, she denied chills, fever, abdominal pain, numbness, or paresthesias. Nausea and vomiting were exacerbated by oral intake of food and fluid. She also complained of generalized weakness in her upper and lower extremities bilaterally, as well as shooting pain down the lateral side of her right lower extremity. On physical exam, she was noted to have dry mucous membranes. The rest of her exam was unremarkable. Vital signs on admission were a temperature of 95.5° F, pulse 94/min, respiratory rate 16/min, and blood pressure of 121/58 mmHg. She was started on 0.9% intravenous sodium chloride and Tamiflu. She was also started on ondansetron, prochlorperazine, and diphenhydramine for intractable nausea and vomiting. Her initial labs showed the following: white blood cell count (WBC) 13,200/mm3, hemoglobin (Hb) 21.5 g/dL, hematocrit 67.3%, platelet count 361,000/mm3, sodium 135 mEq/L, potassium 5.8 mEq/L, chloride 96 mEq/L, blood urea nitrogen 16 mg/dL, creatinine 1.1 mg/dL, glucose 169 mg/dL, calcium 8.4 mg/dL, and lactic acid 7.5 mg/dL.
After IV fluid administration, she stated she was starting to feel better, but this was short-lived. She continued to have generalized muscle pain, weakness, and feelings of heaviness in her lower extremities. At this time, there was no evidence of edema. Repeat labs showed continued elevated hemoglobin and hematocrit levels; therefore, phlebotomy was recommended. Hours later, the patient became lethargic and experienced numbness and tingling in her hands and feet. On generalized exam, her extremities appeared purple and cyanotic. She denied pain but once again described pressure in her lower extremities. Her pulses were unable to be obtained by Doppler ultrasound. She was transferred to the Intensive Care Unit where an arterial blood gas (ABG) depicted a pH of 7.148, pO2 146 mmHg, pCO2 25 mmHg, and a base excess of -18. Her albumin level was 2.4 g/dL, and she was started on replacement therapy. Her creatine phosphokinase (CPK) levels and liver function test were within normal limits. Neurology was consulted after episodes of sensory deficits to rule out Guillain-Barre syndrome.
On physical exam, she continued to have generalized weakness but presented with hyperreflexia of her deep tendon reflexes. It was recommended that a lumbar puncture be used to evaluate albuminocytologic dissociation and to possibly administer intravenous immunoglobulin (IVIG). After discussion, it was agreed that Guillain-Barre syndrome was unlikely and that further workup was not necessary. Labs showed no evidence of methemoglobinemia or carboxyhemoglobinemia. Heparin, insulin sliding scale, prochlorperazine, sodium bicarbonate, and IV fluids were administered. Her blood pressure began deteriorating rapidly, so Neo-Synephrine® was administered and the required doses of Neo-Synephrine escalated quickly. Repeat labs showed a continued elevation of the hemoglobin and hematocrit. Her WBC count increased significantly to 42,000/mm3. Labs confirmed that metabolic acidosis due to lactic acid continued to be present. She required intubation due to respiratory failure induced by her metabolic acidosis. She was coherent before intubation, and she only complained of generalized weakness and pressure on her lower extremities. Peripheral access was needed to run more lab work but was impossible due to the collapsibility of her veins. A central line was planned. However, the patient went into asystole and cardiopulmonary resuscitation (CPR) was attempted to resuscitate the patient to no avail. |
pmc-6029736-1 | A 23-year-old woman with a history of adjustment disorder and previously treated anxiety disorder, not otherwise specified, presented to the dermatology clinic with a two-month history of painful, cracked, and peeling lips that had been unresponsive to oral acyclovir or valacyclovir. Examination revealed thick, yellow keratinaceous crusting on the upper and lower lips, sparing the mucosal lip and vermilion border, with associated edema, erosion, and tenderness to palpation (Figure ). Notably, minimal contact with the keratinaceous material and crusted plaque on physical examination resulted in complete sloughing and revealed a moist base and a nearly normal lip underneath.
Consequently, the histopathological evaluation of a 4 mm punch biopsy from the debrided left lower lip was nonspecific, showing only features of chronic lymphocytic mucositis with spongiosis. Staining for fungal organisms using the Grocott's methenamine silver and periodic acid–Schiff methods was negative, and Treponemal/Fite stains were negative for bacterial organisms. In-situ hybridization for human papillomavirus testing (subtypes 6/11, 16/18, 31/33) was also negative. A wound culture from the lower lip was positive for methicillin-sensitive Staphylococcus aureus.
While the biopsy findings were not specific, the histopathologic pattern of spongiotic mucositis, in conjunction with the clinical features, suggested an exuberant irritant contact dermatitis—in this case, further investigation revealed repeated self-injurious behaviors to the lips, supporting a diagnosis of factitial cheilitis. The patient was treated for secondary impetiginization with anti-staphylococcal antibiotics and topical antibiotic ointment for two weeks, counseled on avoidance of licking her lips, and referred back to her behavioral health specialist for management of her unspecified anxiety disorder. Notably, she had previously been treated for anxiety, but was lost to follow-up prior to presentation at our clinic. However, she noted that recent life stressors had caused exacerbation of her anxiety symptoms, which preceded the onset of her lip findings. The patient reported complete resolution of her lip symptoms within one month of psychiatric treatment and cessation of lip licking. |
pmc-6029737-1 | An 87-year-old man with a past medical history of hypertension and hypothyroidism was told by his cardiologist to present to the Emergency Department (ED) after he was found to have abnormal electrocardiogram (EKG) findings on the Holter monitor. He was seen by his primary doctor two days prior to presentation for arm pain and the EKG at that time showed an irregular rhythm for which the patient was referred to a cardiologist who placed a Holter monitor. The patient denied chest pain, palpitations, shortness of breath, or headaches at the time of presentation to the ED. Physical examination findings were as follows: pulse 36 beats/min, respiratory rate 16/minute, blood pressure 180/70 mmHg, percentage oxygen saturation was 96% on room air. EKG showed new-onset atrial fibrillation with slow ventricular response, frequent episodes of bradycardia to less than 40 beats per minute (bpm), and left bundle branch block (LBBB) as shown in Figure .
The patient was not taking any AV nodal blocking agents such as beta blockers, calcium channel blockers, or digoxin that could explain the cause of the slow ventricular rate. Initial labs showed normal pH and an unremarkable basic metabolic panel; complete blood count was within normal limits for age and gender. Thyroid stimulating hormone (TSH) was elevated to 74.03 IU/ml (reference range 0.39-4.08 IU/ml), with free thyroxine (fT4) decreased to 0.53 mg/dl (0.58-1.64 mg/dl). The patient reported that he was on levothyroxine 25 mcg daily for at least three years and he had been taking it on an empty stomach, one hour before breakfast daily. Baseline EKG two years ago showed LBBB with first degree AV block as shown in Figure .
The patient had a CHA2DS2-VASc score of three and was planned to be started on apixaban for prevention of thromboembolism. It was decided to hold off on rate control therapy for atrial fibrillation due to frequent episodes of bradycardia to less than 40 bpm. Echocardiogram showed ejection fraction (EF) of 46%-50%, mild diastolic dysfunction, and increased pericardial fat.
The patient was admitted to the telemetry unit, and the levothyroxine dose was increased to 50 mcg per oral daily. A repeat EKG the next day showed atrial tachycardia with a variable high-grade AV block as shown in Figure .
The patient also reported symptoms of dizziness with minimal exertion, and it was planned to insert a pacemaker for a high-grade AV block and symptomatic bradycardia. However, a subsequent telemetry review showed complete resolution of the high-degree AV block and pauses were no longer seen four days after increasing the dose of levothyroxine. Permanent pacemaker insertion was avoided, and the patient was discharged on levothyroxine 50 mcg daily, apixaban 5 mg twice daily for new-onset atrial fibrillation, and was advised to follow-up with a cardiologist for repeat thyroid function testing in four to six weeks. |
pmc-6029738-1 | A 61-year-old female was admitted to our hospital with a history of worsening cough and phlegm production for four weeks after choking on an iron pill. She had a history of hypertension, anemia, and immunoglobulin deficiency. The chest X-ray was unremarkable. Computed tomography (CT) of the thorax showed ground glass infiltrates in the right lower lobe (Figure ).
She underwent flexible bronchoscopy which showed distal right bronchus intermedius (RBI) necrosis and stenosis with near-complete obstruction of distal RBI (Figure ).
There was blackish pigmentation noted in the bronchial mucosa secondary to the iron deposition given her history of aspirating the iron pill. The pathology of the bronchial mucosa was reported as “ulceration and necrosis of bronchial wall, with acute inflammation, fibrinous exudate and prominent stromal iron deposition” (Figures -).
A rigid bronchoscopy was performed for cryo-debridement of necrotic tissues and with mitomycin application to the lesion. She had two follow-up bronchoscopies done four weeks apart which showed worsening fibrosis and stenosis of the RBI. Bronchoscopic balloon dilation was attempted with minimal improvement (Figures -). |
pmc-6029742-1 | A 25-year-old man with no past medical history presented to the emergency department (ED) with two days of intractable vomiting, increasing confusion, and progressive difficulty breathing. The patient denied tobacco or recreational drug use.
Upon arrival to the ED, vital signs were within normal range with a blood pressure of 123/61 mmHg, a temperature of 36.8 degree Celsius, a heart rate of 92 beats per minute, and a respiratory rate of 19 respirations per minute. The physical exam showed an obese, lethargic patient, responsive to verbal stimuli. The lung, heart, and abdomen exam were unremarkable and there was no neck crepitus noted. The laboratory evaluation showed leukocytosis of 25.96 x 1000/mm3, elevated creatinine of 2.17 mg/dL, hyperglycemia of 836 mg/dL, hyponatremia of 128 mEq/L, hyperkalemia of 5.2 mEq/L, and bicarbonate of 5 mEq/L. The anion gap was 25 and a venous blood gas showed a pH of 6.91. Urinalysis was positive for 2+ ketones and 1+ protein.
The patient was admitted to the intensive care unit with a new diagnosis of diabetes mellitus complicated with DKA. Intravenous normal saline, bicarbonate, and insulin drip were initiated. The chest X-ray on admission suggested findings compatible with pneumomediastinum (Figure ). Chest computed tomography (CT) was obtained and reaffirmed the findings (Figures -). Pneumomediastinum in the setting of intractable vomiting was worrisome for esophageal rupture and since our facility was not staffed to manage this clinical entity, the patient was emergently transferred to another facility. The patient was treated conservatively with analgesia and respiratory support and was discharged several days later without any complications. |
pmc-6029743-1 | A 21-year-old male had presented to the emergency with the history of one episode of generalized tonic-clonic seizures followed by altered sensorium and repeated episodes of vomiting for one day. His parents also noticed a paucity of limb movements on the right side. Past history revealed that he had sustained a minor head injury following a trivial road traffic accident two months ago, following which he was stable except for an occasional headache. A computed tomography (CT) scan brain was done two weeks after the initial trauma which revealed an incidental left Sylvian Type II arachnoid cyst (Figure ).
He was kept on observation with symptomatic treatment for his headache until the time he presented with seizures and altered sensorium. On admission, his Glasgow coma scale (GCS) was seven (E1V1M5). His pulse rate was 56 bpm and he had features of left uncal herniation. An emergency CT of the brain was done which revealed an acute on CSDH on the left side with bleed into the cyst cavity and a significant midline shift (Figure ).
Emergency surgery was performed and dark machine oil coloured fluid was evacuated through two burr holes. He showed good improvement neurologically post evacuation with complete hematoma evacuation and significant improvement of midline shift on follow up scan (Figure ). His post-operative period was complicated with cerebrospinal fluid leak from the parietal burr hole on suture removal followed by a pulsatile swelling in the frontal burr hole, which was managed conservatively with acetazolamide and pressure dressing of the wound. The swelling spontaneously disappeared in a week and the patient has been asymptomatic since then. |
pmc-6029744-1 | A 25-year-old man, with no known comorbidities but with a known history of intravenous drug abuse (heroin) from the age of 18, presented with a concern of fever and sore throat lasting two days and an altered level of consciousness lasting one day. According to the patient’s attendant, he was in his usual state of health two days prior when he developed a high-grade fever associated with a sore throat. Furthermore, patient’s attendant mentioned that he was found in an altered state of consciousness along with fecal and urinary incontinence. The patient had no history of trauma to the head as well as no positive history of seizures. There was no significant past medical or surgical history. The patient’s addiction history was positive for heroin, Hashish, and methamphetamine both orally as well as intravenously. On examination, his vital signs on arrival were as follows: blood pressure, 110/60 mmHg (reference, 120/80 mmHg); pulse, 100 bpm (reference, 70 to 100 bpm); respiratory rate, 30 breaths/minute (reference range, 18 to 22); and temperature, 102°F (reference, 98.6°F). Furthermore, in his general physical exam, he was positive for jaundice and had marks of cuts on his forearm, suggesting the possibility of multiple needle insertions. His abdominal, respiratory, and cardiovascular exam results were unremarkable. His Glasgow coma scale (GCS) on arrival was 10/15. He had dilated pupils reactive to light, as well as a positive corneal reflex. All cranial nerves were intact; however, signs of meningeal irritation were present. A motor examination showed an increased tone in all limbs, positive rigidity in all four limbs, his patellar reflex was 3+, his plantars were down-going, and clonus (ankle and knee) was negative. Furthermore, the results of the fundoscopic examination were unremarkable.
We immediately did clinical laboratory testing of this patient. His laboratory test results are presented in Table . Given our region, we also tested for malaria and found the patient was positive for Plasmodium falciparum. Given the patient’s intravenous drug abuse, we also tested his viral markers and HIV status, the results of which were positive for both hepatitis B and C and HIV, with a CD4 count of 270 (reference range, 500 to 1500). Immediately, the patient was catheterized with monitoring of urine output, and a nasogastric tube was passed. The patient was started on artemether, 120 mg, delivered in six doses spaced 12 hours apart, and intravenous ceftriaxone 2 g/day for five days. The patient had low random blood glucose levels of 60 mg/dL (reference range, 79 to 160 mg/dL), for which he was started on 5% dextrose saline intravenously as well. At this point, we were suspicious of many differentials such as cerebral malaria, bacterial/tuberculous/fungal meningitis, cytomegalovirus/herpes simplex virus encephalitis, drug overdose, central nervous system (CNS) lymphoma, as well as toxoplasmosis. To arrive at a final diagnosis, we conducted a series of additional investigations. We ordered a chest X-ray and ultrasound of the abdomen, the findings of which were unremarkable. To rule out meningitis, we did a lumbar puncture, and his cerebrospinal fluid (CSF) findings are presented in Table . At the time of the lumbar puncture, his random blood glucose level was 102 mg/dL (reference range, 79 to 160 mg/dL). Results of the tests were negative for Cryptococcus antigen and tuberculosis. A computed tomography (CT) scan and magnetic resonance image (MRI) of the brain were unremarkable, thereby ruling out ring-enhancing lesions due to tuberculoma, CNS lymphoma or toxoplasmosis. To rule out infective endocarditis, we tested blood cultures and conducted an echocardiographic exam, the results of which were both unremarkable. We also ordered a urine toxicology test given the patient’s status as a drug addict. The urine toxicology results were negative for any drug intoxication. By this point in our investigation, we became firm in our belief that cerebral malaria was the correct diagnosis.
However, after 10 days of treatment, the patient showed considerable improvement despite being very critical in condition. He was no longer in an altered level of consciousness, and his fever had subsided. Furthermore, his GCS improved to 15/15, and his neurological examination results were normal. His laboratory findings had also improved. The patient was discharged and referred to an HIV clinic and a hepatitis outpatient department for his viral infections; he was also referred to a rehabilitation program for his addiction. |
pmc-6029745-1 | A 34-year-old Caucasian female presented to the emergency department with complaints of right hemiparesis, numbness, dysphagia, and ataxia. These complaints began as numbness over her right scapular area two weeks prior, and the numbness later progressed to include the right leg. Additionally, she reported five days of waking up during the night with severe headaches. Although she reported a history of migraines, which were usually accompanied by an aura, she stated that these new headaches were different in nature. She also had no known allergies and no previous surgeries. Her mother had a history of breast cancer, and her father had a history of ischemic heart disease. A review of systems was otherwise negative. After workup in the emergency department and consultation by neurology, she was referred to neurosurgery for management of a high-grade brainstem tumor.
Examination
On initial presentation, the patient was alert and oriented to person, place, and time with a Glasgow Coma Scale (GCS) score of 15. Her pupils were equal, round, and reactive to light. Cranial nerves II-XII were grossly intact. Motor testing revealed that strength was 5/5 in both upper and lower extremities. Pronator drift was noted in the right upper extremity. Dysmetria was noted in the right upper extremity during finger-to-nose testing, and discrimination of fine touch was subjectively diminished on the entire right side from the zygomatic process to the foot. Patellar reflexes were 3+ bilaterally. Over the course of her initial admission, the patient demonstrated a progressive decline, to include mild left facial droop, dysarthria, and a worsening dysphagia. A percutaneous endoscopic gastrostomy (PEG) tube was placed because the patient was unable to swallow without effort. These findings continued until her initial discharge. Upon readmission, the patient displayed similar findings. Left lower quadrant abdominal pain radiating to the shoulder and acute numbness of the left chest and shoulder were also noted. These findings progressed and worsened until the patient was intubated after deteriorating to a GCS score of 10.
Imaging
Magnetic resonance imaging (MRI) of the brain with and without contrast revealed a heterogeneous T1 hypointense (Figure ) and T2 hyperintense (Figure ) signal abnormality involving the pontine base and extending into the medulla. There was mildly increased relative cerebral blood flow and blood volume within the enhancing portion of the abnormality, suggesting neovascularity. The pontine component of the lesion also showed no abnormal enhancement or significant hyperperfusion. Mild mass effect on the fourth ventricle was noted. There was no peritumoral edema or significant mass effect. The remainder of the brain revealed no abnormal enhancement. No abnormal leptomeningeal enhancement was observed. A magnetic resonance imaging (MRI) scan of the spinal column with and without contrast also demonstrated no evidence for metastasis to the cervical, thoracic, or lumbar spine, with normal caliber and signal intensity of the spinal cord. A computed tomography (CT) scan of the chest with (Figure ) and without (Figure ) contrast enhancement revealed scattered small ground glass and nodular opacities bilaterally. These were nonspecific, with differentials including inflammatory and infectious etiologies, although metastatic foci remained difficult to completely exclude in the context of malignancy. A CT of the abdomen and pelvis with contrast revealed no intra-abdominal mass or evidence of metastatic disease.
Differential diagnosis
- High-grade glioma
- Primary central nervous system (CNS) lymphoma
- High-grade medulloblastoma
Management
The patient was initially started on dexamethasone 4 mg orally every six hours until imaging was reviewed. Dexamethasone was discontinued due to a concern for possible lymphoma, but shortly thereafter the patient began to complain of worsening ataxia, dysarthria, and dysphagia. Dexamethasone was subsequently resumed.
Operation
The patient was transported to the operating room on Day 5 of her hospitalization to undergo a closed stereotactic needle biopsy. Once the patient was anesthesized, the head was secured and the fiducials on the scalp were registered by cameras into the computerized stealth CT neuronavigation system in the operating room. A minimal amount of hair was shaved from the scalp and a small incision was marked out. This area was then meticulously cleaned and draped in a sterile fashion. An opening in the skull about the size of a quarter was made (burr hole), exposing the dura which was then opened. A stereotactic biopsy needle was then introduced with intraoperative use of the neuronavigation system in order to guide the needle to the target using a contralateral approach (Figure ). Biopsy samples were successfully obtained for pathologic examination. After the incision was closed, a clean and dry dressing was applied. The patient was then extubated in the operating room and transported to the post-anesthesia care unit in stable condition.
Postoperative course
Postoperatively, a nasogastric tube was placed to provide nutrition, due to worsening dysphagia. On Day 10 of her hospitalization, a PEG tube was placed. The patient was scheduled for chemotherapy and craniospinal radiation therapy, and was discharged on Day 12. Four days later, the patient returned due to worsening symptoms, and dexamethasone was increased to 10 mg orally every six hours. The patient was started on temozolomide IV (intravenous) on Day 10 of readmission, but she developed respiratory acidosis/hypercapnia with a CO2 of 115 and was started on a bilevel positive airway pressure (BIPAP), two days later. As a result, a rapid response team was called to transfer the patient to the intensive care unit (ICU). The temozolomide was converted to oral dosing via a PEG tube. The patient’s family was consulted regarding the patient's status and their options. The patient chose to update her status to do not intubate. The patient was extubated prior to her discharge to a hospice care facility, where she passed away four days later.
Pathologic findings
The brain biopsy was originally interpreted as a classic medulloblastoma (WHO Grade 4). The tumor was composed of sheets of small blue cells with rare poorly formed rosettes (Figure -). An immunohistochemical stain for glial fibrillary acidic protein (GFAP) and synaptophysin revealed moderate astrocytosis (Figure ) and little synapse loss (Figure ), respectively. Reticulin staining was also negative. Staining for p53 was positive, and Ki-67 index was about 50%. The sample was then sent to St. Jude’s Children’s Research Hospital in Nashville, TN, for further molecular analysis. Although their differential diagnosis also included medulloblastoma, the diagnosis was amended at St. Jude’s Children’s Research Hospital to small cell glioblastoma (WHO Grade 4). Their analysis indicated only focal immunoreactivity for GFAP and weak immunoreactivity for synaptophysin. However, immunoreactivity for Olig-2 and p53 was present in a majority of the tumor cells. Interphase fluorescence in situ hybridization (iFISH) analysis revealed platelet-derived growth factor receptor A (PDGFRA) amplification. No amplification of MYC, NMYC, or epidermal growth factor receptor (EGFR) was observed. |
pmc-6029746-1 | A 34-year-old female with a past medical history of drug abuse was brought into the emergency department (ED) after a motor vehicle collision; the patient was driving a jeep. The patient was confused in the ED. Initial imaging showed a closed distal right tibial fracture, open distal right fibular fracture, and a Grade 3 open right talus and calcaneus fracture. A computed tomography (CT) scan of the chest showed large hypodense mass within the left atrium of the heart (Figure ).
The patient was emergently taken to the operation theater for an open fracture repair. Initially, intramedullary nailing of the right tibial shaft fracture and incision and debridement of the open right fibular shaft, calcaneus, and talus fractures were done. Multiplanar right ankle spanning external fixation was applied. Wound vacuum-assisted closure (VAC) was also applied to the open wound. The patient subsequently underwent multiple debridements and was placed on broad-spectrum antibiotics but her wound got worse. After a detailed discussion with patient and family, it was decided to proceed with below-the-knee amputation. The patient did develop a wound infection after amputation and continued on broad-spectrum antibiotics.
For the left atrial mass, cardiology was consulted. The patient underwent transthoracic and transesophageal echocardiograms that confirmed the left atrial mass was consistent with a myxoma (Figures -).
Cardiovascular surgery was consulted for the resection of the left atrial myxoma. The patient underwent an uncomplicated resection of the left atrial mass. Pathology findings were consistent with a myxoma. The patient was discharged with intravenous antibiotics for the left knee wound. |
pmc-6029748-1 | A 52-year-old man was admitted to the surgery clinic with a two-day history of mild abdominal pain radiating to the back, yellowing sclera, and dark-coloured urine. He had undergone a Billroth II procedure over 20 years ago for a distal gastric ulcer. Physical examination revealed a midline abdominal incision scar and mild tenderness in the right upper quadrant. Blood test results showed total bilirubin of 5 mg/dl and an unconjugated bilirubin of 3 mg/dl. The serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were two times higher than normal. Ultrasonography revealed multiple subcentimetric gallstones confined to the thin-walled gallbladder with dilated intrahepatic ducts, 12-mm calibrated choledocus, and other organs were normal. Magnetic resonance cholangiopancreatography revealed a semi-obstructing gallstone 13-mm in diameter in the choledocus. Owing to his history of a Billroth II procedure, performing an endoscopic retrograde cholangiopancreatography (ERCP) for gallstone in the choledocus was not possible. Because of this reason, a surgical decision was taken. After a right subcostal incision, the gallbladder and the fatty tissues surrounding the choledocus were found to be oedematous. However, a cholecystectomy was performed without any complication, followed by choledocus exploration and stone extraction with a T-tube insertion. A latex, 12-foot T-tube was used in the operation. The horizontal branch of the T-tube was shortened, and the T-tube was incised to form a gutter, with a V-notch added at the junction of the two arms. The patient made an uneventful postoperative recovery and was discharged on postoperative day 5 with the T-tube spigotted and left in situ.
The patient's bilirubin levels done twice in six weeks were normal, and he had a problem-free recovery. T-tube cholangiogram performed after six weeks did not reveal bile duct stricture or leak. The T-tube was removed without any difficulty. The patient was discharged after six hours of uneventful observation. However, a day after the T-tube removal, the patient presented to the emergency clinic with complaints of fever, vomiting, and severe pain in the right upper quadrant. He was hypotensive (80/60 mmHg), had tachypnea (18 counts/min), and tachycardia (112 bpm). He had mild abdominal tenderness. An increase in the C-reactive protein (CRP) level and white blood cell count were observed in the blood test results. An abdominal ultrasonography showed the presence of perihepatic fluid. Fine needle aspiration revealed that the perihepatic fluid was bilious. An 8F drainage catheter was introduced into the fluid, and a 14F drainage catheter was inserted into the choledocus. Cholangiography detected a bile leak from the choledocus. The tract of the leak was embolized with eight coils during angiography (Video ). After filling the tract with multiple coils and percutaneous drainage of the perihepatic fluid, the patient’s symptoms subsided, and his test results were found to be normal. The T-tube was removed on the fifth day of coiling and the percutaneous catheter was removed after a control ultrasonography on the sixth day of coiling. No perihepatic or abdominal liquid was detected on control ultrasonography on day 14 after removal of the percutaneous catheter. The patient showed no further complications at a three-month follow-up. |
pmc-6029749-1 | A previously healthy, 24-year-old female presented to the emergency department (ED) with a one-day history of shortness of breath (SOB) and left-sided pleuritic chest pain. She complained experiencing progressively worsening dyspnea with physical activity. SOB was associated with left-sided chest pain described as stabbing in character, Th pain rated 6/10 in intensity on the pain scale and was non-radiating. No pertinent medical, surgical, or social history was noted. She denied a recent history of trauma. The patient was on the second day of her menstrual cycle. In the ED, her vitals showed a blood pressure of 125/70 mmHg, a heart rate of 102 beats/minute and a respiratory rate of 19 breaths/min. She was afebrile and was maintaining an oxygen saturation of 99% on room air. On physical examination, she had diminished breath sounds on the left side. Chest x-ray (CXR) showed a moderate-sized left PTX with a 2.6 cm gap but without a mediastinal shift (Figure ).
Needle aspiration with the placement of a left-sided pigtail catheter was performed immediately. Blood work and basic metabolic chemistry were within the normal limits. Venous blood gas was grossly normal with the exception of lactic acidosis (2.8 mmol/L). For her pain, the patient received analgesics and symptomatic improvement was noted. A repeat CXR showed a decrease in the size of the PTX from 2.6 cm to 1.9 cm. A consecutive CXR over the next two days showed significant improvement. A complete resolution of PTX was noted eventually. Consultants from pulmonary and obstetrics/gynecology confirmed the clinical diagnosis of catamenial-pneumothorax possibly associated with thoracic endometriosis. She was eventually discharged on day five of hospitalization on an oral contraceptive medication containing ethinyl estradiol and levonorgestrel. |
pmc-6029867-1 | A 55-year-old woman with history of T2DM on canagliflozin 300 mg daily developed nausea, vomiting, and polyuria over 24 hours. Vital signs showed tachycardia and hypotension (). On examination, she had dry mucous membranes and epigastric abdominal pain. Lab values revealed acidosis with an anion gap of 32 mmol/L, and acute kidney injury (). Treating clinicians diagnosed DKA based upon serum beta hydroxybutyrate (BHB) concentration of 12.43 mmol/L although her serum glucose concentration was 366 mg/dL (20.3 mmol/L). Her lipase concentration of 165 U/L suggested acute pancreatitis. She received continuous insulin infusion and intravenous normal saline resuscitation. In the intensive care unit, her anion gap normalized. She was transitioned to metformin and glipizide and discharged home on hospital day 3. |
pmc-6029867-2 | A 54-year-old man with type 1 diabetes mellitus (T1DM) on insulin (humalog 25/35 units subcutaneous every AM/PM), albiglutide 50 mg, and canagliflozin 300 mg PO daily developed abdominal pain, nausea, and vomiting. Vital signs showed tachycardia and tachypnea. His examination was significant for dry mucous membranes and epigastric tenderness. Laboratory analysis revealed acidosis with an elevated anion gap of 27 mmol/L (). A BHB concentration of 15.35 mmol/L with a glucose concentration of 327 mg/dL (18.2 mmol/L) led to a diagnosis of DKA. He underwent volume resuscitation and an intravenous insulin infusion until the anion gap had normalized. The patient improved and returned home after 3 days of hospitalization. |
pmc-6029987-1 | The patient was a 76-year-old woman with rheumatoid arthritis. Computed tomography (CT) had been performed for a medical checkup, and a small nodule was detected in the left upper lobe (S1+2) a year before she visited our hospital. Follow-up CT showed that the nodule with indentation was growing to 22 mm in size, and she was referred to a nearby hospital (Fig. ). Fluorodeoxyglucose (FDG)-positron emission tomography (PET), brain magnetic resonance imaging, and transbronchial biopsy were performed, showing left upper lung adenocarcinoma classified as cT1bN0M0 Stage IA according to the Union for International Cancer Control classification (seventh edition). Though contrast-enhanced three-dimensional computed tomographic angiography (3DCT) of the pulmonary vessels was usually performed to identify the pulmonary branches preoperatively at that time, it was not performed in the present case due to renal dysfunction, and abnormal branching of the left pulmonary artery was not identified. Video-assisted thoracoscopic left upper lobectomy and lymphadenectomy were performed. A1+2c, the upper pulmonary vein, mediastinal A4+5, A3, and A1+2a+b were detached carefully and dissected. Before separation of the interlobar fissure was completed, a pulmonary mediastinal branch from the left main pulmonary artery was identified descending between the upper pulmonary vein and upper bronchus. The interlobar fissure was separated safely with preservation of A8 (Figs. and ). On retrospective examination, non-contrast CT showed A8 (Fig. ). |
pmc-6029993-1 | A 70-year-old woman visited another clinic with loss of body weight and mucous diarrhea. She had no family history and no characteristic travel history. She underwent total colonoscopy, which showed erythematous polypoid lesions from the lower rectum to the sigmoid colon (Fig. ). Biopsy was taken from several typical polyps. Histopathological examination of biopsy specimens revealed hyperplastic polyps or tubular adenomas with low-grade atypia (Fig. ). She was not judged to be an appropriate case for complete endoscopic polypectomy because of multiple polypoid lesions. She underwent gastroscopy, where slight atrophic gastritis with erythematous edema was confirmed. She was not diagnosed with Helicobacter pylori (H. pylori) infection by rapid urease tests and histopathological findings.
6 months later, the patient returned to clinic with a worse complaint of repeated mucous defecation (5–6 times/day) and bilateral leg edema. Second colonoscopy revealed no remarkable changes of polypoid lesions with purulent mucus. Biopsy specimens showed tubular adenomas which contained moderate atypia. Laboratory tests showed hypoproteinemia (serum total protein 6.0 g/dl; normal range 6.6–8.1 g/dl) and hypoalbuminemia (serum albumin 3.3 g/dl; normal range 4.1–5.1 g/dl) while white blood cell count (5800/mm3) and C-reactive protein levels (0.28 mg/dl) were not elevated. Other laboratory data were unremarkable. The patient sometimes developed tenesmus and mucous bloody diarrhea, and visited our hospital for detailed treatments.
Considering the overall course of examination and taking into account the patient’s strong request for surgery, we judged she was not suffering from inflammatory bowel diseases or hereditary/non-hereditary polyposis coli and diagnosed protein-losing enteropathy which could not be denied malignant potential. Therefore, we performed laparoscopic low anterior resection of the rectum and the sigmoid colon with diverting ileostomy. During operation, we found thick subserosal layer with moderate fibrosis and edematous change. Anal surgical margin was located 3 cm above the dentate line. Surgical specimen revealed more than 100 polyps consisting of elongated and mild adenomatous glands with a thick layer of subserosal tissue (Fig. ). Polyps were covered with a layer of inflammatory granulation tissues and fibrinopurulent exudate. The intervening mucosa of the polyps was relatively normal, so the patient was diagnosed with cap polyposis (Fig. ). After surgery, symptoms improved, including return to normal range of serum total protein and albumin levels. 6 months later, she had no complaints or recurrence of cap polyposis in the remnant colon. We will continue a tight follow-up surveillance. |
pmc-6030249-1 | A 73-year-old male patient was admitted to the Department of Neurology due to tremors of the head, neck, and upper limbs, and moderate tremor of lower limbs. Initial symptoms appeared 20 years prior, primarily as a kinetic tremor of the left upper and lower limbs. During a neurological examination, the patient additionally presented dysarthria, paresis of the right facial nerve, brisk reflexes on the right side, bilateral dysmetria, dysdiadochokinesis, truncal ataxia, ataxia of lower limbs which was more prominent on the left side, and unstable gait. Psychological examination exposed mild cognitive impairment and deficits of executive functions. An MRI in 2016 revealed bilateral hyperintensity in the MCP and white matter of cerebellar hemispheres (Figure ).
The combination of intention tremor, ataxia, the MCP sign, and gait instability of patient's son and daughter instigated diagnosis of FXTAS. Use of the Amplide X FMR1 PCR kit (Asuragen) for extended molecular examination revealed permutations in the FMR1 gene (between 60 and 85 ± 5 repeats of CGG), and confirmed the diagnosis of FXTAS with expansion of CGG repeats in the FMR1 gene.
Due to insufficient effects of pharmacological treatments (primidone and propranolol) to treat the tremors, radiosurgical thalamotomy using GK on the VIM of the left thalamus was performed. The Leksell Model G stereotactic coordinate frame (Elekta AB, Sweden) was attached to the patient's head. High-resolution MRI were acquired using a 1.5T scanner, and contrast-enhanced T1 and T2 images taken at 1 mm. The anterior commissure (AC), the posterior commissure (PC), and the third ventricle were identified (Figure ).
Stereotactic radiosurgical thalamotomy was performed using the Leksell Gamma Knife Perfexion model (Elekta AB, Sweden). The target was localized to the VIM nucleus of the left thalamus. Final isocenter coordinates were selected by means of GammaPlan Software (Elekta AB, Sweden), and were set in the following manner: (x coordinate) 16 mm lateral to the AC-PC line which constituted 50% of the width of the III ventricle plus 11 mm; (y coordinate) 8 mm anterior to the PC; and (z coordinate) 3 mm superior to the AC-PC line.
Final optimization of the x coordinate was safely adjusted in order not to exceed the 18 Gy dose in the posterior limb of the internal capsule. A maximum dose of 130 Gy was delivered with a single, fully opened 4-mm collimator. Control examination was done 6 months after GK thalamotomy. Improvement was observed 4 months after the procedure.
Comparison of tremor intensity before and after the procedure was done using the FTRS and the Tremor Rating Scale (TRS) (Table ). The case presented in this study showed improvement in both rating scales (FTRS 54 – before and 44 – after, TRS – 28 – before and 14,5 after). The improvement was observed in the right side corresponding to the lesion (Videos , ). This represents the most significant change in the context of the head tremor, postural tremor of the upper extremity and daily activities. Neurological examination showed that features such as dysarthria, central paresis of right facial nerve, bilateral dysmetria, dysdiadochokinesis, lower limb ataxia remained unchanged or insignificantly changed.
Psychological examination did not present any relevant changes. Physiotherapist's examination revealed certain changes in the period before (2016) and after thalamotomy (2017). In the timed up and go (TUG) test before GK the patient did not present any abnormalities. After GK thalamotomy TUG showed dynamic imbalance. Examination also demonstrated static imbalance after GK thalamotomy, which was not observed before the procedure. Deterioration in certain features of the examination performed by the physiotherapist were associated with the progressive nature of FXTAS. This could also be observed during MRI, in the form of an increased signal in T2-weighted image within both hemispheres of cerebellum and bilaterally within middle cerebellar pedunculi, rather than side effects of GK thalamotomy (). Approval of the Ethical Committee was not necessary for preparation of this article, as this work is a case study. |
pmc-6030255-1 | An 11-year-old boy with Alagille syndrome received a related liver allograft during first year of life. Being EBV-negative at transplantation, seroconversion occurred 2 years later. Initial immunosuppression was based on tacrolimus, followed by a combination with mycophenolate mofetil. Ten years after transplantation, he suffered from severe headache, nausea, vomiting, and phono-/photophobia without B symptoms. Funduscopic examination revealed bilateral papilledema. Magnetic resonance imaging (MRI) studies of the brain demonstrated multifocal lesions in the left hemisphere (Figure A). After initial treatment for suspected toxoplasmosis, biopsy of the lesion revealed a monomorphic EBV-associated PTLD with features of a diffuse large B-cell lymphoma without MYC translocation (Figure B). Immunohistochemistry showed expression of CD20 and CD30. Most lymphoma cells expressed EBERs (Epstein–Barr encoded RNAs), LMP1 (EBV latent membrane protein 1), and LMP2a while EBNA2 (Epstein–Barr nuclear antigen 2) and BZLF1 (EBV immediate-early protein) were detected in a low number of neoplastic cells (Figure C). EBV PCR was negative in cerebrospinal fluid and weakly positive in peripheral blood (<1,000 copies/ml). Therefore, the diagnosis of EBV-related primary CNS PTLD was made.
Total body imaging and bone marrow aspirate histology displayed no evidence for systemic disease. During initial treatment with dexamethasone, symptoms rapidly improved. Immunosuppression was stopped and immune-/chemotherapy was initiated with six doses of intravenous (i.v.) rituximab (375 mg/m2) and weekly intrathecal (i.th.) therapy with rituximab (40 mg), methotrexate (12 mg), cytarabine (30 mg), and prednisone (10 mg) over 10 weeks (). A partial response by MRI was observed after 3 weeks evolving to complete remission at the end of immuno-/chemotherapy. Due to poor prognosis and the lack of EBV-specific T cells in the patient’s peripheral blood, we decided to consolidate treatment by transfer of partially HLA-matched EBV-CTLs. |
pmc-6030785-1 | A 37-year-old female was referred to our hospital for anal mass prolapse accompanied by bloody stools. Her symptoms started 3 years ago and continued until the time at which she was examined for this report. The patient had no abdominal pain, diarrhea, or weight loss. Prolapsus of the anus and rectum was detected by digital rectal examination. According to these findings, the patient was diagnosed with hemorrhoids. During the digital rectal examination, a mass of approximately 1.5 cm × 1.5 cm under the rectum mucosa 8 cm from the anus was also detected. |
pmc-6030797-1 | An 18-year-old Japanese woman, with a history of intestinal Behçet’s disease, complained of abdominal pain on the day she was brought to our hospital. She took prescription colchicine at a dose of 15 mg (30 tablets 0.5 mg each), which is equivalent to 0.2 mg/kg. As her condition did not improve, she was brought to the emergency department. She had a past medical history of fibromyalgia, in addition to intestinal Behçet’s disease. Prior to admission, she was taking Neurotropin® (non-protein extract isolated from the inflamed skin of rabbits inoculated with vaccinia virus), pregabalin, butylscopolamine bromide, Lactomin (lactic acid bacteria – Lactobacillus acidophilus, Bifidobacterium longum), and colchicine. She neither smoked tobacco nor drank alcohol. Her social and environmental history was unremarkable. She had never worked. Her mother had schizophrenia.
Her vital signs on arrival included blood pressure (BP) of 128/90 mmHg, pulse of 102 beats per minute, regular respiration rate of 18 breaths per minute, blood oxygen saturation (SpO2) of 98% room air, and body temperature of 37.5 °C; she was alert and conscious. She had upper abdominal tenderness with no rebound tenderness. The results of her cardiac, pulmonary, and neurological examinations were unremarkable. Complete blood count, renal function tests, urine analysis, and bacteria tests were normal except for mild hepatic dysfunction and elevated d-dimer levels (Table ). Although colchicine was administered below the lethal dose, she was admitted for observation.
On the day after the hospital admission, she developed acute respiratory distress syndrome (ARDS), thus, tracheal intubation using an artificial ventilator was performed. She also became dehydrated, due to diarrhea, which developed after admission. Peripheral circulatory insufficiency gradually worsened, and large amounts of infusion loads (including blood transfusions) were unable to maintain her BP. Her lactic acid level reached a maximum of 19 mmol/L. At maximum disease severity, she required noradrenaline 1 μg/kg/minute, vasopressin 2 units/hour, steroids 200 mg/day, dopamine 10 μg/kg/minute, dobutamine 4 μg/kg/minute, and adrenaline 0.15 μg/kg/minute to maintain BP. She also showed other organ failures, including worsening hepatic dysfunction after admission with maximum aspartate aminotransferase (AST) level of 16,520 U/L as well as kidney dysfunction with associated anuria. Continuous renal replacement therapy was initiated for anuria and metabolic acidosis. At 36 hours post-admission, her lactic acid level reached its peak, and her hemodynamic reached a level at which she could tolerate water removal. At 72 hours post-admission, she was administered with granulocyte colony-stimulating factor due to the appearance of myelosuppression. In addition to the broad-spectrum antimicrobial administration, she was commenced on a course of antimycotic medication. Table shows the laboratory results of organs function over time.
Approximately 1 week after admission, her BP could be maintained without the use of vasopressor drugs; however, as she showed poor alert wakefulness, a head computed tomography was performed. The results showed multiple intracranial hemorrhages of up to 12 mm in diameter (Fig. ), thought to be associated with disseminated intravascular coagulation (DIC). As the disease course progressed, the hematomas were absorbed and our patient was able to respond to verbal commands. Approximately 2 weeks post-admission, she presented with marked hair loss.
Subsequently, her general condition improved and, at discharge, all organ insufficiency had improved. The progress of her Sequential Organ Failure Assessment (SOFA) scores and D-dimer levels are shown in Fig. . She worked hard at rehabilitation. By 6 months after discharge, she had no disorders and had resumed normal life as before her hospitalization. Table shows the blood tests at follow-up. |
pmc-6030946-1 | A 41-year-old woman, 21-week pregnant, presented to the Emergency Department with left-flank pain, hypotension (100/60 mmHg) and tachycardia (95 bpm). Blood tests were: haemoglobin 7.3 g/dL, leukocytosis 23.4 cell × 109/L, lactate 3.6 mmol/L, base excess −8.4 mol/L and serum creatinine 82 mmol/L. Abdominal ultrasound was normal but foetal monitoring demonstrated a drop in heart rate suggestive for impending demise. Placental abruption was suspected and following resuscitation she was brought to theatre. We found a large left-sided retroperitoneal haematoma and a non-viable foetus. Exploration also revealed a ruptured left RAA and a contralateral RAA. At this point, it was felt a percutaneous approach would have been more appropriate. Contrast-enhanced computed tomography showed a massive retroperitoneal haematoma, a ruptured left RAA (Fig. ), and an intact right-sided RAA measuring 2.2 cm (Fig. ). Under selective angiography, the aneurysm was embolized and the bleeding controlled []. Recovery was rapid but a DMSA scan performed 2 weeks later, demonstrated reduced function in the treated kidney (37%). The risk of rupture of the right RAA was deemed significant and a plan for repair was made. The lesion was saccular, wide-necked and located at the artery bifurcation thus preventing endovascular treatment. We opted for hand-assisted retroperitoneoscopic nephrectomy, ex-vivo repair and autotransplant.
The patient was placed in left lateral decubitus. An 8-cm-long suprapubic incision extended to the right iliac fossa was performed and the retroperitoneum was entered. A hand-port (GelPort® Laparoscopic System, Applied Medical, USA) and three 12-mm ports, one for the 30° camera and the others for the instruments were inserted. Ureter, renal artery and renal vein were divided. The kidney was extracted through the incision, flushed and immerged into a cold solution (Soltran, Baxter Healthcare, USA). The aneurysm was resected and the remaining two arteries were prepared for implantation (Fig ). The patient was placed supine and the hand-port access was used for the autotransplant. The renal vein was anastomosed to the external iliac vein whereas the renal arteries were anastomosed to the external iliac artery. The ureteral-vesical anastomosis was performed according to Lich-Gregoire. The procedure took 481 min. Extraction, cold ischaemia and anastomosis times were 2, 48 and 52 min, respectively. Intra-operative blood loss was 280 mL. The postoperative course was uneventful. Histology showed myxoid medial degeneration of the renal artery. Three years later, her serum creatinine is 79 mmol/L with no RAA recurrence. She also completed two uncomplicated pregnancies. |
pmc-6031073-1 | A 57-year-old African American male with past medical history of coronary artery disease, systolic heart failure, alcohol abuse, and stage-IV sarcoidosis on long-term oral prednisone at 10 mg daily for over two years presented with massive hematemesis and melena that started the night before his arrival to the Emergency Department. Upon physical examination, the patient appeared pale and was noted to have tachycardia, hypotension, and hematochezia. He denied any recent alcohol intake, chest pain, abdominal pain, and abdominal or rectal trauma. Vital signs consisted of a blood pressure of 84/33 mmHg, heart rate of 118 bpm, room air saturation of 92%, and temperature of 98.8 degrees Fahrenheit (37.1 degrees Celsius). He was admitted to the medical ICU for hypovolemic shock secondary to massive bleeding that required immediate endotracheal intubation for airway protection. An initial blood work showed a hemoglobin count of 13.9 g/dL, white blood cell count of 18.7 K/UL, platelet count of 159 K/UL, sodium of 140 mmol/L, potassium of 3.8 mmol/L, chloride of 101 mmol/L, bicarbonate below 10 mmol/L, calcium of 2.2 mmol/L, albumin of 2.7 g/dL, CRP of 233 mg/L, ESR of 36 mm/hr, BUN of 17 mg/dL (6.07 mmol/L), creatinine of 1.5 mg/dL (132.6 Umol/L), lactic acid of 17.0 mmol/L, BNP of 1140.63 pg/ml, total bilirubin of 0.4 mg/dL, troponins of 0.020 ng/mL, and CK-MB of 408 ng/mL. Resuscitation was initiated with administration of isotonic solutions, blood transfusions, and initiation of vasoactive medications, and pantoprazole drip was provided. An initial urgent endoscopy revealed a bleeding Dieulafoy lesion, which was immediately clipped. Acute kidney injury was addressed with aggressive fluid resuscitation as evident by increase in BUN of 33 mg/dL (11.78 mmol/L) and creatinine of 2.9 mg/dL (256.33 Umol/L). CT scan of abdomen performed to rule out ischemic colitis in the setting of progressive abdominal distention showed a diffuse pericolonic inflammation most pronounced at the rectosigmoid colon. Repeat endoscopy showed multiple ulcerated lesions of the gastric mucosa () with a biopsy that revealed necrotic debris with acute leukocytic exudate and numerous variably sized, 90-degree angulated fungal hyphae favoring mucormycosis (). Intravenous liposomal amphotericin-B (5 mg/kg/day at 60 kg weight) was started at a dose of 300 mg daily. Due to deterioration of renal function on the second day, treatment was switched to oral suspension of posaconazole at a dose of 400 mg twice a day for a total of 6 days (of note, no levels of posaconazole were drawn during hospital course). Repeat CT scan of the abdomen showed mild-to-moderate hydroureter with hydronephrosis and bladder wall thickening Figures and . Cystoscopy showed distorted anatomy of bladder lumen with multiple mounds of tissues all throughout the bladder most notable in the trigone with failure to locate ureteral orifices. Random biopsies taken from the bladder tissue also showed necrotic debris with active inflammatory and numerous variably sized angulated fungal hyphae consistent with Mucorales species. Once bladder biopsy confirmed Mucor spp, treatment was switched from posaconazole to intravenous isavuconazonium sulfate at a dose of 372 mg every eight hours for 48 hours as loading dose and then to an oral dose of 372 mg daily. Unfortunately, no sensitivity of the isolate to the newer triazoles was available. Surgical approach was considered; however, given patient's frail state and multiple comorbidities, such approach was deferred. Hydronephrosis significantly improved after placement of bilateral nephrostomy tubes. In addition, continuous bladder irrigation with amphotericin-B was given for localized bladder infection for a total of 5 days. A three-way Foley catheter was inserted for continuous amphotericin-B bladder irrigation where 200 ml of medication was administered via catheter; then, the catheter was clamped for 90 minutes and drained to gravity every six hours. After three weeks of systemic treatment with antifungals, a third endoscopy showed only inflammatory changes with a pathologic report, in which mucormycosis was no longer appreciated. His kidney function improved and remained stable after clamping and removal of his nephrostomy tubes. The patient completed 6 months of therapy with 372 mg of oral isavuconazonium sulfate per day. He is currently at home with resolution of his symptoms and no clinical evidence of relapse after 12 months since completion of his treatment. |
pmc-6031080-1 | A 66-year-old female with a medical history of hypertension, hyperlipidemia, diabetes mellitus type 2, and obesity presented with hip pain after a fall. Radiograph of the hip was negative for dislocation or fracture. On physical exam, she had proximal muscle weakness greater in the lower extremities, and the rest of her examination was negative for skin rashes, lymphadenopathy, joints inflammation, and pedal edema. Her EKG showed normal sinus rhythm with no ST-T changes. Blood work showed elevated CPK (9767), CKMB (101.50), and aldolase (60) levels and LFT derangement (AST-302 and ALT404) (Tables and ). Other lab findings were negative for ANA, anti-Jo antibody, and antimitochondrial antibody. Thyroid function tests were normal. At that time the patient was taking atorvastatin 80 mg which recently replaced simvastatin for better hyperlipidemia control. Atorvastatin was discontinued, and the patient was followed closely in the medical clinic. The proximal muscle weakness continued to progress rapidly during the following month with significant impairment of her walking and rising from a chair without support. The physical exam revealed a decrease to 2 out of 5 motor strength of the proximal upper extremities and 3 out 5 of the proximal lower extremities, and the rest of the physical examination remained normal. Laboratory results with persisting elevated muscle enzyme (CPK, CKMB, and aldolase) levels remained elevated. Testing for anti-HMGCR antibody with ELISA was positive at 34 (reference value: greater than 20 is positive). Muscle biopsy was consistent with necrotizing myopathy (Figures and ). Taken with the antibody results, the final diagnosis of statin-induced necrotising autoimmune myopathy was performed. Findings were consistent with diagnosis of necrotizing autoimmune myositis. Prednisone therapy started at 1 mg/kg per day and tapered over the next three months. The patient's proximal upper and lower strength and mobility improved back to her baseline with normalization of the muscle enzymes and the liver function tests. Upon further review of the patient's chart it was found that she had been taking simvastatin for at least thirteen years which was recently discontinued after which she was switched to atorvastatin. Due to necrotizing myopathy's high association with malignancy, cancer screening reports were reviewed. The patient had a normal colonoscopy in 2014, cervical cancer screening was negative, and calcifications on serial mammograms remained stable. The patient started complaining of weakness one month after initiation of the new therapy with atorvastatin. Liver function tests recorded during treatment with simvastatin were within normal limits. |
pmc-6031089-1 | A 49-year-old Caucasian woman was referred to a general surgeon by her primary care physician for a right nipple lesion. She reports no past medical history, and social history is notable for tobacco use. The patient stated that she first noticed a small, yellow, and fleshy bump on her right nipple approximately two years prior. She became concerned due to its rapid growth in the last year. Four months prior, she visited her primary care provider who performed a shave biopsy of the lesion. Initial pathology results suggested squamous cell carcinoma, but this was thought to be discordant with the clinical picture. At consultation, she reported that her right breast had also started feeling hot and tender for two weeks duration. On examination, her right breast was erythematous and rigid with a 2.2 cm lesion consuming the right nipple. Her nipple also drained yellow pus. The patient was placed on a course of antibiotics due to concerns for an abscess.
An MRI was ordered to investigate possible underlying breast malignancy. Results of the MRI showed no solid mass underlying the nipple. A wedge biopsy of the nipple was performed to confirm the initial shave biopsy pathology (). Pathology showed verrucous histologic features with chronic inflammation and underlying abscess with concerns for a possible cutaneous malignancy. Specifically, there was marked papillomatosis with hypergranulomatosis between the papillae as well as a lack of granules at the papillary surfaces. Immunohistochemical testing was also positive for HPV L1 capsid protein. Because the initial shave biopsy showed SCC with positive margins, this clinical picture suggested that an inadequate sample was taken during the wedge biopsy. After consultation of literature, excision with wide margins was determined to be appropriate for removal of the lesion.
The patient underwent a wide excision of the right nipple-areola complex for removal of the growth. A 6.2 cm x 3.2 cm skin ellipse was excised encompassing the 2.2 cm nipple lesion. On final histologic examination, the lesion was found to be a large tumor with verrucous features, hyperkeratosis, dyskeratosis, and nests of keratin pearl formation (). Two areas showed microinvasion of the dermis with loss of the basal lamina. There were also significant acute and chronic inflammatory responses in the deep dermis representing an underlying abscess. These findings are congruent with the diagnosis of a well-differentiated squamous cell carcinoma with arising within verruca vulgaris. All margins were found to be clear of tumor cells. The post-op course was uneventful. She continues to follow up to monitor potential recurrence of the growth. |
pmc-6031107-1 | A 36-year-old Chinese man without any systemic medical illnesses presented to our emergency department with a 5-h history of acute general weakness that occurred upon waking up. On the day prior to symptom onset, he suffered from the feeling of low-grade fever and ate heavily before sleeping. There was no history of recent strenuous exercise or diuretic use. He denied any history of palpitations, hand tremors, abdominal pain, diarrhea, body weight loss, and numbness of limbs. Neither he nor his family members had previously experienced any such attack.
On physical examination, his blood pressure was 121/63 mmHg, body temperature was 36.6 °C, pulse rate was 102 beats per minute, and respiration rate was 18 breaths per minute. The patient had decreased muscle power especially in the lower extremities (lower limbs: proximal muscles 2/5 and distal muscles 4/5; upper limbs: proximal muscles 4/5 and distal muscles 5/5), but there was no flaccid paralysis of the lower extremities or areflexia. Other physical examination findings were unremarkable. The results of biochemical studies conducted on admission are shown in Table . Marked hypokalemia (2.2 mmol/L) and mild hypomagnesemia (1.8 mg/dL) were prominent findings. Urine potassium excretion indicated renal potassium wasting [transtubular potassium gradient (TTKG): 7.02; and fractional excretion of potassium (FeK): 7.12]. Potassium supplementation was initiated with potassium chloride (KCl) infusion (20 mEq of KCl in 500 mL of normal saline infused at a rate of 120 mL/h). The serum potassium level was corrected (from 2.2 to 3.8 mmol/L) within 4 h via KCl replacement (total: 19.2 mEq). The hormonal profile, including low thyroid-stimulating hormone (TSH) (< 0.0025 uIU/mL), elevated free tetraiodothyronine (T4) (1.8 ng/dL), and normal triiodothyronine (T3) (1.4 ng/mL), indicated hyperthyroidism. Because TPP was highly suspected due to the rapid resolution of hypokalemia despite high renal potassium excretion, an oral beta-blocker and anti-thyroid medication (propranolol, 10 mg BID, and methimazole, 5 mg BID) were prescribed for controlling the hyperthyroidism. Normal anti-thyroid peroxidase antibody (< 1.0 IU/mL) and high titers of thyroid-stimulating hormone receptor antibody (18.4%) indicated Graves’ disease. Thyroid sonogram revealed bilateral multinodular goiter (Fig. ). Finally, he was discharged on day 3 of admission in a stable condition with normal serum potassium levels.
No hypokalemia was noted during the outpatient follow-up (at 9 days after discharge). Methimazole was continued to control his hyperthyroidism, and the dose was adjusted according to the thyroid function test findings. Moreover, no more muscle weakness episode occurred during the one-year follow-up in the outpatient department of endocrinology. |
pmc-6031139-1 | A 30-year-old male patient presented for a consultation in the Prosthodontics department of the Lyon University Hospital (France). The patient complained of a pain caused by the mobility of his maxillary right central incisor (11) (Fig. ). Patient history revealed a trauma with luxation and periradicular infection of the tooth, as well as daily use of tobacco and cannabis. Clinical examination of the oral cavity indicated poor hygiene, dental discolorations, moderate periodontal disease, and edentulous zones due to upper first premolar extractions. The painful tooth was horizontally and vertically mobile (more than 2 mm), partially extruded with vestibular position and gingival inflammation, without local signs of active infection. The dental radiograph of the incisor revealed periradicular bone loss (Fig. ).
An early treatment was proposed to the patient to manage the loose incisor. It involved the extraction of the traumatized incisor due to its very poor prognosis, and then the immediate replacement of the lacking tooth with an artificial one. Several options were proposed to the patient, including the placement of a dental implant with a provisional crown, of a cantilever bridge, or of a removable partial denture. All options were discussed, and after a one week period of reflection the patient finally selected the realization of a temporary removable denture for economic reasons. The patient was informed that final prosthetic rehabilitation will be initiated only after treatment of the periodontal disease and disappearance of tissue inflammation. Clinical and technical steps were summarized in a timeline (Additional file ).
To avoid the risk of extraction of the loose incisor that could occur when taking an impression with alginate, we decided to make a digital impression of the patient’s maxillary and mandibular dental arches with an intraoral scanner (TRIOS 2; 3Shape Copenhagen, Danemark) (Fig. and ). Vestibular areas were scanned using lip and cheek retractors (Optragate, Ivoclar, France). Arches were then virtually aligned using two vestibular records, as recommended by the manufacturer. Color registration was performed with the Vivodent PE shade guide (Ivoclar, France) (Fig. ). Arch digital impressions were converted into STL files and imported in dental CAD software (DentalCad, Exocad, Germany) (Fig. ). The traumatized incisor was then removed virtually (Fig. , and ).
A virtual central incisor was generated from the contralateral central incisor (21) by using the mirror tool (Fig. ) and saved in an independent file. The latter was then imported into a second CAD software (Freeform, 3D Systems, US) and two small cylindrical volumes were created into the palatal side of the virtual incisor to create a retention area (Fig. and ). In the same software, limits of the denture base were virtually designed by using the point and click tool. The denture was generated with a volume (average thickness of 2.5 mm) corresponding to a replica of the patient palatal surface (Fig. and ). Data generated were then exported to a 5-axis milling machine (DWX 52 DC, Roland, Japan) and the resin artificial incisor was made by milling a stratified ivory disk of PolyMethylMethAcrylate (PMMA) (Trilux, Vipi, Italy). The denture base was produced from a disk of pink PMMA (Ivobase CAD, Ivoclar, France). The incisor was bonded onto the denture base using an adhesive agent (Probase, Ivoclar, France). Two metal clasps were manually designed and manufactured on a model printed in parallel (ProJet 3500 HD, 3D Systems, US) by using clap wires (Wironit, Bego, France). Clasps were integrated into the denture base with autopolymerisable resin (Probase, Ivoclar, France) (Fig. ). Upon reception to the clinics (three weeks after digital impressions), the removable denture was cleaned, and finishing and polishing were checked.
After oral disinfection with 0,5% chlorhexidine (Eludril Pro, Pierre Fabre Oral Care, France), a local anesthesia was realized in the buccal and palatal oral mucosa next to the loose incisor. The latter was then extracted atraumatically and hemostasis was realized by compress application (Fig. ). After having controlled the formation of the initial clot, the immediate removable partial denture was positioned into the mouth (Fig. and ). No correction was needed. The initial retention of the denture base was excellent. The patient reported no difficulty with mastication and expressed his great satisfaction for aesthetical appearance of the prosthesis. Occlusal integration was checked to prevent any static or dynamic dysfunctional contacts. The form, volume and texture of the milled central incisor was adequate. The tooth color was fine, although translucency matching was difficult to obtain with a resin stratified ivory disk. He was recalled after one week to assess wound healing and the patient tolerance to the immediate prosthesis. Patient reported an excellent aesthetic and occlusal integration (Fig. ). Healing of the oral mucosa was confirmed by the closure of extraction socket and the non-inflammatory aspect of the oral mucosa (Fig. ). There were no adverse and unanticipated events to report. |
pmc-6031141-1 | A 30-day-old Caucasian baby girl was referred to our hospital by a gynecologist who had detected an intraabdominal cystic mass during prenatal US in the last trimester. A physical examination revealed a mass of approximately 6 cm in diameter that could be palpated in the midline below the umbilicus. No other factors were present in her history. Imaging studies showed intraabdominal cystic mass. The tumor markers that were examined to determine the presence of malignancy were within normal ranges. During surgery, torsion was detected in her left adnexa; a left cystic mass with torsion was necrotic in appearance and was completely lacking normal ovarian and fallopian tube tissue. A left salpingo-oophorectomy was performed. An oophoropexy was performed on her right ovary with absorbable suture at the level of the pelvic brim of the sidewall of her abdomen after draining peripheral cysts (Fig. ); an incidental appendectomy was performed. Pathologic examination of the specimen confirmed the diagnosis of a necrotic ovary. She had cysts smaller than 1 cm on the existing single contralateral ovary they were aspirated during surgery. Postoperatively the cysts redeveloped up to 2 cm and spontaneously disappeared after 6 months of follow-up. She developed no wound infections or peritonitis during the PO period and was discharged on the third PO day. Her ovary was checked regularly with pelvic and Doppler imaging after surgery; the development of her ovary was also age appropriate. She has been followed for 2 years. |
pmc-6031141-2 | A 55-day-old Caucasian baby girl was referred to us by a gynecologist who had detected an intraabdominal cystic mass during prenatal US in the last trimester and was normal upon physical examination; no palpable abdominal masses were found. No other factors were present in her history. Imaging studies showed intraabdominal cystic mass. The tumor markers that were examined to determine the presence of malignancy were within normal ranges. She underwent surgery; her right ovary was found to be autoamputated due to torsion. The amputated necrotic, wandering ovary was removed, and the cysts in the contralateral ovary were drained (Fig. ); an incidental appendectomy was performed. Pathologic examination of the patient confirmed the diagnosis of a necrotic ovary. She had cysts smaller than 1 cm, they were aspirated during surgery. Postoperatively the cysts redeveloped and spontaneously disappeared after 1 year of follow up. She developed no wound infections or peritonitis during the PO period and was discharged on the third PO day. After surgery, her ovary was checked regularly using pelvic and Doppler imaging. The development of her remaining ovary was age appropriate. She has been followed for 2.5 years, and she continues to be followed uneventfully. |
pmc-6031141-3 | An 8-year-old Caucasian girl was admitted to our hospital with acute abdominal symptoms 60 hours after the complaints started; serious acute pelvic pain, sudden onset of nausea, vomiting, and pelvic pain and tenderness were present. No other factors were reported in her history. Direct abdominal X-ray images were normal. She was pre-diagnosed as having appendicitis or ovarian pathology based on anamnesis, a physical examination, and laboratory findings. She was operated on under emergency conditions and without prior US investigation. On operation, torsion was detected in her right ovary. She had a necrotic right ovary and salpinx (Fig. ); a salpingo-oophorectomy was performed due to the adnexa showing no improvement in its black color and necrotic appearance after detorsion. The contralateral ovary was normal, and an incidental appendectomy was performed. The pathology report indicated a hemorrhagic infarct in the ovary. She developed no wound infections or peritonitis during the PO period and was discharged on the fifth PO day. After surgery, her remaining ovary was examined regularly using pelvic and Doppler imaging. The development of her remaining ovary was also age appropriate. She has been followed for 6 years, and she continues to be followed uneventfully. |
pmc-6031141-4 | A 10-year-old Caucasian girl was admitted with serious acute pelvic pain 4 hours after the complaints started. Acute abdominal symptoms were present in the patient; sudden onset of nausea, vomiting, and pelvic pain and tenderness were reported. Direct abdominal X-ray images were normal. No other factors were present in her history. She was pre-diagnosed as having appendicitis or ovarian pathology. On exploration, left adnexal torsion was detected, and detorsion was performed (Fig. ). The ligaments were extremely long; the ipsilateral mesosalpinx was shortened with a nonabsorbable suture, and an incidental appendectomy was performed. No wound infections or peritonitis developed during the PO period and she was discharged on the third PO day. After surgery, her ovaries were checked regularly using pelvic and Doppler imaging. In the second month, the affected ovary was similar in size to the contralateral ovary, and normal blood flow was observed on US. The development of her ovaries was also age appropriate. She has been followed for 6 years. She continues to be followed uneventfully and has regular menstrual cycles. |
pmc-6031141-5 | A 16-year-old Caucasian girl visited our hospital with serious acute pelvic pain 30 hours after her complaints started. Acute abdominal symptoms were present; sudden onset of nausea, vomiting, and pelvic pain and tenderness were reported. She had a normal menstrual cycle. Direct abdominal X-ray images were normal. No other factors were present in her history. The tumor markers that were examined to determine the presence of malignancy were within normal ranges. Preoperative abdominal US was performed; Minimal pelvic fluid, an increase in the diameter of her appendix, and a large right ovary with increased diameter relatively to the left ovary were found on US examination and torsion was suspected in her right ovary. During the operation, right ovarian torsion with a hemorrhagic cyst approximately 8 cm in diameter was detected. A cystectomy was performed to protect her ovary against retorsion, and her ovary was repaired (Fig. ). The contralateral ovarian cysts were drained by aspiration. Her appendix was turgid and edematous and was evaluated as periappendicitis. In this case, as with the other four patients, an incidental appendectomy was performed. She developed no wound infections or peritonitis during the PO period and was discharged on the fifth PO day. After surgery, her ovaries were checked regularly using pelvic and Doppler imaging. In the fourth month, her affected ovary was similar in size to the contralateral ovary, and normal blood flow was observed on US. She has been followed for 1.5 years. She continues to be followed uneventfully and has regular menstrual cycles. The development of her ovaries was also age appropriate. |
pmc-6031155-1 | A 34-year-old man, father of 2 kids, presented to urology outpatient clinic complaining of swelling in the left testis for the last 3 years. The swelling gradually increased in size and was not associated with any pain or fever. The patient also denied prior history of any trauma, infections, or scrotal surgeries. The past medical and surgical history was otherwise unremarkable.
Physical examination revealed a circumcised penis with bilateral developed hemiscrotum and normally descended testes. The right testis was normal, and the left testis had a firm, nontender 2.5 × 2.0 cm smooth mass attached near its lower pole with an associated lax hydrocele.
The baseline hematological and biochemistry workup was normal. Serum markers for germ cell tumor of the testes were all normal with serum lactate dehydrogenase (LDH) 275 IU/l (N = 208–378), alpha feto protein (αFP) 4.0 IU/ml (N ≤ 6.7), and beta human chorionic gonadotropin (βHCG) <2.0 mIU/ml (N < 10). An ultrasound scan was done which showed a 23.4 mm × 22.6 mm well-circumscribed lesion closely associated with the left testis. On color Doppler, no significant vascularity was observed (). The radiologist could not definitely determine the nature of the lesion, and it was labeled as suspicious for neoplastic process. An ultrasound of the abdomen did not reveal any evidence of lymphadenopathy. |
pmc-6031163-1 | A 69-year-old lady with a background of penicillin allergy and left-sided breast cancer treated with mastectomy and axillary node clearance was admitted to the hospital in Liverpool in 2016 with a right-sided breast abscess. The patient had been aware of a lump in her right breast for 3 years and had been told it was a sebaceous cyst. However, it had become painful, and she was now feeling unwell and tachycardic but remained apyrexial.
Antibiotic therapy was initiated with IV clindamycin 450 mg TDS and IV teicoplanin 12 mg/kg BD and then with 12 mg/kg OD after 2 days. The abscess was aspirated and subsequently incised and drained under local anaesthetic. The frank pus drained was malodorous, so oral metronidazole 500 mg TDS was added on. As there was no clinical improvement after 48 hours, a second incision and drainage was performed under general anaesthetic, revealing a large abscess cavity extending 10 cm into the right breast and 7 cm into the left mastectomy scar. This combined with continued triple antibiotic therapy resulted in clinical improvement, so after 4 days of IV therapy, antibiotics were stepped down to oral erythromycin 500 mg QDS of 7-day course and metronidazole 500 mg TDS of 10-day course and the patient was discharged. The abscess healed well in the community.
Twenty-one days after sending aspirated pus for MC + S, Actinomyces europaeus was grown in the culture. The patient was still clinically well, the abscess was healing well, and no underlying chest source of infection was identified.
The breast abscess pus was sent to the microbiology laboratory for testing. There were Gram-positive cocci visualised on the direct Gram stain, but there was no evidence of any Gram-positive bacilli. The pus was cultured onto the following plates: blood agar, MacConkey agar, selective anaerobic agar with a 5-microgram disc, and a fastidious anaerobic agar (FAA) plate in accordance with the laboratory standard operating procedure (SOP) based on the UK Standards for Microbiology Investigations (SMI) []. After 4 days of growth, two colonies grew on the direct FAA plate, which were different. The first colony was identified, using a matrix-assisted laser desorption/ionisation (MALDI-TOF) machine, as a Staphylococcus epidermidis. The MALDI-TOF equipment used was Bruker Microflex utilising MALDI Biotyper Version 3.1 software. The MS method of identification was the AutoX setting, and the software library was the BDAL and Filamentous Fungi Library Version 1.0. The second colony failed to be identified using MALDI. Therefore, the second organism was subbed onto an Actinomyces plate (blood agar with supplementary metronidazole and nalidoxic acid) in accordance with the local SOP. Four days later, there was fine growth on the Actinomyces plate. Sensitivities were obtained via the British Society for Antimicrobial Chemotherapy (BSAC) method of disc diffusion. The organism was sensitive to penicillin 42 mm (sensitivity > 23 mm), erythromycin 40 mm (sensitivity > 10 mm), vancomycin 30 mm (no breakpoint available), tetracycline 42 mm (no breakpoint available), and ciprofloxacin 22 mm (no breakpoint available). The organism was initially identified as Actinomyces europaeus, but the MALDI score was only 1.82; therefore, an extraction MALDI was performed to increase the MALDI score, so a more confident identification of the organism was obtained. The subsequent MALDI identity was Actinomyces europaeus with a score of 2.10. As per the guidance from the SMI, the organism was sloped and sent to the Anaerobic Reference Unit at Colindale. The ID was confirmed as Actinomyces europaeus by partial sequencing of 16sRNA.
Microbiological advice consisted of a long course of antibiotics to eradicate the infection, and the first-line treatment was 2 weeks of IV ceftriaxone followed by 6 months of oral amoxicillin. This was not feasible due to the patient's penicillin allergy.
Second-line advice was 2 weeks of IV erythromycin followed by 6 months of oral erythromycin. However, this was also not feasible. The outpatient parenteral antibiotic therapy (OPAT) service enables patients to receive IV therapy in the community but was unable to provide the four times daily dosing required for IV erythromycin. The OPAT service was only able to provide twice daily regimens.
The third-line approach was acceptable: 2 weeks of IV tigecycline given in twice daily doses followed by 6 months of oral doxycycline. The patient had a midline inserted and received her IV therapy in the community with OPAT services.
After completing the IV tigecycline course with no issues, the patient complained of side effects following 6 weeks of oral doxycycline therapy: oral thrush and leg blisters. Antibiotic therapy was switched to oral clarithromycin to complete a 6-month course of oral antibiotics. There has been no evidence of recurrence to date. |
pmc-6031204-1 | A healthy 15-year-old male presented with a three-week history of irretractable abdominal pain, vomiting, and anorexia. There was no previous similar history or abdominal surgery. He associated the onset of symptoms with a recent contraction of gastroenteritis within the family. There was no improvement in his condition despite his family contacts recovering.
On examination, he was afebrile and haemodynamically stable. There was a scaphoid abdomen with maximal tenderness in the right upper quadrant. There was significant guarding. Bowel sounds were audible. His abdominal X-ray and routine blood test results were both unremarkable, other than a raised C-reactive protein of 92.
His high opioid requirement, chronicity of symptoms, and examination findings prompted further evaluation with CT. This revealed right-sided abdominal mass and a layering effect at the caecal pole consistent with an intussusception. The appearance was similar to a “pseudokidney,” as shown in . There was marked free fluid within the abdominal cavity. After resuscitation, he proceeded to a laparotomy.
A diagnostic laparotomy was performed for the inspection of abdominal contents. It confirmed radiological findings of an intussusception of the terminal ileum within the caecal pole. A hard mass was noted within the hepatic flexure region. There was a dilated terminal ileum and multiple lymph nodes noted within the mesentery.
A right hemicolectomy was performed. Vascular pedicles were taken high for an appropriate oncological resection, given the suspicion. Primary ileocolic stapled side-to-side anastomosis was performed. The recovery was uncomplicated and the patient was discharged home three days postoperatively.
As shown in , the histopathology of the excised mass proved to be Burkitt's lymphoma of the terminal ileum causing ileocolic intussusception. It extensively involved the appendiceal serosa, mesentery, and omentum.
The tumour was an ulcerated lesion infiltrating all layers of the bowel wall. As shown in microscopically, the characteristic starry sky growth pattern was visualised, with the cells having round nuclei, finely clumped chromatin, and small basophilic nucleoli.
A panel of immunostains was performed on bowel tumour, the regional lymph nodes, and the omentum. The tumour cells in all three sites are CD20, CD10, BCL6, and C-MYC positive (see ). The tumour has a very high proliferative index with almost 100% of the tumour expressing Ki67. BCL2, Cyclin D1, TdT, CD3, CD5, CD23, and CD30 are negative. Fluorescence in situ hybridisation (FISH) detected a reciprocal t(8;14) translocation and rearrangement of the C-MYC gene. As only chromosome-specific probes were used, the presence of other abnormalities cannot be excluded. The above profile supports the original diagnosis of BL.
The medical literature in the PubMed and Medline/EMBASE databases was reviewed for cases of Burkitt's lymphoma-related intussusception. All publications were scrutinized that contained keywords of “intussusception” and “Burkitt lymphoma.” The literature search was limited to paediatric patients under 18 years old, and restricted to papers written in English.
A systematic review of relevant literature was performed to ascertain variance of clinical features in primary presentations of BL-related intussusception. The following information on patient numbers, demographic, main presenting complaint, disease stage, and diagnostic methods were obtained in this meta-analysis. |
pmc-6031205-1 | The 2-month-old African American infant was born at full term, at 2890 grams, following an uncomplicated pregnancy and delivery with an Apgar of seven and nine at one and five minutes, respectively. In the newborn nursery, her physical exam did not note any murmur, and her congenital cardiac screen was documented as normal (pre- and postductal saturations 100%) on day two of life. She had no family history of congenital heart defects, arrhythmias, or other cardiac diseases. The infant was discharged home with her mother on day of life two with follow-up arranged with the pediatrician.
At home, she remained stable with no respiratory distress, no feeding difficulties, and adequate weight gain. Her mother did note that the infant's hands and feet appeared slightly “dark” at home, but she was reassured that this was normal acrocyanosis. Her pediatrician saw her at two separate routine office visits, where no murmur or other abnormality was reported. Approximately three days prior to presentation, the infant developed cough, congestion, and rhinorrhea. She was due for her two-month well-child exam, so her mother took her to the clinic for an evaluation. At that time, the infant weighted 4.3 kg placing her at approximately the tenth percentile on a Center for Disease Control growth chart. The pediatrician was concerned by her visible acrocyanosis and pulse oximetry documented an oxygen saturation of 80% in room air, so she was immediately referred to an emergency department for further evaluation.
On arrival to the emergency department, she was hypoxic with oxygen saturations of 20–30% and hypothermic to 32°C. No murmurs were noted on physical exam. Peripheral access was established, first with a tibial intraosseous catheter and then with a peripheral intravenous line; she received a normal saline bolus, a blood culture was drawn (which remained negative), and a dose of ceftriaxone was administered. A rapid sequence intubation was performed with etomidate and vecuronium. The child was successfully intubated with no complications. Despite this and increasing oxygen administration up to 100% FiO2, her oxygen saturation peaked at 80%. Initial labs were significant for blood glucose 95 mg/dL, white blood cell count 7 × 109/L, hemoglobin 22 g/dL, hematocrit 68%, and lactate 5.5 mmol/L. Chest X-ray could not view the aortic knob or descending aorta well and could not rule out a right-sided aortic arch (). With these findings and the severity of her illness, the decision was made to transfer the patient to a tertiary pediatric medical center.
On arrival, a brief transthoracic echocardiogram (TTE) was concerning for Tetralogy of Fallot with critical pulmonic stenosis versus atresia. She received an additional normal saline bolus, sodium bicarbonate, midazolam, and fentanyl. On attempts to draw labs, her blood was noted to be very dark and hyperviscous, clotting almost immediately.
The infant was admitted to the pediatric intensive care unit (PICU) for further stabilization and started on a prostaglandin drip with the hope of reopening her ductus arteriosus to increase pulmonary blood flow. Repeat TTE confirmed the diagnosis of Tetralogy of Fallot with severe pulmonary stenosis, hypoplastic main and branch pulmonary arteries, right-sided aortic arch, and small secundum atrial septal defect (). At no point was a murmur heard by any provider from the ED, PICU, or Cardiology. Over the next several hours, she continued to decompensate with worsening hypoxia and hypotension despite phenylephrine infusion, fentanyl infusion, and aggressive fluid resuscitation. Pediatric cardiothoracic surgery evaluated the patient, and the decision was made to perform an emergent Blalock–Taussig (BT) shunt.
The patient underwent modified BT shunt with a 3.5 mm Gore-Tex shunt anastomosing the innominate artery to the main pulmonary artery. There were no complications, and she returned to the PICU for post-op recovery. Daily TTE demonstrated normal biventricular function without pericardial effusion, as well as flow across BT shunt and through both branch pulmonary arteries. On post-op day one, she was extubated and weaned to room air, where she was able to maintain oxygen saturations around 88%. By post-op day two, she was tolerating oral feeds. She was discharged home on post-op day four with 40.5 mg of aspirin daily to help prevent shunt thrombosis. On discharge, her parents noted a vast difference in the infant's general appearance and energy level. The plan is to take the infant back to the operating room to perform a complete repair with a transannular patch at 4–6 months of life. |
pmc-6031206-1 | A 75-year-old Chinese male with a past medical history of hypertension, hyperlipidemia, coronary artery disease, diabetes mellitus, benign prostatic hyperplasia, and osteoarthritis arrived to our Emergency Department (ED) due to unstable gait. The patient stated that he was a resident of Yonkers, New York (NY). His only travel history involved a train ride to Flushing, NY, the day prior to presentation. The patient reported frequent walks in local parks around Yonkers, NY. At the time of initial examination, he denied headaches, dizziness, shortness of breath, back or chest pain, rashes, focal weakness, or loss of sensation. He had not noticed any ticks or tick bites on his skin in the past year. On physical exam, he was noted to have normal extraocular muscle movements, neurologic exam without focal deficits, and musculoskeletal, cardiac, and respiratory exams without abnormalities. His electrocardiogram showed normal sinus rhythm with a rate of 69 beats per minute. His labs revealed slightly decreased hemoglobin and hematocrit levels and thrombocytopenia along with increased bilirubin levels. His chest X-ray (CXR), rapid influenza swabs, blood cultures for bacteria, and urine analysis were negative. He was treated in the ED until he was hemodynamically stable and asymptomatic, after which he was then discharged.
He returned 4 days later complaining of worsening fevers since discharge, with a maximum recorded temperature of 105 degrees Fahrenheit (40.55°C), occasional rigors, chills, diaphoresis, diffuse myalgias, generalized weakness, malaise, confusion, and decreased appetite. The patient's gait was noted to be unstable with difficulty maintaining balance. On physical examination, the patient appeared lethargic. He was noted to be tachycardic at 98 beats per minute (bpm), to have a black discoloration of patient's tongue, and to have a recorded temperature of 102.8 degrees Fahrenheit (39.33°C). His blood pressure, respiratory rate, and the remainder of his neurological exam was normal. His CXR was significant for fullness in the right mediastinum, cardiomegaly, and unfolded aorta. His hemoglobin, hematocrit, and comprehensive metabolic panel remained stable from previous admission. It was noted that the patient had decreased white blood cell (WBC) count and severe thrombocytopenia on readmission (). Initial serum ELISA for Lyme disease, used as a screening tool, was negative. Magnetic resonance imaging (MRI) of the brain showed no plaque deposition, demyelination, or other acute pathologic processes. A computed tomography (CT) scan of the abdomen revealed a small aneurysmal dilatation of the thoracic aorta and splenomegaly with an acute appearing splenic infarct. He was started on ceftriaxone, ampicillin, and vancomycin to cover for a bacterial infection.
During the second night of hospitalization, the patient's heart rate spiked to over 200 bpm and he became hypotensive. He was transferred to the Intensive Care Unit (ICU) for management of severe hemodynamic instability and decompensated respiratory status. The patient's fever of unknown origin, encephalopathy, and negative initial screening tests prompted us to draw additional sets of serum tests for multiple possible bacterial, viral, and fungal etiologies. Unlike the initial serology, this new set yielded positive test results for the Lyme IgG and IgM ELISA test, Lyme IgM Western blot, and Babesia microti IgG and IgM antibody tests (). To confirm the diagnosis, a lumbar puncture (LP) was performed after patient's thrombocytopenia was corrected. During the LP procedure, the CSF was noted to be proteinaceous and congealed by the performing interventional radiologist. The LP resulted in positive tests for Lyme IgG and IgM antibody levels by Western blot, as well as a positive Western Blot for Lyme IgM (). Cytomegalovirus, Epstein-Barr virus, West Nile virus, and rheumatoid factor were not present. The patient was then started on doxycycline for the diagnosis of Lyme neuroborreliosis. Within 48 hours of doxycycline administration, the patient clinically improved, his mentation returned to baseline, and his gait returned to normal. The patient was treated for 5 days and was discharged on an oral course of doxycycline for a total of 21 days.
Here we have presented a patient who had no characteristic clinical signs of LD, whose main symptoms were a high fever, encephalopathy, diffuse myalgia, and tachycardia. Blood culture, urine culture, and CSF gram stain were negative. An MRI of the brain and CT of the chest/abdomen showed no source of infection. The single diagnostic modality yielding concrete results was the patient's CSF Lyme serology. |
pmc-6031209-1 | A 51-year-old male was referred to our hospital because of a three-month history of gradually progressing renal failure. During his first hospitalization, he complained of lumbar pain. On physical examination, he had conjunctival pallor and severe percussion tenderness of his back. No skin lesions or neurological deficits were seen. Laboratory test results were as follows: hemoglobin, 8.7 g/dL; creatinine, 7.01 mg/dL; total protein, 7.4 g/dL; albumin, 3.2 g/dL; calcium, 14.8 mg/dL; phosphate, 6.2 mg/dL; beta-2-microglobulin, 27.9 mg/L; IgG, 341 mg/dL; IgA, 21 mg/dL; IgM, 18 mg/dL; free kappa light chain, 99,900 mg/L; and free lambda light chain, 9.7 mg/L. Chest X-ray results were normal. Computed tomography (CT) showed vertebral compression fractures of Th8 and L1 and bilateral pleural effusions without calcified lesions. Urine immunoelectrophoresis showed a positive result for the Bence-Jones protein. Bone marrow aspiration revealed plasma cell proliferation (65% of total nucleated cells, ) with expression of CD38 and CD56, absence of CD19 and CD20, and an MIB-1 labeling index of 25%. Chromosomal analysis of the bone marrow by G-banding showed a normal 46,XY karyotype, but fluorescence in situ hybridization revealed the abnormalities del(13q) and t(4;14). He was diagnosed with Bence-Jones protein type MM (stage III according to the International Staging System, and stage IIIB according to the Durie–Salmon classification system).
We began treatment with intravenous fluids and intramuscular injections of calcitonin to treat the severe hypercalcemia. Simultaneously, he received bortezomib-dexamethasone (Bd) therapy (subcutaneous injection of 1.3 mg/(m2·day) bortezomib plus 20 mg/day dexamethasone orally on days 1, 4, 8, and 11). Unexpectedly, he experienced severe acute heart failure on day 8, and temporarily required the support of a mechanical ventilator. Bd therapy was discontinued during the first treatment cycle. Because renal function had not improved, maintenance hemodialysis was initiated. Subsequently, we continued MM treatment with two cycles of vincristine-doxorubicin-dexamethasone (0.4 mg/body of vincristine and 9 mg/m2 of doxorubicin on days 1 to 4; and 40 mg/body of dexamethasone on days 1 to 4, 9 to 12, and 17 to 20 of a 28-day cycle) and the M2 protocol (multiple chemotherapeutic agents, not including proteasome inhibitors), followed by lenalidomide-dexamethasone (Rd) therapy (5 mg/day lenalidomide on days 1 to 21 plus 20 mg/body dexamethasone on days 1, 8, 15, 22 of a 28-day cycle).
About four months after starting Rd therapy, the patient suffered from myoclonus-like movement of the lower extremities. During the sixth cycle of Rd therapy, he complained of pain in both lower legs, but did not have skin lesions or tenderness. He had been taking loxoprofen, fentanyl (patch and buccal tablet), mecobalamin, ferrous fumarate, lansoprazole, amlodipine, furosemide, alfacalcidol, and darbepoetin alfa. It was unlikely that his pain was drug-induced.
The patient's serum creatinine kinase level was elevated to 1,268 U/L. Diffusion-weighted and short tau inversion recovery magnetic resonance imaging revealed diffuse high signal intensity in the crural muscles (). A muscle biopsy was performed on the right tibial anterior muscles () and 40 mg/day prednisolone was prescribed by a neurologist because of suspected polymyositis/dermatomyositis. However, typical pathologic findings of polymyositis/dermatomyositis, like lymphocyte infiltration around muscle fibers, were absent and vessel calcification was noted. Prednisolone was ineffective against his symptoms. During steroid administration, well-demarcated ulcers developed on the right lower leg, both exterior thighs, and the penis. These ulcers gradually worsened () and the patient experienced severe pain, especially during dialysis or exercise. He could not continue dialysis because of this exacerbation of pain. Moreover, muscle atrophy of his lower limbs impaired his daily activities. The administration of 40 mg/day prednisolone was continued for 42 days and was then stopped on the 84th day after tapering. The patient's serum intact parathyroid hormone (PTH) level was 429 pg/mL, while previously it was permissively controlled within the range of 140–250 pg/mL. Before dialysis, his levels of serum albumin, calcium, and phosphate were 4.0 g/dL, 7.4 mg/dL, and 7.9 mg/dL, respectively. He was diagnosed with secondary hyperparathyroidism (HPT). We could not exclude a relationship between MM and HPT, although his free light chain ratio was decreasing.
CT showed widespread centrilobular opacities of both lungs () and high-density lesions along small blood vessels in the trunk and extremities (), but the mediastinum, abdominal organs, and large vessels like the thoracic and abdominal aorta were intact. A pulmonary function test demonstrated restrictive impairment with reduced diffusion capacity: the predicted forced vital capacity was 48.6%, the forced expiratory volume of the first breath was 87.9%, and the predicted diffusing capacity of lung for carbon monoxide was 67.1%. 99mTc-hydroxymethylene diphosphonate scintigraphy revealed abnormal diffuse accumulation in both upper lung fields (). Echography revealed no enlargement of the parathyroid glands.
Next, a transbronchial lung biopsy was performed, and microscopy confirmed the presence of calcifications of the alveolar walls () and of a small vessel in the right anterior tibial muscle (). However, pathological calcification was absent from the right exterior thigh ulcer. Healing of the skin biopsy wound was delayed.
The patient was ultimately diagnosed with muscle and skin ischemia from CUA. He was treated with cinacalcet, and his intact PTH levels fell to a normal range. He underwent four additional cycles of Rd therapy, but ulcer infections occurred repeatedly in both thighs, occasionally progressing to sepsis. He has since been monitored closely for MM, without treatment, for four months. Meanwhile, the ulcers have achieved epithelialization after topical treatment, but his serum free light chain ratio level increased from 290 to 1532. He is currently undergoing Pd therapy (2 mg/day pomalidomide on days 1 to 21, plus 4 mg/day dexamethasone on days 1, 8, 15, and 22 of a 28-day cycle) without any adverse events. Since severe heart failure occurred during the combined regimen with bortezomib, we have avoided administering proteasome inhibitors. His disease is stable according to the International Myeloma Working Group criteria. |
pmc-6031211-1 | The patient was a 76-year-old man who came to the office in 2014 looking for possible treatments of his fractured central incisors. Nothing was found relevant about his medical condition. The patient shows a high risk for caries and also eccentric bruxism. He has partial edentulism in the superior left quadrant and multiple decay and fractured teeth. The initial approach was conservative aiming to keep the upper front by means of composite fillings (Figures and ). Then, the posterior superior quadrants needed to be restored with implants.
Three years after, in 2017, the patient came back to the office referring pain of endodontic origin in the upper left canine. New and secondary subgingival caries were found in the six front teeth. The conservative prognosis was considered poor due to the subgingival depth and extent of decay presented by the lesions from canine to canine. After having discussed the treatment options, especially the surgical lengthening of the front teeth or the orthodontic extrusion, the patient decides to replace the residual teeth with a new implant-supported bridge similar to the recently performed prostheses of the posterior areas that were judged by him as a highly satisfactory treatment. The patient preferred not to involve these restorations in the present anterior treatment and limited it to place only two implants in the lateral incisors' positions ().
The treatment was carried out in a staged approach. Briefly, first, we extracted the lateral incisors, using the SST, and placed two immediate implants. The four residual teeth were then prepared to be used as abutments of a temporary bridge for the purpose of maintaining the aesthetics and function of the patient during the early osseointegration period. In a further step, the four remaining teeth were also extracted using the SST, and the initial provisional bridge was replaced by the second provisional screwed on the uncovered implants. Only one out of the four abutment teeth used for the temporization of root canal treatment was needed due to a periapical infection.
When placing the two immediate implants into the alveolus of the lateral incisors, a section of the buccal part of the root (about the two middle thirds) was left in place and no biomaterial was used at all. An impression of the implants was taken to have the second temporary bridge available in the second surgery. Healing abutments were then attached with the proper height for the soft tissue to cover them but at the same time facilitating the uncovering. Finally, a temporary acrylic bridge was cemented onto the four abutment teeth 13-11 and 21-23 ().
Three months later, the implants were uncovered, the four abutment teeth were extracted, again with the SST—partial extraction of the roots—but this time no more implants were placed in these sockets. The first provisional cemented onto the teeth was then replaced by a second acrylic bridge screwed onto the implants though temporary abutments ().
The partial extraction of the canines, aiming to leave a buccal slice of the root, was so hard to perform, and further instrumentation would lead to the socket destruction that a decision was intraoperatively made and a greater portion of the root, including the apex, was finally left. As the locations of the canines did not involve the implant sites, any potential complication could be addressed efficiently.
One month later, the prosthodontic phase was undertaken. Little if any differences in the buccal tissue volume and no noticeable aesthetic impact could be found after the multiple extractions (). The desired position of the incisal border was determined by various try-ins, and five months after implant surgery, the definitive prosthesis was placed. The final clinical aspect can be appreciated in the pictures (Figures and ). |
pmc-6031420-1 | A previously healthy 25-year-old man presented with multi-fragmentary fractures
of the lower third of right tibia and fibula as a consequence of a motorcycle
accident (). Upon hospital
admission he was alert, coherent and had no motor deficits. His vital signs and
the rest of a physical examination were normal. He was admitted to the hospital
for surgical stabilization. Forty-eight hours after admission, he developed
confusion and agitation followed by a rapid decline in his level of
consciousness that progressed to coma with bilateral extensor posturing. His
pupils were equal, slightly large and reactive. He was tachypneic (44/min),
tachycardic (137/min), febrile (39.3°C) and hypertensive (147/101mmHg). His
pulse oximetry was 92% on room air. Petechial hemorrhages were noted in the
sclerae, conjunctivae, buccal mucosa and the upper third of the thorax.
Resuscitation was initiated with fluids, supplemental oxygen, tracheal
intubation and mechanical ventilation under deep sedoanalgesia. A head computed
tomography (CT) scan revealed multiple and bilateral frontal subcortical
hypodense areas without a midline shift. No hemorrhage was evident, and the
basal cisterns and sulci remained visible ().
A chest CT was normal except for small bilateral basal atelectasis with no
evidence of pulmonary embolism. Transthoracic echocardiography revealed normal
ventricles, normal valve function and an absence of patent foramen oval or signs
of pulmonary hypertension. An electroencephalogram revealed diffuse slowing
without epileptiform discharges. The laboratory parameters were normal except
for elevations of the following inflammation markers: leukocytosis
17300/mm3 and C-reactive protein (CRP) 141mg/L. Thrombocytopenia
(110.000/mm3) was also noticed. A diagnosis of FES was reached.
Supportive therapy was provided, and methylprednisolone (10mg/kg/day) was
administered for 3 days. Two days later, given his clinical stability, the
sedation and analgesia were withheld to assess his neurological status. After 10
minutes, bilateral and spontaneous extensor posturing was observed in
combination with mechanical ventilation asynchrony, tachypnea (46/min), profuse
and generalized sweating, arterial hypertension (215/112mmHg) and tachycardia
(137/min). These manifestations were recurrent and led to the diagnosis of PSH.
Consequently, propranolol (120mg/d) and morphine (3mg) every 4 hours were added
to the therapeutic regimen. The sedoanalgesia was reinstituted. Two days later,
a brain MRI revealed multiple small lesions in both cerebral hemispheres that
were hypointense on T1 and hyperintense on T2 and FLAIR, did not exhibit
enhancement after gadolinium injection and were located in the periventricular
white matter of both frontoparietal regions ().
The episodes of PSH had durations that oscillated between 20 and 35 minutes,
occurred with a frequency of 3 to 4 times daily and were mainly associated with
stimuli such as the turning, bathing, aspiration of secretions and pain. On the
8th intensive care unit (ICU) day, remifentanil and propofol were
withdrawn and replaced with dexmedetomidine while the propranolol and morphine
were maintained at the previous doses. The episodes of sympathetic discharge
became progressively briefer and less severe and reached no more than 2 per day.
The motor responses improved to the localization of the nociceptive
stimulus.
On ICU day 12, the patient was extubated. Sixteen days after admission, he was
alert, had normal motor responses and was able to communicate with his family.
The doses of propranolol and morphine were reduced to half without worsening of
the episodes of PSH. Twenty-two days after admission, the fracture was
surgically fixed. The post-operative course proceeded without complications.
Three days after surgery, the patient was discharged home to continue ambulatory
rehabilitation. One year after the event, the lesions found in the MRI had
disappeared, and neuropsychological testing revealed a mild impairment of
executive functions with alterations of short and long-term memory. |
pmc-6031420-2 | A 21-year-old man without any medical history presented with a closed fracture of
the lower third of his tibia secondary to a motorcycle accident without evidence
of cranial trauma (). His vital
signs and neurological examination were normal. The patient was hospitalized and
treated with skeletal traction. One day later, he became confused and agitated
with progressive depression of consciousness that progressed to coma. He
exhibited an extensor motor response, but the brainstem reflexes were normal.
The vital signs were as follows: arterial blood pressure, 127/76mmHg;
respiratory rate, 18/min; heart rate, 133/min; and rectal temperature, 38.8°C.
His pulse oximetry was 85% on room air. Invasive mechanical ventilation and
hemodynamic resuscitation were initiated immediately. After cardiorespiratory
stabilization, a head CT scan revealed small and multiple subcortical hypodense
lesions in both the frontal and left parietal regions ().
A chest CT revealed small filling defects in the left ventricle and the superior
vena cava. A nodular image was observed in the right pulmonary artery and could
have corresponded to an embolus. Fat embolism syndrome was diagnosed. A
transthoracic echocardiogram revealed an estimated mean pulmonary artery
pressure of 35mmHg with no disorders of ventricular motility and preserved
systolic ventricular function. The size and function of the right ventricle were
normal, and there were no interatrial or interventricular shunts. An
electroencephalogram revealed background slowing without epileptiform
discharges. The biochemical profile was unremarkable with the exception of
thrombocytopenia (98.000/mm3), anemia (Hgb 8.7gr/dL) and an elevation
of the CRP level to 134 mg/L. General critical care supportive measures were
provided, and the patient received IV methylprednisolone at 10 mg/kg/day for 72
hours.
On ICU day 5, sedoanalgesia (remifentanil-propofol) was stopped. After 20
minutes, the patient exhibited generalized and profuse sweating, tachycardia
(166/min), arterial hypertension (187/99mmHg), and tachypnea (36/min) with
asynchrony of mechanical ventilation and bilateral extensor posturing; these
manifestations were all compatible with PSH. The symptoms were alleviated with
the restoration of sedoanalgesia but continued to occur 4 times per day and were
triggered by physiotherapy maneuvers, aspiration of secretions and baths with
cold water. The average duration of the episodes was 33 minutes. A brain MRI
revealed multiple T2-hyperintense lesions in the periventricular white matter
and bilateral frontal and parietal subcortical regions ( and ).
Treatment with propranolol at 120mg/day, morphine at 18mg/day and gabapentin at
900 mg/day resulted in progressive reductions of the frequencies, intensities
and durations of the PSH episodes. The patient was extubated without
complications on hospital day 11. After 20 days, he was transferred to the
general ward; he was alert, oriented, communicating with his family and feeding
on his own. He was maintained on propranolol at 40mg/day and morphine at
6mg/day. Surgical fixation of the fracture was performed one month after
admission. He was discharged to home three days after the surgery. At 12 months
after the event, the previously noted lesions were no longer visible on a follow
up MRI, and the patient returned to his work and his classes at the university
without alterations in neurocognitive testing results. |
pmc-6031552-1 | A 63-year-old Japanese man, a current smoker of more than 45 pack-years, was revealed by routine examination’s chest X-ray to have an abnormal shadow in the left middle lung field. His past medical history was unremarkable. A chest computed tomography (CT) scan showed a 3.6 × 2.8 cm pulmonary lesion in the left upper lobe, with a clear boundary and heterogeneous contrast enhancement (Fig. ). Positron emission tomography (PET) showed an accumulation of 18F-fluorodexyglucose (FDG) with a maximum standardized uptake value (SUV max) of 4.95 in the early phase and 6.31 in the late phase in the nodule. No accumulation of FDG was noted in the pulmonary hilum, or mediastinal lymph nodes.
A trans-bronchial lung biopsy (TBLB) of the mass in the left upper lobe was performed; it revealed non-small cell carcinoma. Although the serum carcinoembryonic antigen (CEA) level was 52.1 ng/mL (normal range 0–5.0 ng/mL), there were no distant metastases or other cancer lesions. The preoperative clinical diagnosis of primary lung cancer, stage cT2aN0M0 stage IB was considered.
A left upper lobectomy and mediastinal lymph node dissection were performed by video-assisted thoracic surgery. The resected tumor measured 3.8 × 2.8 × 2.8 cm in diameter.
Grossly, the specimen showed a well-established boundary as a grayish lesion with areas of necrosis. The histopathological examination showed a solid proliferation of columnar atypical cells with cell cytoplasm and complex glandular structures with abundant desmoplastic stroma, a morphological resemblance to fetal lung and significant tumor necrosis (Fig. , ). Conventional lung adenocarcinoma, another histological component and morulae were not found. Periodic acid Schiff (PAS) staining demonstrated glycogen in the cytoplasm of the neoplastic cells. Stains for mucin were negative. The immunohistochemical analysis showed positivity for AE1/AE3, CEA, and alpha-fetoprotein (AFP) (focal) (Fig. ). Thyroid transcription factor-1 (TTF-1), chromogranin A, synaptophysin, CD56, and p40 were negative. The beta-catenin result was positive, predominantly in the cell membrane (Fig. ). The final histopathological diagnosis was H-FLAC.
Both lymphatic and vascular invasion were detected. Microscopically, all margins were free and mediastinal lymph nodes (#5) showed metastasis. The final pathological staging was stage IIIA (pT2aN2M0). Both epidermal growth factor receptor (EGFR) mutations and anaplastic lymphoma kinase (ALK) gene rearrangements were negative. The expression of programmed cell death 1 ligand 1 (PD-L1) (Dako, clone 22C3) by resected specimen was negative.
The patient received cisplatin plus vinorelbine as adjuvant chemotherapy, but it was canceled at two courses because of the dehydration and hyperglycemia induced by the chemotherapy. At 25 months after the surgery, the patient had relapsed pleural dissemination. Postoperative serum CEA and AFP was 1.5 and 3.7 ng/mL, respectively. But, serum CEA at the time of relapse was 132.4 ng/mL, and AFP was unfortunately not searched. |
pmc-6032015-1 | A healthy 40-year-old man presented at the emergency room after a high energetic accident with multiple fractures and comprehensive soft tissue defects on his left lower leg and foot. See . After clinical examination in combination with X-ray images and CT-scan of his left lower leg the following fractures were identified: a Gustilo grade IIIB fracture, a medial malleolus fracture, a Tillaux-Chaput fracture, a nutcracker fracture of the cuboid, an avulsion fracture of the navicular bone, fractures of the third, fourth and fifth metatarsal head, a mid-shaft fracture of the third proximal phalanx and base fractures of the first and second proximal phalanx. See . In the CT-scan of the left lower leg and foot is displayed. Active extension and flexion of the toes were preserved, as well as sensibility in the toes and forefoot. Disturbed sensibility to touch and pain was identified at the foot sole. No signs of a compartment syndrome were found.
Within the first thirteen hours after admission placement of an external fixator and debridement of the proximal medial wound was conducted. The soft tissue defects were temporarily covered with EpigardTM (Biovision, Ilmenau, Germany) and absorbent dressings. Within the first 24 hours postoperatively NIRF imaging was performed. See and .
The imaging head of the FluobeamTM (Fluoptics, Grenoble, France) was positioned approximately 8 inches above the skin. Real-time images were obtained during 120 seconds, starting 10 seconds after intravenous injection of 2 mL (5 mg) of ICG. The fluorescent region (white area) indicates the area of vital tissue. Regions of no fluorescence (black area) and less fluorescence (surrounding grey area) were marked to indicate the area of non-viable tissue.
The patient underwent a second debridement approximately 2.5 days after initial admission. The remaining defects were covered with EpigardTM and absorbent dressings. Postoperative non-viable skin was marked again guided by NIRF imaging. Remarkably, a slight increase of the fluorescent area was visualized at the lateral malleolus. A final debridement was performed 6 days after hospital admission in the same session as definitive soft tissue coverage. The remaining avital soft tissue was removed under real-time intraoperative NIRF image-guidance. Intraoperative imaging showed no difference in the extension of the fluorescent area compared to the previous NIRF imaging. A free anterolateral thigh flap was harvested to cover the defect on the medial malleolus and foot sole. Additional split skin graft was placed to cover the superficial defect at the lateral malleolus. No complications were recorded and the patient was discharged after five weeks. Nine weeks postoperatively the patient and flap were doing well. Clinical rehabilitation care is completed and he mobilizes with crutches. See for illustration of the clinical course of the lower leg and foot. |
pmc-6032380-1 | A 50-year-old male patient underwent right lobe liver transplantation from a living
donor. The patient was in end-stage liver disease secondary to ulcerative colitis
and sclerosing cholangitis. Impairment in liver function tests and massive pretibial
edema developed three months after surgery. The patient’s fever was 38,5
°C, and he was hospitalized for treatment. In laboratory analysis, ALT levels
were 400 IU/L; AST levels were 300 IU/L; GGT levels were 118 IU/L; direct bilirubin
levels were 0,7 mg/dL; and the white blood cell count was 25000/µL (neutrophils
82 %). Meropenem 3 × 1 gram and teicoplanin 1 × 400 mg were started for
initial treatment. A pseudoaneurysm measuring 8 × 6.5 cm and having central
turbulent flow with peripheral thrombus in CDUS was detected in the portal hilus
associated with the hepatic artery (Figure ).
MDCT angiography was applied for a better anatomic orientation; the pseudoaneurysm
originated from the hepatic artery anastomosis site and extended anterior to the
artery. The central portion of the HAP was filling with contrast media while the
peripheral portions were thrombosed (Figure ).
The hepatic artery was 2 mm in diameter, and string-like narrowing was observed
distal to the pseudoaneurysm best seen in the arterial phase of the examination. A
loculated fluid collection around the pseudoaneurysm extending to the
subdiaphragmatic and perihepatic spaces was also observed (Figure ). In the follow ups, total bilirubin increased
to 9,3 mg/dL, direct bilirubin increased to 6,6 mg/dL, and the white blood cell
count was 30000/µL. The patient was hemodynamically unstable. Stenting of the
hepatic artery was planned, but the patient died before the procedure. |
pmc-6032381-1 | We report the rare case of a 33 years old pregnant woman with an acute gestational obstructive pyelonephritis. A right ureteral stent was placed at the twelfth pregnancy week in a private institution with a good evolution of the symptoms.
When time came to remove the stent after the pregnancy, the urologist could not find it nor in the bladder, neither in the all urinary tract at ureteroscopy. A thoraco-abdomino-pelvic computed tomography (CT) was performed and showed that the stent had moved intravascularly. It had migrated through the right iliac vein into the inferior vena cava and right atrium (Figs. , ). The patient was sent to our institution for extraction.
The patient complained about insidious onset of palpitations and moderate right flank pain. She had no macroscopic hematuriprobably due to intracatheter blood clotting.
The patient was then transferred to the vascular surgery department and an endovascular extraction was performed by puncture of the femoral vein. Extraction of the endoprosthesis was made by a curved guide introduced through the vena cava up to the right atrium under fluoroscopic control. The guide was subsequently twisted around the stent and pulled it out. No other complication was noted and the patient was discharged on the following day. |
pmc-6032430-1 | A 41-year-old man presented at the emergency department with complaints of
intolerable pain in the left upper quadrant. He had undergone extracorporeal shock
wave lithotripsy (ESWL) eight hours prior as treatment for a left-sided kidney stone
in the upper pole. Ultrasound demonstrated a large subcapsular hyperechoic
collection in the spleen (Figure ). A
computerized tomography (CT) scan confirmed a laceration of the lower pole of the
spleen with a subcapsular hematoma and a discrete amount of surrounding free fluid.
Adjacent to the splenic laceration, a smaller subcapsular hematoma was also present
in the left kidney (Figure ). In the meantime,
a fragmented stone in the proximal left ureter was visualized (1400 HU). The therapy
was conservative with hemodynamic follow-up in the intensive care unit with normal
patient recovery |
pmc-6032466-1 | An 81-year-old man with an history of Alzheimer’s disease, excess weight and type-2 diabetes presented to the emergency department with a two-day history of diffuse abdominal pain. Contrast-enhanced abdominal computed tomography (CT) showed diffuse fluid distention of the small bowel loops indicative of obstruction. In addition, there was a profusion of dilated small bowel diverticula along the mesenteric border (Figure – coronal posterior and – more anterior views. White stars indicate the diverticula). An isolated jejunal diverticulitis was diagnosed in the right flank (Figure – axial, – coronal, and – sagittal views. White stars indicate the distended inflammatory diverticulum and white arrows show the surrounding inflammatory fat stranding). The cause of the small bowel obstruction was a 3 cm large enterolith impacted in the distal ileum (white arrows on Figure – sagittal and – axial views). A Meckel’s diverticulum was also incidentally discovered on the antimesenteric border of the ileum (black arrow on Figure ). Small bowel obstruction caused by the release of an enterolith from jejunal diverticulitis in the context of Diffuse Jejuno-Ileal Diverticulosis (DJID) was the final radiological diagnosis.
At the time of laparotomy, the enterolith had migrated further than the Meckel’s diverticulum, causing opportune dilatation of this diverticulum. Surgeons then performed an elective resection of the Meckel’s diverticulum wherein the enterolith could be manually and proximally retrogradely pushed. The inflamed jejunal diverticulum was also resected. The post-surgical period was uneventful. |
pmc-6032468-1 | The patient is a 66 years old male patient who had prior spine surgery 15 years ago for a right L4-L5 disc herniation with initial good clinical results. Three years later, he complained of pain recurrence in the right L5 territory. Imaging at that time showed no new disc herniation and he was referred to the pain clinic. Several intra-foraminal L4-L5 and L5-S1 corticosteroid injections were performed during the last 10 years, as well as percutaneous radiofrequency denervation at the L4-L5 and L5-S1 facet joints. Long term complains were moderate.
In June 2014, the patient complained of a very severe bilateral L5 sciatalgy with paresthesias but no motor dysfunction. Mictional delay was also reported.
MRI performed in July 2014 shows a disc herniation originating from the L4-L5 disc. It perforates the common longitudinal ligament and the anterior aspect of the dura, and enters the thecal sac (Fig. and ), The intradural “part” of the herniation is very large and extends downwards (Fig. and ), reaching the level of the upper sacrum, the disc fragment being surrounded by the rootlets ( to Fig. ). The upper part of the disc fragment, at the level when it enters the dura, is “compact”, as usually seen in extradural disc herniations (Fig. ), and some peripheric contrast enhancement can be seen after gadolinium injection, while the intradural part seems to be less compact, with a “crumbled” appearance (Fig. and ), irregular borders and no contrast enhancement.
Surgery was performed in prone, antilordotic position under general anesthesia, via hemilaminectomy L4 and L5 on the clinically dominant right side with adjacent hemiflavectomy L3/4 and L5/S1, making a sharp parasagittal dural opening possible from a normal aspect of the dural sac into a heavily, scar tissue related metaplastic part of it (as often seen in reoperations and accountable for a higher risk of dural leak). The herniation was plain to see inside the dural sac, clearly causing a compression of most adjacent rootlets, but not really adherent to them (maybe due to the contact of cerebro-spinal fluid).
The neurosurgeon found the disc fragment in the spinal fluid to be “spongious, more friable and softer than the usual extradural herniation (Fig. and ).
Patient was discharged asymptomatic on day 5 and had an uneventful recovery. |
pmc-6032477-1 | A 47-year-old woman was referred to the imaging department for Computed Tomography Angiography (CTA) of the supra-aortic arteries in order to investigate the cause for systemic blood pressure asymmetry. Prior Doppler ultrasonography of the cervical arteries had failed to demonstrate the vertebral arteries.
CTA (Figure ) confirmed major hypoplasia of the vertebral arteries (red and white arrowheads on a) especially on the left side (red arrowhead). These hypoplastic vertebral arteries were not connected to the basilar artery (blue arrowhead on a) which was only fed by a large ascending artery (white arrows on Figures and ) emerging from a large left internal carotid artery (green circle). 3D volume rendering views without bone removal (Figure and ) showed that this atypical artery was penetrating the skull through the hypoglossus canal (red circle on Figures and ). The artery was clearly identified as a persistent hypoglossal artery. The finding was considered as fortuitous. |
pmc-6032479-1 | An 80-year-old woman with a history of subarachnoid haemorrhage was brought to the emergency room because of speech problems. The patient was found in the morning by her relatives with severe speech comprehension and production difficulties. The exact time of symptom onset was unclear. Clinical examination at the ER revealed a sensory aphasia without other neurologic deficits. Stroke was suspected and the patient underwent a brain CT with an optimized stroke protocol consisting of an unenhanced CT, perfusion CT, and a CT angiography. Unenhanced CT showed no abnormalities, apart from already known and unchanged chronic hypodense gliotic changes in the left temporo-insular region (Figure ). Perfusion CT showed a normal to slightly diminished mean transit time (MTT), a diminished time to drain (TTD) and a clearly increased cerebral blood flow (CBF) and cerebral blood volume (CBV) in the entire left parietotemporal region (Figure ). No intra-arterial clots were seen on CT angiography. Urgent brain magnetic resonance imaging (MRI) was performed. Diffusion weighted images (DWI) showed subtle diffusion restriction in the left parietotemporal region, corresponding with the area of hyperperfusion on CT, without accompanying signal alterations on FLAIR (Figure ). Based on the clinical presentation and imaging findings, a nonconvulsive status epilepticus or postictal Todd’s paresis was suspected. An Electroencephalography (EEG) was performed within two hours of the MRI. Only interictal epileptiform changes were seen with sharp theta waves and spike waves in the left temporal region. These EEG findings were not compatible with an ictal state – seizure or status epilepticus – as the discharges were neither rhythmic nor continuous. Therefore, a diagnosis of postictal sensory aphasia was made, presumably after a focal seizure involving Wernicke’s area. An antiepileptic treatment with levetiracetam was started. There was gradual improvement of the patient’s speech difficulties over three days and she was discharged home after six days without residual symptoms. |
pmc-6032481-1 | A 67-year-old woman was admitted for retro-orbital and periorbital pain, increasing over 1 week and accompanied by progressive exophthalmia and palpebral ptosis on the left side. She was being treated for lung cancer metastasized to the liver and cerebellum. Physical examination revealed a complete ptosis of the left eyelid and complete absence of extraocular movements of the left eye, referable to the third, fourth and sixth cranial nerve. Head computed tomography (CT) showed mucous thickening of the left sphenoid and maxillary sinuses. Brain MRI revealed an expansive lesion involving the left cavernous sinus (Figure ), which proved to be a new occurrence by comparison with a contrast-enhanced CT performed several weeks earlier. The lesion in the cavernous sinus appeared to be inseparable from T2-hypointense changes in the posterior part of the left sphenoid sinus (Figure ,), whereas the remaining wall thickening of the ethmoidal, sphenoid and maxillary sinuses appeared to be smooth and T2-hyperintense (Figure ). The lesion in the cavernous sinus showed marked enhancement on contrast-enhanced T1WI and seemed to be inseparable from the carotid artery (Figure ). Time-of-flight MRA (TOF-MRA) confirmed the presence of a saccular aneurysm of the cavernous portion of the left internal carotid artery, with evidence of turbulent flow within the aneurysm and with expansion of the aneurysm toward the left side (Figure ,). In addition, circumferential wall thickening of the parent carotid artery was seen on T2WI immediately proximally (Figure ) and distally to the aneurysm (Figure ), with a corresponding concentric narrowing of its lumen visible on MRA. Endoscopic sinus surgery was performed with removal of mucus and fungal debris, which was confirmed as aspergilloma on pathologic examination. The diagnosis was made of a mycotic aneurysm of the intracavernous carotid artery resulting from local spread of invasive aspergillosis from the adjacent sphenoid sinus, secondary to an immunosuppressive status resulting from chemotherapy and longstanding systemic corticosteroid treatment. Because of the dismal prognosis of the malignancy and contraindication of antiaggregative drugs for reasons of a single hemorrhagic metastasis in the right cerebellar hemisphere, the multidisciplinary decision was made to refrain from endovascular treatment. The patient died a few weeks later. |
pmc-6032484-1 | An 8-year-old boy with a history of 5 years of acute lymphoblastic leukemia was referred for radiographic evaluation of the pelvis because of left hip pain and a limp. The patient had undergone radiation therapy of the total body and bone marrow transplantation at age 6. Since the onset of the disease he had been receiving intravenous and oral corticosteroids. Three months post-transplantation, the patient developed lung disease secondary to graft- versus-host disease and received high doses of intravenous and oral steroids. Subsequently, the patient developed autoimmune hemolytic anemia and was treated again with high doses of intravenous and oral steroids.
Physical examination revealed a small for his age Cushingoid boy with severe loss of muscle bulk due to steroid-induced myopathy. His height was 47 inches, which lies between the 3rd and 9th centile and his weight was 54.3 lb, which lies between the 24th and 50th centile. Radiographic evaluation of the spine and hips revealed compression fractures in thoracic spine (T11, T12) and mild deformity of the femoral epiphyses (Fig. ). Because of the history of long-term corticosteroid use, BMD was measured in the lumbar spine (L1-4) [0.485 g/cm2, Z-score -1.97] and proximal femur [0.481 g/cm2] using DXA (Fig. ). Trabecular BMD was measured at the 4% distal radius using peripheral quantitative computed tomography (pQCT) [109.5 mg/cm3, Z-score -2.2]. On the basis of established pediatric densitometric criteria [], a diagnosis of low bone mineral content was made and patient was started on treatment with vitamin D and calcium. Follow-up DXA studies to determine response to treatment at 12 months demonstrated collapse of the femoral head and increasing BMD (Fig. ). The lumbar spine and hip bone densities were 0.542 g/cm2, Z-score -1.46 and 0.567 g/cm2, respectively. Sequential follow-up DXA scans at 24 months showed further destruction of the femoral head and an increase in bone mass (Fig. ). The lumbar spine and hip bone densities were 0.591 g/cm2, Z-score -1.35 and 0.788 g/cm2, respectively.
Trabecular BMD at the 4% distal radial site by pQCT at 12 and 24 months was 135.8 mg/cm3, Z-score -1.6 and 133.7 mg/cm3, Z-score -1.9, respectively. The concurrent visual assessment of the DXA images showed progressive deterioration of the left hip joint, beginning as vague osteosclerosis and becoming severe with destruction of the femoral head, suggesting the diagnosis of osteonecrosis. |
pmc-6032485-1 | A 15-year-old girl who attempted suicide using sulfonylurea, an oral hypoglycemic drug and subsequently became comatose was admitted to the pediatric emergency department. The patient had no known previous disease. On admission, vital signs (blood pressure, pulse, respiratory rate, and temperature) were normal. On neurological examination, the patient’s pupils were isochoric and reacted to light promptly. Corneal and oculecephalic reflexes were intact. The patient showed responses to painful stimuli. Except for a blood glucose level of 10 mg/dl, all other blood laboratory tests (complete blood count, electrolytes, liver and renal functions, arterial blood gases) were normal. Intravenous dextrose was injected immediately, followed by glucose infusion and somatostatin treatment to restore the blood glucose level. As there was no doubt about the use of the oral hypoglycemic drug for suicidal purpose, a toxicology test was not performed.
One hour after admission, a T2-weighted image on MRI (1.5 Tesla Siemens Magnetom Symphony Quantum, Erlangen, Germany) showed isolated focal hyperintensity in the SCC and a decrease in the apparent diffusion coefficient (ADC) compatible with diffusion restriction, in addition to a significant signal increase in the DWI (Fig. ). After receiving treatment for 24 hours, the patient became conscious, and her blood glucose level returned to normal (90 mg/dl). DWI was repeated two days after the first MRI, and a control DWI (Fig. ) showed complete resolution of the lesion in the SCC. |
pmc-6032491-1 | A 63-year-old man followed up for diabetes mellitus, hypertension and recently increasing vertigo with bilateral murmer at his neck. The patient had no neurological deficit and referred to radiology for the examination of cerebrovascular diseae. Bilateral carotid color duplex Doppler examination showed mild atherosclerotic plaques of the common carotid bifurcation without any hemodynamically significant stenosis. Cerebral MRI revealed chronic ischemic signal changes in pons and nonspesific signal changes in white mater of bilateral frontal lobes. Selective carotid and vertebral angiographies were performed in order to confirm the stenoses in proximal parts of both internal carotid arteries; occipital arteries were arising from the cervical segments of internal carotid arteries on both sides (Fig. ). Selective right vertebral angiography revealed the patency of the artery with hypoplasia of V3–V4 segments and intracranial and intervertebral collateral flow to the left vertebral artery (Fig. ). Left subclavian angiography prior to catheterization of vertebral artery demonstrated severe stenosis of the vertebral artery. Left vertebral artery is also opacified through the anastomoses between the muscular branches of occipital and vertebral arteries (Fig. ). A baloon-expandable intravascular stent was placed to the ostium of the left vertebral artery in order to treat the high grade stenosis (Fig. ). |
pmc-6032495-1 | A 49-year-old woman complains of new onset episodes of vertigo post stapedectomy 12 years ago. Symptoms can be evoked by pressing or when lying on the left ear. No remarkable findings on ear inspection or clinical exam. Epley maneuvers had no effect for treatment of possible benign paroxysmal positional vertigo (BPPV). Other differential diagnoses were perilymphatic fistula, intravestibular granuloma, labyrinthitis and stapes prosthesis protrusion.
A temporal bone high resolution CT (HR CT) was performed and showed migration of the stapes prosthesis into the vestibule (protrusion) (Fig. ). Depth of protrusion was measured 14 millimeters from tip of the prosthesis to the oval window. The diagnosis of intravestibular stapes prosthesis protrusion related post stapedectomy vertigo was made and revision surgery was proposed. |
pmc-6032496-1 | A 65-year-old woman presented with a three-month history of persistent ocular-nasal catarrh, sub-febrile state, myalgia, night sweats, weight loss and early daily bi-temporal pain. Severe biological inflammatory syndrome with CRP at 105 mg/l and sedimentation rate at 97 mm was present. Colour Doppler Ultrasound (CDU) (Figure ) showed a typical inflammatory “halo sign” (black arrowhead on a) of the temporal arteries. A similar “halo sign” was found along the common carotid (white arrows on c, d, and e) and vertebral arteries (black arrows on d and e). The intima remained visible (white arrowheads on e). Computed Tomography (CT) angiography (Figure ) showed blur homogeneously enhancing wall thickening of the aortic arch (white arrows on a and d) and of its large emerging arteries (white arrows on b). The axillary arteries were also affected (black arrows on a). Moderate thickening of the abdominal visceral aorta was also found (not illustrated). Hypodensity of the intima contrasted with enhancement of the inflamed media (white arrowheads on b). Temporal artery biopsy (Figure and ) confirmed typical giant cell arteritis (GCA) with involvement of the media (yellow star) and adventice (white star) by chronic lymphocytic inflammation. Reactive intimal hyperplasia (black star) causing luminal collapse (white arrow), characteristic fragmentation of the internal elastic lamina (black arrows) and giant cells (black circles) were also diagnosed. Classical massive corticosteroid treatment was immediately started with rapid clinical and biological improvement. The “halo sign” and diffuse arterial thickening had, drastically, nearly completely resolved on post-therapeutic CDU (Figure and ) and CT (Figure and ) nine months later. |
pmc-6032525-1 | A 64-year-old woman was admitted to the neurosurgery department for V2–V3 right- sided trigeminal paresthesia, which had developed six months earlier. Magnetic resonance imaging (MRI) showed a well-circumscribed, fleshy, 36 mm tumor in the Meckel cavum involving the Gasserian ganglion, following the V3 nerve into the foramen ovale, and generating a mass effect on the temporal lobe. This lesion was discreetly hyperintense on T1-weighted images and iso- to hypointense on T2-weighted images, and it showed a slightly heterogeneous enhancement after contrast administration. Small cystic areas were noted. Diffusion-weighted imaging was negative. An abnormality was already seen on a computed tomography (CT) performed two years previously in the context of otitis, without any alarm in relation to the benign presentation, a well-defined petrous lacunar image, and the fortuitousness of the discovery (Figure ). Diagnosis of schwannoma was proposed, without specification. The patient underwent a macroscopically complete surgical resection by an exclusive extradural subtemporal approach with excellent symptomatic recovery. The postoperative CT demonstrated no complications. Immunohistochemical examination finally specified the diagnosis of MS, a very rare pigmented tumor (Figure ). The clinical course was good. However, a recurrence was detected on the MRI performed at three months. 11C-methionine positron emission tomography (MET-PET) confirmed suspicions (Figure ), and a new surgical intervention was planned followed by adjuvant radiotherapy. |
pmc-6032537-1 | A 32 year old female patient presented to the cardiology clinic with an atypical chest pain. Her history revealed no other condition than Leopard syndrome which was diagnosed on her birth. However, her family history revealed that her older sister who also had Leopard syndrome, was diagnosed with pulmonary stenosis 5 years ago and underwent successful baloon valvuloplasty. The patients mother, who also had Leopard syndrome had undergone open heart surgery due to pulmonary stenosis 20 years ago and 5 years earlier she had had percutaneous coronary artery angioplasty due to coronary artery disease. On her physical examination; pectus excavatum, multiple lentiginous lesions on the face and hands (Figure ), ocular hypertelorism, and short height were detected. Sinus rythm and left branch block was detected on her electrocardiography. Her echocardiography was as follows: left ventricular ejection fraction 60%, normal heart chambers, atrial septal defect (4mm) (Figure ), low grade pulmonary valve stenosis (maximal gradient 18 mmHg, mean gradient 9 mmHg), right ventricular pressure 20 mmHg.
On her coronary CT angiography, atrial septal defect, 3 mm in its widest place, widened left pulmonary artery (diameter 34 mm), mild thickening of the pulmonary semilunar valves were detected. The right coronary artery was dominant and diffusely ectatic (diameter: 7.5 mm widest). The right ventricular branch of the right coronary artery was directly originating from the sinus of Valsalva and this branch also gave the conus branch. PDA and PLB reached the apex through the interventricular sulcus. LMCA originated from the right coronary sinus and had a prepulmonic course (Fig. ). LMCA (6,5 mm) and LAD (6,2 mm) were ectatic throughout their course. Circumflex artery structure and callibration were normal. The structures of the large cardiac vein and coronary sinus were normal. The anterior interventricular vein was draining directly to the left atrium.
Coronary angiography revealed similar findings as the coronary CT angiography. Maximum gradient of 20 mmHg was observed in pulmonary valve hemodynamic study. As a result of the investigations it was decided to follow up the patient with medical treatment. |
pmc-6032547-1 | A 39-year-old woman was admitted to our hospital presenting with dizziness and cerebellar ataxia.
Computed tomography (CT) scan revealed a large partially cystic mass in the left cerebropontine angle (CPA) with calcifications, compressing the brainstem and the cerebellum (Fig. ).
MR imaging (Figs. , ) confirmed the presence of a large mass in the left CPA. The mass had two components, cystic and solid. The cystic component of the mass was located in the left CPA, extending anteriorly to the 7th cranial nerve and medially compressing the brainstem. It showed low signal intensity on T1W and high signal intensity on T2W ones and with no enhancement after intravenous administration of contrast media. On DW images the mass displayed high signal intensity, ADC 0.8 and the spectral pattern showed a medium lipid/lactate peak at 1.3 ppm, findings typical for IEC. The juxtaposed solid component of the mass compressed the cerebellum causing perilesional edema. It showed low signal intensity on T1W and T2W images. After intravenous administration of contrast media, it displayed heterogeneous, mainly peripherical and in some sites nodular enhancement. DW imaging showed low signal intensity inside the mass and ADC values of 1.34. MRS revealed a high lipid/lactate peak, Choline/Creatine ratio (Cho/Cr) 2.13 and Choline/N-Acetylaspartate ratio (Cho/NAA) 0.766.
Surgery was planned and the mass was completely resected. Histopathologic analysis (Fig. ) revealed that the cystic component was consistent with benign IEC. The adjacent solid component consisted of squamous epithelial cells, with nuclear pleomorphism and mitotic activity. The superficially lined cells were filled with laminated keratin. There were also islands of squamous epithelium with an infiltrative growth pattern. |
pmc-6032555-1 | A 31-year-old nonsmoking woman, complained of dyspnea and polyarthralgia following a cesarian section. She had no fever, no sputum production, and no cough. The patient worked as domestic help. Physical examination was normal, without auscultation abnormalities. Bloods tests showed D-dimer elevation and a slight hypereosinophilia (780/mm³).
A CT angiogram revealed no evidence of pulmonary embolism. However, diffuse groundglass centrilobular nodules without tree-in-bud pattern were observed (Figure ), in association with small centrilobular apical emphysema (white arrow Figure ) and confluent condensed areas in the basal segments of the lower lobes (black arrow Figure ). The first diagnostic hypothesis was hypersensitivity pneumonitis, though there was no air-trapping on the CT. The etiological investigation failed to find any causative agent for this pathology.
Cytology of bronchial alveolar lavage (BAL) showed 16% of neutrophils, 29% of lymphocytes, and 55% of macrophages. There were no eosinophils found. A surgical pulmonary biopsy was performed in order to assess the interstitial pathology. Microscopic examination revealed an important non-caseating granulomatous interstitial inflammation, with lymphocytes (black arrow Figure ), numerous macrophages and multinuclear giant cells, sitting preferentially in the peribronchiolar regions (black star Figure ). These granulomas contain characteristic needle-shaped birefringent crystalline material in polarized light (black arrow Figure ). The morphological aspect of these crystals are similar to that of the talc.
A second patient history was carried out. The patient admitted to using abundant cosmetic talcum powder in order to soften the skin daily over several years. She denied any intravenous drug abuse. Based on the patient’s history and the clinical, radiological, and histological findings, the diagnosis of talc induced interstitial lung disease (talcosis) was made based on massive use of talc powder. |
pmc-6032558-1 | A 53-year-old woman with invasive ductal breast carcinoma diagnosed 4 years previously was admitted to our hospital with a history of swelling in her left EAC for 2 weeks and a gradually worsening hearing loss. The patient had undergone right radical mastectomy and followed chemoradiotherapy following the diagnosis. The primary tumor was multifocal (5 different focuses) and located in upper and lower outer quadrants of the breast. The size of the largest tumor focus was 2.2 cm. The immunohistochemical study showed positive Estrogen Receptor. All of the 33 dissected axillary lymph nodes were free of metastasis. Post surgical staging was stage IIB. In follow up, bone and lung metastases appeared and relevant treatment was performed. Initially, she had noticed a moderate hearing loss in her left ear, but mentioned no otalgia, otorrhea, tinnitus, or vertigo. Physical examination revealed complete obstruction of the left EAC by a soft tissue mass. No neurologic symptoms were observed. HRCT of the temporal bone showed that totally obstructed left EAC by soft tissue density mass, but no bony erosion or destruction (Fig. ). MRI showed well defined, fusiform, soft tissue mass entirely filling the left EAC. The mass was iso-intense with muscle on T1-weighted images, slightly hyperintense on T2-weighted images. Following the administration of the gadolinium based contrast agent homogeneous enhancement was seen. Although the mass was located in both bony and cartilaginous EAC, there was no sign of bony or cartilaginous invasion (Fig. ). DWI showed slightly hyperintense signal on isotropic trace image and the apparent diffusion coefficient (ADC) map revealed slightly restricted diffusion in the mass. There was a small, non-enhancing hemorrhagic fluid collection, which is hyperintense on both T1- and T2-weighted images, between the mass and the tympanic membrane. The middle and internal ear structures were normal on both MRI and temporal CT images. We reported that the mass may be consistent with primary tumor of the EAC, but due to the history of metastatic breast carcinoma, mentioned EAC metastasis in the differential diagnoses.
The patient underwent surgical operation by the ENT surgeon and the mass was subtotally removed. Histopathologic examination of specimens revealed metastatic carcinoma that has same histopathologic features with the primary breast carcinoma. Histopathological finding of the excised lesion showed microscopically a infiltrative carcinoma compatible with breast origin (Fig. ) based on immunohistochemical study result that was positive for Estrogen Receptor (ER), Cytokeratin 7 and E-cadherin (Fig. ) but negative for C-Erb-b2 and Progesterone receptor (PR). Tumor was composed of atypical cells with large nuclei and prominent nucleoli in solid pattern. There was no ductal or tubular structure. Positivity of both ER and E-Cadherin also supported ductal type when compared to lobular carcinoma of the breast. |
pmc-6032563-1 | The patient was a 50-year-old woman with a chief complaint of edema. She had previously been healthy, and previous physical examinations revealed no abnormal urinalysis results. She experienced fever, cough, malaise, and low back pain for 6 days and edema of the face and limbs for 5 days before visiting our hospital. She was diagnosed with type B influenza by a local physician 3 days prior to visiting our hospital. The patient later exhibited an exacerbation of facial edema, decreased urine output, and a high level of proteinuria. She was subsequently diagnosed with nephrotic syndrome and was referred to our department.
The physical findings on admission were as follows: height, 150.1 cm; weight, 48.3 kg; body mass index, 21.4; body temperature, 36.2 °C; blood pressure, 109/83 mmHg; and oxygen saturation on room-air, 99%. In addition, the patient experienced facial edema and pitting edema of both lower legs. The laboratory findings on admission were as follows: total protein, 5.2 g/dL; albumin, 2.0 g/dL; blood urea nitrogen, 13 mg/dL; creatinine, 0.84 mg/dL; estimated glomerular filtration rate, 56.5 mL/min/1.73 m2; total cholesterol, 308 mg/dL; immunoglobulin G (IgG), 1320 mg/dL; IgA, 354 mg/dL; IgM, 192 mg/dL; IgE, 2966 IU/mL; complement 3, 83.3 mg/dL (normal range 65–141 mg/dL); complement 4, 40.9 mg/dL (normal range 13–40 mg/dL); and total hemolytic complement (CH50), 40.5 U/mL (normal range 31–48 U/mL). The patient was negative for hepatitis B virus surface antigen, hepatitis C virus antibodies, and human immunodeficiency virus antibodies. She had previously been infected with parvovirus B19. Her proteinuria level was 20.88 g/gCr, her selectivity index value was 0.13, and her urine sodium level was 8 mEq/L. Urine sediments showed 1–4 red blood cells per high-power field without any abnormal casts. There were no notable abnormalities electrocardiography or chest X-ray images.
A kidney biopsy performed on the fourth day of hospitalization under suspicion of MCNS, IgA nephropathy, or post-streptococcal acute glomerulonephritis did not show glomerular basement membrane thickening, or endocapillary or mesangial cell proliferation (Figs.
and ). An immunofluorescence study showed nonspecific granular IgM deposits in the mesangium. Electron microscopy showed extensive foot process effacement, but immune complex deposits were not observed (Fig. ). Based on the above findings, the patient was diagnosed with MCNS.
After admission, the patient’s proteinuria decreased to 0.06 g/gCr with rest and sodium restriction (6 g/day) alone, and a complete remission from nephrotic syndrome was observed at approximately 2 weeks after the onset of symptoms. No recurrence of nephrotic syndrome was observed at an outpatient visit one month later (Fig. ). No recurrence has been noted in the one years since. |
pmc-6032566-1 | An 80-year-old man with progressive pain lasting for 5 days and with focal swelling of the left foot was referred to the radiology department. Wearing shoes aggravated the pain. Inspection of his footwear showed a bump at the inner sole, corresponding with the location of the clinical abnormality at the heel of the patient. Clinically, there was suspicion of plantar fasciitis.
Plain films showed the absence of inferior calcaneal spur formation. Ultrasound revealed a normal plantar fascia. Medially from the plantar fasciitis, adjacent to the course of the medial plantar artery, hypoechoic tubular structures were seen, which were not compressible. There was no intralesional flow on color Doppler imaging (Figure ). Comparison with the right foot showed normal compressible veins. Subsequent MRI confirmed thrombosis of the medial plantar veins (Figure ).
The symptoms disappeared after conservative therapy with change of footwear and non-steroidal anti-inflammatory drugs. Follow-up ultrasound 2 months later revealed no residual thrombus in the plantar veins. |
pmc-6032606-1 | A 19-year-old man was admitted in the intensive unit after a suicide attempt by hanging. He had been found in cardiac arrest of imprecise duration. Cardiac massage was performed for 40 minutes during transfer. The patient was in a deep coma with Glasgow score at 3/15. Unenhanced Brain Computed Tomography (CT) performed after five hours (Figures and ) demonstrated bilateral hypodensity of the basal ganglia (white arrowheads). Hyperdensity of the cerebral arteries seemed to be related to the underlying brain hypodensity, with decrease in gray-white differentiation due to edema. Unenhanced CT after 30 hours (Figures and ) showed progression of brain edema with collapse of the sylvian fissures, basal cisterns and cortical sulci (black arrows). Diffuse hyperdensity of all collapsed sub-arachnoid spaces had become prominent (white arrows) evocating pseudo-subarachnoid hemorrhage (PSAH). Multiple evoked potential confirmed brain dead after 48 hours. |
pmc-6032608-1 | A 48-year-old man with a history of alcohol abuse presented to the gastroenterology department for the first time in November 2006. He was suffering from severe epigastric pain and nausea for two days. Because the laboratory findings were suspicious for pancreatitis, the patient underwent computed tomography (CT) (Fig. ). The examination showed an edematous pancreas tail and fluid in the anterior prerenal space and in the anterior, posterior and lateroconal fascia. There were no signs of chronic pancreatitis or vascular complications. In the following months the patient had two similar episodes of acute tail pancreatitis.
In September 2009 he presented with vague epigastric pain of several weeks duration. Serum amylase and lipase levels were normal, however the cholestatic parameters were mildly elevated. Therefore, a magnetic resonance (MR) study was performed. The examination demonstrated moderate chronic pancreatitis of the tail with atrophy and irregular bording of the Wirsung’s duct. There were no other abnormalities. Further examinations also revealed a Helicobacter pylori gastritis.
In February 2012 the patient presented again to the gastroenterologist with epigastric and left upper quadrant pain. Laboratory findings were not suspect for acute pancreatitis. However, contrast-enhanced CT scan was performed in order to evaluate the known chronic pancreatitis and other causes of pain (Fig. ). This study surprisingly revealed a hypervascular lesion in the pancreatic tail consisting of a conglomeration of small hypervascular spots and blood vessels. Further examinations were performed in order to differentiate a vascular malformation from a hypervascular pancreatic tumor, especially an islet cell tumor or a hypervascular metastasis. Contrast-enhanced MR study showed a focal area of heterogeneous contrast enhancement without an apparent nodular component. The subsequent angiography showed two large feeding arteries in the early arterial phase, followed by a racemose vascular network, an early transient dense parenchyma stain in the early portal phase and early wash-out of the lesion in the portal phase (Fig. ). Based on these findings, a pancreatic arteriovenous malformation was suggested. The patient was treated with a resection of the pancreas tail and postoperative recovery was uneventful. Histopathologic examination revealed sequelae of chronic pancreatitis and numerous dilated blood vessels accompanied by blood clot formation and intimal hyperplasia (Fig. ). The histopathological findings were consisting with a pancreatic AVM. |