We present the case of a 12-year-old male who came to our emergency department complaining of progressive visual loss and intermittent frontal headache of moderate intensity, not accompanied by photophobia or ocular pain.
Symptoms started 2 weeks before, and had a progressively worsening course over the next days. Associated symptoms included apathy, accentuated in the last 3 days, nausea and 2 episodes of vomiting. He had no history of migraine headaches or any recent illness or fever. His medical records showed several episodes of recurrent laryngitis in the past.
The general examination was within normal limits, except for the blood pressure, which was 140/ 102+ mmHg. His weight was 81 kg, height 170 cm and BMI 28 kg/ m2.
On clinical examination, BCVA was 20/ 120 in the right eye and 20/ 200 in the left eye. The patient also presented a mild red-green color vision defect. Non-contact tonometry indicated an IOP value of 20 mmHg in both eyes. The patient had normal function of the cranial nerves III, V, VI and VII bilaterally.
Slit-lamp examination revealed a normal appearance of the anterior segment in both eyes. On fundus examination, an anomalous optic disc was noted with similar aspect in both eyes. Abnormal findings consisted of an elevated and highly hyperemic optic disc with blurred margins and filling the optic disc cup. Moreover, retinal and choroidal folds were present. Vascular changes included venous congestion of arcuate and peripapillary vessels, flame-shaped hemorrhages, cotton wool spots and hard exudates (). Macular examination showed no apparent changes.
An Optical Coherence Tomography was performed in order to assess the degree of severity of retinal damage. Macular examination revealed deformation of the foveal contour in both eyes and increased retinal thickness due to the accumulation of intraretinal fluid (). On tomographic examination of the optic nerve, changes included an artefactual increase of the thickness of the retinal nerve fiber layer (RNFL), a blurred margin of the papilla and a significant decrease of the topographic parameters of the excavation.
Humphrey Visual Field Analyzer perimetry was attempted and showed a mean deviation of -13.37 dB in the right eye with enlargement of the blind spot and a dense nasal field defect. In the left eye, the mean deviation was -16.81 dB and also showed an enlargement of the blind spot and a dense nasal field defect (). Also, both eyes presented unpatterned reduction of the light sensitivity.
An immunologic screening demonstrated negative anti-nuclear antibodies and anti-dsDNA in normal limits. A serologic infectious screening was performed and was negative for HIV infection, borreliosis, cat scratch disease, listeriosis and toxoplasmosis.
When he presented to our clinic, the patient had already undergone an MRI that showed mild ventriculomegaly with no apparent obstacle in the drainage of CSF.
We suspected the diagnoses of idiopathic intracranial hypertension and referred the patient to a pediatric neurologist for further workup and management.
In the first day of hospitalization in the pediatric neurology department, the patient developed diplopia due to abducens nerve palsy.
Cerebral angio MRI showed: mild internal tetraventricular hydrocephalus, periventricular white matter macrostructure alteration, a complete configuration of the circle of Wills without any cerebral arteriovenous malformation, a selar and orbital region with normal MRI signal; hypoplasia of the right transverse sinus.
The patient was put on a low-calorie diet. Mannitol 150 mg/ ml for 10 days was initiated, associated with Dexamethasone 8 mg/ 2 ml injection for 10 days, the latter being subsequently switched to Methylprednisolone 16 mg for 4 days.
The neurologist performed a lumbar puncture that showed an elevated opening pressure of 25 mmHg (34 cm H2O) and thus confirmed the diagnosis of intracranial hypertension. Subsequently, Acetazolamide 250 mg was added to the treatment in progressive highly dosage, from 250 mg/ day to 1000 mg/ day.
After 14 days of hospitalization, the patient was discharged with the following treatment: Methylprednisolone, Acetazolamide and proton-pump inhibitors.
We reevaluated the patient after his hospital discharge. BCVA was significantly improved, 20/ 20 in both eyes and normal ocular motility in both eyes with no diplopia.
Slit-lamp examination revealed a normal appearance of the anterior segment in both eyes. On fundus examination, we identified a significant reduction of the papillary edema, blurred margins of the optic disc but no elevation of the disc head and hard exudates in the foveolar and parafoveolar area. Also, the vascular configuration and calibre improved ().
An Optical Coherence Tomography was performed to assess the degree of severity of retinal damage. Macular examination showed no subretinal liquid, retinal thickness returned to normal values and hard exudates (hyperreflective dots) were present in the macular area (). On tomographic examination of the optic nerve, the average thickness of the retinal nerve fiber layer (RNFL) in both eyes was still above normal limits, with slightly blurring of the optic disc margins ().
On visual field examination, a significant improvement was noted: a mean deviation of -8.97 dB in the right eye with normal blind spot size and the persistence of a nasal defect. In the left eye, the mean deviation was -7.57 dB with some residual nasal and temporal defects ().


A 27-year-old woman presented with decrease of vision in her left eye, of acute onset and progressive course for 2 weeks. She had a history of a flu-like condition 2 weeks prior. She had no other history of any systemic condition.
Examination revealed a best corrected visual acuity of 20/ 400 in her left eye and 20/ 20 in the right one. Anterior segment exam using slit lamp was normal in both eyes. Examination of the left macula revealed cystoid edema of the macula, neurosensory detachment, flame shaped retinal hemorrhages inferior to her fovea, hard exudates at the edges of the neurosensory detachment, and two juxta-foveal yellowish peculiar lesions, nasal and temporal, to the fovea (). Examination of the periphery of the retina was unremarkable. Posterior segment exam of her right eye was completely normal.
Fluorescein angiography of the left fundus was done and showed blockage of background fluorescence by the superficial retinal hemorrhages with patchy areas of mild hyperfluorescence in the early phase (). Diffuse fluorescein leakage that was present at the level of the retinal pigment epithelium under the area of retinal hemorrhages appeared in the late phase () and increased in the very late phase (), with dye pooling also evident in the subretinal space.
Optical coherence tomography imaging of the macular area revealed cystoid macular edema, cystic spaces involving both the outer and inner layers of the retina, neurosensory detachment, and 2 hyperreflective juxta-foveal globular lesions visible in the outer retina, which were nasal and temporal to the fovea, that corresponded to the two juxta-foveal yellowish peculiar lesions seen on fundus examination. There was no evidence of a choroidal neovascularization ().
Laboratory investigations including complete blood picture, erythrocyte sedimentation rate, C-reactive protein, tuberculin skin testing, angiotensin converting enzyme level, rapid plasma reagin, antinuclear antibodies, and IgM antibodies against Bartonella and Toxoplasma, were all negative.
The condition of the patient gradually improved over several months on low dose systemic prednisone treatment with evidence of residual macular scarring and persistent moderate impairment of vision at the final follow-up visit.


A 45-year-old male was admitted to COVID-19 ICU with RT-PCR positive for COVID-19. The blood bank received a request for two units of packed red cells for the patient along with the patient's sample. The sample showed autoagglutination when received at the blood bank. The patient had no history of blood transfusion in the past.
The initial haemoglobin of the patient was 6.9g/dl, and C-reactive protein (CRP) was raised to 120.10mg/dl. There was a noticeable increase in CRP and a drop in Hb to 6.0g/dl coinciding with the worsening of pneumonia. Total leukocyte count was 5.98 X10 3/ microliter and platelet count was 30X 10 3 / Microliter. His course was further complicated by progressive thrombocytopenia, with a nadir of a platelet count of 20 X 10 3 / microliter. Reticulocyte count was 1.15%. Urea was elevated to 105.34mg/dl and creatinine to 2.01mg/dl. He developed renal failure and renal replacement therapy was advised. Alkaline phosphatase was 310.21U/L. Serum bilirubin was elevated to 7.76mg/dl, and unconjugated bilirubin was 2.91 mg/dl. Total protein was low (4.24g/L), Albumin was 2.03g/L. The presence of target cells and spherocytes in blood films was observed indicative of autoimmune hemolytic anemia (AIHA) []. The patient's sample was received in the blood bank for blood grouping and cross-matching. The results of cell and serum grouping showed ABO discrepancy (Table ).
The direct Coombs test was positive. Reverse grouping at 4 degrees Celcius showed increased agglutination of B and O cells, suggesting the presence of cold agglutinins. This antibody reacted with all patient and donor RBCs at cold temperatures. Both the Ethylenediaminetetraacetic acid (EDTA) tube and plane tube were kept at 37 degrees Celcius. Prewarmed saline kept at 37 degrees Celcius was used to wash one ml of red cells of the patient. Cell grouping was done with red cells washed with prewarmed saline in prewarmed tubes with anti-A, Anti B, and Anti AB. A 5% red cell suspension of A, B, and O cells were made and kept at 37 degrees. Patient serum was separated and kept at 37 degrees centigrade for 1 hr. An equal volume of serum and Red cells were incubated at 37 degrees for 45 minutes. The auto-adsorbed serum was used for cross-matching. A Prewarmed pipette tip was used for cell and serum grouping and cross-matching. The ABO Discrepancy was resolved with a warm saline wash of patient red cells and the use of prewarmed serum for serum grouping, and the results are elaborated in Table .
The direct Coombs test was positive. The blood group was Interpretated as B Positive. The patient had no history of previous transfusions therefore previous exposure to blood products and the presence of Red cell alloantibody was unlikely. Rh typing of the patient cell was done using the prewarming technique (Table ).
Crossmatch was performed using auto-adsorbed prewarmed serum by Tulip gel cards, and Rh matched compatible units were selected for the patient. Blood grouping was done by tube technique and extended Rh phenotype for ANTI-C, ANTI-c, ANTI-E, ANTI-e was done by Tulip reagents. A cold agglutinin titer was performed using pooled O Cells from donors, and phosphate buffer saline and patient serum separated and kept at 37 degrees Celcius. Serial dilution was prepared, and titer was negative at 37 degrees Celcius, the titer of 2 at room temperature, and a titer of 64 at 4 degrees Celcius was observed. This antibody reacted with all patient and donor RBCs at cold temperatures.
Rh compatible units were cross-matched with auto adsorbed serum of the patient, and compatible B Positive units were safely transfused. It was advised to keep the patient warm, monitor the patient during transfusion with a slow transfusion of blood products. The transfusion was uneventful. ABO Discrepancy was resolved after a warm saline wash of patient red cells, and prewarmed serum was used for serum grouping.


A 68-year-old woman presented to her family physician with fatigue and 5 kg of weight loss over a six-month period. Her past medical history was significant for a hiatus hernia and hypercholesterolaemia. Her regular medications included rabeprazole and rosuvastatin. She intermittently took cholecalciferol 1000 IU/day. Physical examination was unremarkable except for pallor.
The following instrument platforms were used in the initial investigation of the case: serum chemistry, including total calcium, phosphate, albumin, creatinine, and globulins, on the Vitros (Ortho Clinical Diagnostics, Rochester NY, USA); ionized calcium on the Radiometer ABL800 (Radiometer Medical, Brønshøj, Denmark); PTH and 25OHD on the Abbott Architect (Abbott Laboratories, Chicago, Illinois, USA); 1,25(OH)2D on the Diasorin Liaison XL (DiaSorin, Saluggia, Italy); and serum electrophoresis (sELP) on the Sebia HYDRASYS 2 (Sebia, Paris, France).
Initial investigations revealed haemoglobin of 87 g/L (115-160), corrected serum calcium of 2.83 mmol/L (2.1-2.6), serum albumin of 39 g/L (33-47), ionized calcium of 1.33mmol/L (1.13-1.3), and serum globulins of 61g/L (24-41). Further investigations in hospital showed an intact PTH of 4.9pmol/L (2.0-9.5), 25OHD of > 400nmol/L (50-150), 1,25(OH)2D of 152pmol/L (48-190), phosphate of 1.44 mmol/L (0.9-1.6), creatinine of 74 µmol/L (45-90), and an IgM kappa paraprotein in the gamma region of 44 g/L on sELP (). The PTH result was confirmed using the Beckman Coulter DxI 800 (Brea, California, United States).
A positron emission tomography/computer tomography scan showed extensive mildly fluorodeoxyglucose (FDG) avid lymphadenopathy above and below the diaphragm, with large FDG avid mass lesions in the pelvis, and widespread bone marrow infiltration. A bone marrow aspirate showed moderate to heavy marrow involvement with a mature lymphoproliferative disorder, consistent with a diagnosis of lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinaemia (LPL/WM).
The 25OHD level was felt to be inconsistent with clinical findings and the possibility of an aberrant result was considered.
Vitamin D results using different assays are summarised in . The steps taken to confirm interference are summarised in . The 25OHD result of > 400 nmol/L was confirmed on repeat analysis using the Abbott Architect immunoassay platform. In order to confirm our suspicions, a series of steps was taken. Firstly, a 1-in-2 dilution of the sample with Architect Multi-Assay Manual Diluent returned a result of 84 nmol/L – highly suggestive of the presence of an interferent. The presence of rheumatoid factor was excluded with a result of < 15 IU/L (reference range < 30 IU/L, Abbott Architect, Abbott Laboratories, Chicago, Illinois, USA). Treatment of the sample with antibody blocking reagent (Scantibodies, Santee, California, United States) was not consistent with heterophile antibody interference. Analysis of 25OHD via liquid chromatography tandem mass spectrometry (LC-MS/MS) returned a result of 82 nmol/L (Waters LC-MS/MS, Milford, Massachusetts, United States). Measurement of the sample on an alternative immunoassay platform (Siemens Centaur, Bellport, New York, United States), produced a result of 75 nmol/L, in agreement with the LC-MS/MS result. Following chemotherapy, and in the setting of normal globulins, the patient’s repeat 25OHD result on the Abbott Architect instrument was 64 nmol/L, consistent with the monoclonal paraprotein interfering with the original assay.
At the time of the patient’s initial presentation, a 24-hour urine collection demonstrated calcium excretion of 1.8 mmol/day, with a calcium:creatinine clearance ratio (CCCR) of 0.005. On questioning previous pathology providers, multiple corrected serum calcium levels between 2011 and 2016 had been normal. No relatives were available for screening for hypercalcaemia. Bone densitometry was assessed following her discharge from hospital and was found to be normal.


Our case was a one-day-old term male neonate born to a 29-year-old Indian female. There was no significant antenatal history. The baby was born of an uneventful normal vaginal delivery and weighed 2.5 kg. He had eversion of both upper lids with chemosis, which progressively increased with crying (Figure 1 ). Upper palpebral conjunctival prolapse with copious discharge was evident in both eyes. The attempts to manually invert the lids in position were not successful. Systemic examination of the baby was normal. On retracting the eyelids, the eyeballs appeared structurally normal. Posterior segment was also normal. Treatment with topical 5% hypertonic saline hourly as well as cotton gauzes soaked in 5% hypertonic saline were placed over the eyes constantly. Topical antibiotic (tobramycin 0.3%) was instilled three times a day along with lubricants every hour. The effect of the treatment was evident within 2–3 days with a reduction in chemosis and prolapse of the conjunctiva. Until the 4th day of treatment, the eyelids were in a normal position and reverted again if the baby cried. Following five days of treatment, the eyelids and conjunctiva were almost normal with spontaneous eye-opening. There was no recurrence thereafter even if the baby cried. The baby was discharged on the 7th day and was normal at subsequent follow-ups.


A 53-year-old man, American Society of Anesthesiologists (ASA) physical status I, with malignant peritoneal mesothelioma, underwent extensive resection, involving total peritonectomy, bilateral diaphragmatic stripping, mesenteric, and meso-colic omentectomy and cholecystectomy. The Peritoneal Carcinomatosis Index (PCI) was 26 and a completeness of clearance (CC) score 0 was achieved, which was followed by HIPEC with Mitomycin 20 mg and Cisplatin 75 mg at 42 °C for 60 min. The HIPEC phase was delayed due to hemodynamic instability necessitating fluid resuscitation and noradrenaline infusion. Noradrenaline requirement went up from 0.1 to 0.3 μg/kg/min by the end of surgery.
On admission to the surgical intensive care unit (ICU), patient was hypothermic (34 °C) with cold clammy extremities, heart rate (HR) 157/min, MAP 75 mm Hg and noradrenaline infusion ongoing at 0.3 mcg/kg/min. Serum potassium in arterial blood was 2.99 mmoL/L. Fluid boluses of 1.5 L crystalloid and 1 L albumin 4% were given. A bolus dose of 1 mEq/kg of sodium bicarbonate was given in view of severe worsening high anion gap metabolic acidosis (pH−7.16) on the arterial blood gas (ABG), followed by an infusion at the rate of 0.5 mEq/kg/h. After 4 h, blood pressure began to drop and 1 L crystalloid bolus was given. The patient then went into cardiac arrest, cardio-pulmonary resuscitation (CPR) was started and patient was successfully resuscitated with return of spontaneous circulation (ROSC) after 150 s. Echocardiography showed a collapsing inferior vena cava, for which 3 L of crystalloid and 1 L albumin 4% was rushed. Drain output was high in the immediate post-operative period and was serous in nature, along with deranged coagulation parameters and required aggressive fluid resuscitation including crystalloids, albumin, packed red blood cells and fresh frozen plasma. Noradrenaline support was slowly tapered and tracheal extubation was done over non-invasive ventilation (NIV) on second post-operative day (POD). He required intermittent NIV and oxygenation by high flow nasal cannula (HFNC) for 3 days and was shifted to the ward on POD 5.


A 36-year-old woman, case of pseudomyxoma peritonei, ASA physical status II, hypothyroid, who underwent total peritonectomy, near total gastrectomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, splenectomy, appendicular stump revision, loop sigmoid colostomy, and an accidental inferior vena cava rent which necessitated inferior vena cava repair, with bilateral intercostal drain placement followed by HIPEC with Doxorubicin 20 mg and Mitomycin-C 20 mg for 45 min. The PCI was 26 and a CC-0 was achieved. Noradrenaline infusion was started during CRS, requirement increasing up to 0.6 µg/kg/min during IVC repair, which was gradually tapered. Coagulopathy correction was started intra-operatively due to massive blood loss (9.4 L), abnormal coagulation parameters and oozing in the surgical field. Fluid management was goal directed as in case 1. The HIPEC phase was shortened to 45 min because of hemodynamic instability.
On shifting to the surgical ICU, patient had a HR of 93/min, MAP: 81 mm Hg, with cold clammy extremities, oropharyngeal temperature of 34.4 °C and noradrenaline infusion ongoing at 0.16 mcg/kg/min. Serum potassium in arterial blood was 2.61 mmoL/L. Subsequently, she developed tachycardia (HR 160/min), hypotension and required fluid boluses (500 mL crystalloid, 250 mL 4% albumin). After 45 min of shifting, patient had cardiac arrest, CPR was given and ROSC was attained after 20 min. Fluid resuscitation and coagulopathy correction was continued in the post-operative period including crystalloids, albumin, blood, and fresh frozen plasma. She was weaned from mechanical ventilation gradually and trachea was extubated on POD 6, required HFNC for 4 days and was shifted to ward on POD 11.


A 6-year-old male, undervaccinated and with a history of exposure to COVID-19, presented with acute onset high-grade fever, nonbilious nonbloody vomiting, periumbilical diffuse abdominal pain, and watery diarrhea. Patient symptoms worsened over the prior 7 days. His caregiver was diagnosed with symptomatic COVID-19 infection by PCR about 6–8 weeks before presentation. However, the patient had no symptoms and was not tested at that time. He had no history of gastrointestinal disease or weight loss prior to this illness.
On presentation, he complained of shortness of breath and fatigue. Physical examination revealed fever (104°F) and toxic appearance. He had tachycardia with a short self-resolved episode of heart rate over 200 beats/min and had muffled heart sounds. He showed tachypnea, intercoastal retraction, and diffuse abdominal tenderness without peritoneal signs. Computerized tomography (CT) scan of the abdomen and pelvis with contrast showed extensive phlegmonous changes in the right lower quadrant around the ileocecal junction and terminal ileal loops. The terminal ileal loops, cecum, and ascending colon had wall thickening and abnormal enhancement. The ileocecal valve was edematous and was protruding into the cecum. Multiple enlarged (up to 2 cm) reactive lymph nodes were seen at the base of the mesentery and right lower quadrant. The CT scan also showed retrocolonic positioning of the appendix with borderline wall thickness and minimal fat stranding around the appendix (Fig. A, B).
Blood work revealed evidence of inflammation including elevated C-reactive protein, erythrocyte sedimentation rate, fibrinogen, procalcitonin, d-dimer, ferritin, and neutrophils, and reduced lymphocytes and albumin. Transthoracic echocardiogram showed mildly dilated left and right atrium, trivial mitral and tricuspid valve insufficiency, and moderately diminished left and right ventricular systolic function. The estimated left ventricular ejection fraction by volume was 33% with normal diameter coronaries. Chest X-ray was positive for pulmonary edema and signs of fluid overload. Infectious evaluation was positive for COVID-19 immunoglobulin G with negative nasopharyngeal PCR (Table ).
Our patient was treated with intravenous ceftriaxone and metronidazole, immunoglobulin (IVIG) (2 g/kg/dose) for 2 doses within 24 hours, and methylprednisolone (1 mg/kg/dose twice daily for 3 days). He required inotropic support with milrinone. After initiation of IVIG and steroids, the patient showed improvement in fever and other systemic symptoms, including abdominal pain and diarrhea. Repeat CT scan of the abdomen and pelvis with contrast after 7 days of therapy showed complete disappearance of the previously found ileocecal phlegmon (Fig. C).


A 38-year-old female with an extensive medical and surgical history presented to the orthopedic oncologist with a complaint of increasing right arm pain and an existing and worsening deformity after a fall sustained one year prior. Imaging studies revealed a malunited humeral shaft and an associated destructive lytic lesion (8.1 cm in length) of the proximal right humeral shaft with cortical destruction and extraosseous extension with close proximity to the brachial vessels. The patient had a prior history of hypertensive emergencies and was therefore on dual antihypertensive therapy (metoprolol and prazosin).
Her extensive surgical history dated back twenty years when she underwent a partial bladder wall and right adrenal resection for malignant paraganglioma confirmed by histopathology. Six years later, she fell and was found to have multiple lesions in her right tibia, left 7th rib, lower cervical spine, and right humerus on CT scan. A bone biopsy revealed metastatic paraganglioma. She was treated with systemic chemotherapy and underwent in remission for 10 years. Then, she sustained an injury affecting her back and her right shoulder. An MRI imaging raised concerns of tumor recurrence within her humerus with no recurrence in the adrenal gland. Laboratory testing revealed increased free normetanephrines (5410; ref </ = 148) and chromogranin A (782; ref 25–140 ng/ml) but normal free metanephrines and calcitonin.
Due to large tumor size (7.4 × 4.1 × 3.4 cm) and patient's right arm pain, a wide resection and reconstruction with a cemented intercalary endoprosthesis was planned. A multidisciplinary approach for perioperative management of the patient was initiated. The patient was admitted four days prior to surgery for medical optimization. On admission, patient's blood pressure was 152/82 mmHg, HR 73 bpm, and respiratory rate was 17. Blood pressure during the four days ranged (113–220)/(68–110) mmHg. Presurgery hospital medications included PO phenoxybenzamine 30 mg BID prior to surgery, metoprolol 75 mg BID, IV fluids 200 ml/hr, oxycodone 5 mg/10 mg, and lorazepam 2 mg PO once as needed. Twenty-four hours prior to surgery the patient's blood pressure and heart rate ranges were (113–148)/(68–94) mmHg and (65–98) bpm, respectively. Echocardiogram showed normal ejection fraction and no evidence of pulmonary hypertension. EKG was normal with a HR of 62 bpm.
On the morning of surgery, the patient was given midazolam 2 mg IV in the preoperative holding area. In the operating room, an arterial line was placed. After administering 100 mcg of IV fentanyl, an ultrasound-guided right interscalene brachial plexus nerve block was performed. A catheter was concomitantly inserted in-plane using a high frequency linear transducer with a 13-6 MHz bandwidth (). A total of 12 ml of 0.5% ropivacaine was given for intraoperative pain, with increase in arm temperature and dilation of some hand veins noted prior to intubation. For the surgical procedure, general anesthesia with endotracheal intubation was induced with IV lidocaine (60 mg), etomidate (10 mg), propofol (50 mg), fentanyl (100 mcg), and rocuronium (40 mg). After induction, a central venous triple lumen catheter was inserted.
During the surgery, there were expected fluctuations in the blood pressure, with a range of (75–179)/(40–90) mmHg. The highest blood pressure was during surgical manipulation of the tumor, and the lowest blood pressure was after extraction of the mass from the surgical bed. Periods of hypotension were managed with titration of vasopressin 2-3units/hr and norepinephrine 5–10 mcg/min as well as the addition of fluid boluses. At the end of the procedure, the patient's blood pressure stabilized without requiring vasoactive support. She was extubated and transported to PACU with a BP of 110/52 mmHg. Surgical time was 4.5 hours. Total intake was 2.5 L Plasmalyte, 1L 5% albumin, and 1U PRBC (300 ml), and output was estimated blood loss of 1100 ml and urine output of 1300 ml.
Postoperatively, patient's blood pressure range was (110–118)/(52–58) mmHg and her HR was (86–91) bpm. The interscalene catheter infusion was started with 0.2% ropivacaine at 5 ml/hr for postoperative pain management. Pain scores and total opioid consumption during hospitalization () showed a maximum pain score of 4 on a VAS scale of (0–10) in the first 24 hours after surgery. The interscalene catheter was removed on POD#2, and the patient was placed on oral opioids (oxycodone 5 mg/10 mg q4hrs prn), acetaminophen 975 mg q8hrs, and lidocaine 5% patch. Following the procedure, the patient did not require alpha blocker agents and was discharged home on POD#4 with oxycodone 5 mg q4hrs prn and lorazepam 2 mg orally daily as needed for anxiety.
Two weeks after surgery, she was seen in follow-up by both the orthopedic surgeon and the endocrinologist. The patient exhibited full range of motion of her right elbow and fingers and was neurovascularly intact (). Repeat laboratory tests revealed a decrease in the total catecholamine level to 616pg/ml, a decrease in free normetanephrine to 188pg/ml, a normal metanephrines of 51pg/ml, and a normal chromogranin A of 128 ng/ml.


We present the case of an 11-year-old female with GPA diagnosed one year prior to presentation, stage III chronic kidney disease (CKD), hypertension (HTN), obstructive sleep apnea (OSA), and chronic anemia who presented with headache, nausea, vomiting, and diplopia and was found to have IIH.
The patient initially presented at the age of 10 with intermittent abdominal pain, nausea, and vomiting for about one year. She had a previous history of sleep apnea and nose bleeding. Exam showed paleness, HTN, ankles arthritis, rashes, and saddle nose. Work up showed anemia with hemoglobin 8.8 g/dL (normal 12-16 g/dL), elevated erythrocyte sedimentation rate (ESR) 127 mm/hr (normal 0-20 mm/hr), elevated C-reactive protein (CRP) 103 mg/L (normal <10 mg/L), elevated potassium, elevated creatinine 3.26 mg/dL (normal 0.6-1.2 mg/dL), estimated glomerular filtration rate (eGFR) 18 mL/min/1.73 m^2, elevated blood urea nitrogen (BUN) 45 mg/dL (normal 7-20 mg/dL), urinalysis with +3 hemoglobin and +3 protein, protein to creatinine ratio 2.88 (normal <0.2), positive antinuclear antibody (ANA) 1/160, positive antineutrophil cytoplasmic antibodies (ANCA) 1/640, negative myeloperoxidase (MPO), and positive PR-3 at 188 U/mL. Kidney biopsy showed pauci-immune glomerulonephritis (Figure ). Chest CT was prominent for minimal patchy ground glass opacity and two equivocal nodular densities in the lower lobes. Sinus CT scan was prominent for nasal septum perforation. Mild mucosal thickening was noticeable in the left maxillary antrum. She had a normal echocardiogram. She was diagnosed with GPA based on the American College of Rheumatology criteria and treated successfully with IV methylprednisolone, oral steroid taper over one year, seven-month induction treatment regimen with cyclophosphamide, and started later on rituximab. She was also on enalapril for HTN and iron for anemia. She became symptom free and blood work showed normalizing of her ESR, CRP, BUN, and PR-3. ANCA became negative. She continued to have elevated urine protein/creatinine ratio 0.6 and serum creatinine around 0.9 mg/dL with eGFR 50-60 mL/min/1.73 m^2 which places her in stage III CKD.
A year later since the diagnosis, she presented with vomiting and headaches. Completed work up did not show any GPA flare up. Brain MRI with and without contrast was unremarkable. A couple of days later, she developed double vision and left cranial nerve VI palsy. Exam showed bilateral papilledema. MRI orbit revealed tortuosity of the optic nerves with optic nerves bulging, apparent transverse sinus stenosis, and empty sella (Figures -). Lumbar puncture was prominent for elevated opening pressure at 36 cm H2O (normal 7-18 cmH2O). CSF analysis was not consistent with any infectious etiology with normal nucleated cells, glucose, and protein. Magnetic resonance venography (MRV) showed focal stenosis of the lateral right transverse sinus and diffuse narrowing of the left transverse sinus. Subsequently, the patient was diagnosed with IIH and was started on acetazolamide 5 mg/kg twice daily which eventually was titrated to 10 mg/kg for persistence of papilledema and headaches. The patient was maintained on acetazolamide throughout the admission with gradual improvement of symptoms. Upon discharge, the patient was back at baseline without double vision or vomiting and improvement of headaches.


A 70-year-old man with laryngeal cancer underwent contrast-enhanced computed tomography (CT) to identify metastasis, and a pseudoaneurysm with a diameter of 40 mm was found incidentally at the anterior superior pancreaticoduodenal artery (ASPDA) (). Liver transaminase and pancreatic enzyme levels in his blood laboratory testing were normal. Because CT showed no celiac axis stenosis and occlusion (), and, therefore, the ASPDA was not a collateral artery feeding any visceral organs, we selected endovascular coil embolization of the ASPDA to treat the aneurysm. The following is the detailed sequence of the procedure: First, under local anesthesia, superior mesenteric artery (SMA) arteriography was performed with a 4.2-Fr catheter (Excellent EN; Hanako Medical Co., Ltd., Saitama, Japan) inserted from the right femoral artery to confirm the location of the aneurysm and the surrounding vascular anatomy. Next, a 2.0-Fr microcatheter with two radio-opaque markers (Excelsior™ 1018; Stryker Corporation, Kalamazoo, MI, USA) was advanced with a guide wire into the ASPDA, passing over the aneurysm and placed with its tip located distal to the aneurysm. Then, coil embolization was performed using six coils with a diameter of 6 mm (Tornado; Cook Medical, Bloomington, IN, USA). Next, we retracted the microcatheter to a point proximal to the aneurysm and continued the coil embolization using five coils with diameters of 5–6 mm (Tornado; Cook Medical). Finally, successful exclusion of the aneurysm was confirmed by completion angiography (). The patient was uneventfully discharged the next day, and no recurrence of the aneurysm was seen on follow-up CT scans.


A 63-year-old woman with chronic rheumatoid arthritis and dyslipidemia underwent abdominal ultrasonography for a medical check-up, which incidentally revealed a mass shadow near the pancreas. Subsequent enhanced CT detected a fusiform-type aneurysm with a diameter of 19 mm at the ASPDA. The celiac axis appeared to be occluded, likely owing to compression by the median arcuate ligament, and distal circulation of the celiac artery appeared to be provided by the SMA through the ASPDA because tortuous and dilated transformations in the ASPDA were also confirmed (). Even in this case, we considered endovascular treatment possible because detailed evaluation of the CT images revealed two other collateral pathways connecting the SMA and branches of the celiac artery, which referred to the posterior superior pancreaticoduodenal artery (PSPDA) and the dorsal pancreatic artery (). In other words, these collaterals could provide blood flow to the celiac artery after embolizing the aneurysm and the ASPDA. The following is the detailed sequence of the procedure: First, SMA arteriography was performed with a 4-Fr catheter (CNS1; Terumo Clinical Supply, Gifu, Japan) inserted from the right femoral artery to identify the location of the aneurysm and the surrounding vascular anatomy (). Next, preceded by a guide wire, we advanced a 2.0-Fr microcatheter with two radio-opaque markers (Excelsior™ 1018; Stryker Corp.) into the ASPDA through the 4-Fr catheter, passing through the aneurysm and placed with its tip distal to the aneurysm. Then, coil embolization was performed using the following coils: one detachable coil with a diameter of 8 mm and length of 20 cm (Target XL™; Stryker Corp.) and four coils with diameters of 8–10 mm (Tornado; Cook Medical). Then, we retracted the microcatheter back to the aneurysm and performed coil embolization of the aneurysm itself using three detachable coils with diameters of 18–24 mm and lengths of 30–40 cm (Target XL™; Stryker Corp.). Next, we retracted the microcatheter further back, to the ASPDA proximal to the aneurysm, and continued the coil embolization using three coils with diameters of 5–6 mm (Tornado; Cook Medical), being careful to not occlude the PSPDA, which originated from proximal to the aneurysm. The final angiography showed that the contrast agent pooled slightly within the aneurysm (). However, we did not add more coils because of the risk of occluding the PSPDA. Subsequently, post-treatment enhanced CT showed occlusion of the aneurysm and the ASPDA and patency of the PSPDA, the dorsal pancreatic artery, and the celiac artery branches. The patient was discharged the next day without signs associated with visceral organ ischemia. No recurrence of the aneurysm was observed in follow-up CT scans.


This report involves a 22-year-old male patient reporting to the Department of Endodontics, Qazvin University of Medical Sciences, with the chief complaint of fractured teeth caused by trauma with a wrench, 3 days before. The medical history was normal and classified as ASA I. In clinical evaluations, extra oral examination showed no bleeding, fracture of facial bones, or draining sinuses. Intra oral examination showed no swelling and sinus tract; teeth #8, #23, and #24 were traumatized with pulp exposure (Figure ). According to the pulp sensibility test, teeth #23 and #24 showed a severe response but tooth #8 exhibited a mild response. None of the teeth above were tender to percussion/palpation. Interestingly, tooth #9 did not show any response to cold/heat test, exhibited tenderness to periapical tests and showed grade I mobility. The crown fractures of traumatized teeth were revealed on the panoramic radiograph. Intra oral periapical radiograph demonstrated a horizontal root fracture in the apical third of tooth #9; crown and oblique crown fracture line extending subgingivally in tooth #8 (Figure ). The radiographic examination also revealed crown fractures of teeth #23 and #24 and no damage to the adjacent teeth. Based on the clinical and radiographic findings, the patient was diagnosed with complex traumatic dental injuries involving crown-root fracture and root fracture in teeth #8 and #9, respectively. Teeth #23 and #24 were also diagnosed with complicated crown fracture.
All the traumatized teeth were subjected to emergency treatment on the first visit. After obtaining informed consent, to evaluate the fractures and determine the restorability, the lingual mobile segment of teeth #8 was removed (this was a part of the diagnosis). To immobilize tooth #9, semi-rigid splinting (33) was performed using 0.5 mm diameter round stainless steel wire and composite resin (Figure ). Also, emergency pulpectomy was initiated for teeth #8, #23, and #24. After local anesthesia (2% lidocaine with 1:80 000 epinephrine; Daroupakhsh) and rubber dam isolation, a fissure diamond bur in a high-speed handpiece with water spray was used to prepare an access cavity. The working length was determined by Root ZX electronic apex locator (J Morita MFQ) and confirmed with x-ray (Figure ). Cleaning and shaping of the root canal system were completed using the crown-down technique followed by irrigation with normal saline solution and 5.25% sodium hypochlorite. The root canals were dried with sterile paper points. A creamy mix of calcium hydroxide as the intracanal dressing was placed with a lentulo spiral. Finally, the access cavity was sealed temporarily with reinforced Zinc Oxide-Eugenol. The patient was recalled 1 month later. In the next visit, after splint removal, obturation was performed with gutta-percha and AH26 sealer by the lateral compaction technique (Figure ). In the 8-week follow-up visit, the existing gutta-percha was removed to within 5 mm of the root apex with a selection of Gates-Glidden drills in teeth #8, #23, and #24. A temporary hook was cemented inside the canals using zinc phosphate cement to aid in extrusion (Figure ). A customized orthodontic appliance was made for the patient and orthodontic elastic (size 1/8 for teeth# 23, and #24, and size 3/16 for tooth #8) was applied from the hook to the specially designed loop for rapid extrusion (Figure ). One month later, circumferential supracrestal fiberotomy was performed to avoid any relapse. After the active period of extrusion, the teeth were stabilized for 8 weeks, using a splint. Following the retentive phase, the retainer was removed and the prosthodontic treatment planning and a primary impression were undertaken (Figure ). A custom-made post and core was prepared using a direct technique followed by permanent cementation. In the 6-month follow-up, the teeth were asymptomatic with no sign of root resorption or lesion (Figure ). In the 1-year follow-up, tooth #9 was responsive to pulp sensibility tests. The previously treated teeth did not show any pain, sign, symptoms, and tenderness to percussion. Interestingly, the healing pattern of the horizontal root fracture was demonstrated to be with calcified tissue.


A 42 years old male presented to the emergency room with the alleged history of suicidal ingestion of an unknown poison 2 hours back following a family dispute. The patient immediately had two episodes of nonbilious nonprojectile vomiting followed by excessive lacrimation, bowel and bladder incontinence, and dizziness within 2 hours of ingestion of the poison. There was no history of shortness of breath, chest pain, loss of consciousness, or seizures. He did not have any past medical or psychiatric history or similar suicidal attempts in the past. On examination in the emergency room, the patient was drowsy but followed verbal commands. He had a radial pulse of 50 beats per minute, blood pressure of 90/50 mm Hg, respiratory rate of 16 breaths/minute, temperature of 98.6 degrees Fahrenheit, and oxygen saturation of 94% in room air. His pupils were three millimeters in size bilaterally and reacting to light. Furthermore, the peculiar garlicky smell of organophosphate was not detected from his breath. His chest auscultation was clear, abdomen was soft and nontender, and the remaining systemic examination did not reveal any abnormality.
With the provisional diagnosis of a possible organophosphorus poisoning, the patient was managed initially by administering an intravenous dose of atropine 0.6 mg following which his heart rate increased to 100 beats per minute showing a normal sinus rhythm in the 12-lead electrocardiogram. Dermal decontamination was done by removing the patient's vomitus soaked clothing. The skin was cleaned thoroughly with soap water, and gastric lavage was performed simultaneously. A bolus dose of 500 mL of 0.9% normal saline and an intravenous dose of pralidoxime 2 grams over 30 minutes were administered. His investigations comprising a full blood count, arterial blood gas, renal function tests, a random blood sugar level, liver function tests, a coagulation profile, and a serum cholinesterase level were all within normal limits. A chest radiograph and an ultrasonogram of the abdomen and pelvis did not reveal any abnormalities.
A bottle of the herbicide pretilachlor 50% emulsifiable concentrate (EC) was retrieved from his room and was brought to the emergency room by his son after 2 hours. The patient confirmed to have consumed the full bottle containing 250 mL of the herbicide. Close monitoring with continuous supportive management was done for 24 hours with intravenous fluids and antiemetics following which he gradually became asymptomatic. His heart rate remained within the normal range, his blood pressure normalized, and a normal urine output was maintained throughout the hospital stay. Symptoms of lacrimation and incontinence subsided. Repeat blood counts, renal, and liver functions were within normal ranges. He was kept under observation with strict hemodynamic monitoring for the next 2 days which was uneventful. He was discharged from the hospital on the third day after a behavioral counseling session. He was in good health during a 1-week follow-up in the outpatient department.


A 23-year-old man presented to the emergency department with sudden onset post-tussive pleuritic chest pain and associated left-sided neck pain. He complained of dyspnea in addition to rhinolalia—a symptom of the voice gaining a higher pitch. He had three days of coryzal symptoms prior. He had no past medical history or contributing family history, took no regular medications, and was not known to have any allergies. He did not smoke or use illicit substances for recreational use, drank minimal alcohol, and was physically active. On examination, the gentleman was tall with a medium build. On admission, he was in mild discomfort with a tachycardia but was hemodynamically stable and had no hypoxia or respiratory distress. There was no JVP elevation or muffling of heart sounds to suggest tamponade. He had surgical emphysema palpable in the left supraclavicular fossa, but had a nondeviated trachea and lung fields clear to auscultation with normal resonance.


A 22-year-old man presented to our hospital with left-sided nasal obstruction for more than 2 years. The patient's medical history, including the previous history of allergy or surgery, was nonspecific. In physical examination, left-sided septal bulging, which was soft and compressible, was observed (Figure ). A more careful inspection of the upper part of the septum revealed a distinct small round mass (Figure ). CT scan without contrast revealed a hypodense mass with 1.5 cm thickness (Figure ). Further evaluation with magnetic resonance imaging (MRI) to inspect any possible intracranial connection showed an amorphous heterogenous 54*8*94 mm mass located in the left anterior part of the nasal septum. The mass was a hyper signal in T2-weighted images and did not enhance after gadolinium injection, contrary to its overlying normal nasal mucosa (Figures and ). An incisional biopsy was performed on the mass, and the homogenous yellow specimen was reported lipoma. On histopathological examination, the left nasal cavity lesion excision showed a piece of creamy, firm tissue. Under general anesthesia, the mucoperichondrial flap was elevated using left hemitransfixion incision, and the tumor was excised completely. Histopathology report confirmed lipoma. The patient presented no complications during or after surgery. The patient was followed up for 6 months. His nasal obstruction was improved significantly, and the apparent nasal deviation was alleviated to some degree. No recurrence of the tumor was detected in the follow-up period.


A 36-year-old man with history of asthma, hypertension, uncontrolled diabetes, and obesity presented with sudden onset of redness and swelling in the right eye that started 6 days prior to hospitalization and was gradually getting worse. He initially went to an urgent care where he was prescribed cephalexin 500 mg twice a day. After 2 days, he did not notice significant relief and developed a new blurry vision associated with photophobia in the right eye. Later, he spiked a fever of 103°F at which point he decided to come to the hospital and got admitted. He denied reports of trauma, local injury, swimming pool exposure, and use of contact lens. He denied any recent sick contacts and lives with his spouse and three young kids, none of whom were sick in recent times. He reported a monogamous relationship with his wife and denied any history of sexually transmitted diseases. He denied intravenous drug use, smoking, or alcohol use. He had two pet dogs, none of whom were sick with similar illness. His vital signs were stable at presentation except for fever as mentioned above. On examination, he had severe redness of palpebral conjunctiva with diffuse subconjunctival hemorrhage. Upper and lower right eyelid showed redness, ecchymoses, swelling, crusting, and discharge from the corner of his right eyelid (Figure ).
His left eye was normal. Pupils were equally reactive to light on both sides. Intraocular pressure measured by tono-pen was within normal limits. Visual field testing showed mild generalized constriction of visual field of the right eye. Bilateral corneas, anterior chamber, iris, and lenses were grossly normal without any abnormality. Fundoscopic examination via indirect ophthalmoscopy after pupillary dilatation showed normal vitreous and absence of retinal or optic disc changes. All cranial nerves were intact, and extraocular muscles showed full range of motion with tenderness on full extension. Motor, sensory examination, and reflexes were equal and bilaterally symmetrical.
Upon admission, he was started on broad-spectrum antibiotics. White blood cell count, hemoglobin, platelets, serum electrolytes, serum creatinine, and liver function tests were all within normal limits except for an elevated blood sugar of 268 mg/dL (normal range [NR]: 70-110 mg/dL). Procalcitonin was normal at 0.04 ng/mL (NR: <.1 ng/mL), erythrocyte sedimentation rate was elevated at 54 mm/hour (NR: 1-13 mm/hour), and C-reactive protein was 0.94 mg/dL (NR: <1 mg/dL). HIV 1&2 antigen and antibody test were negative. Magnetic resonance imaging (MRI) of face showed preseptal soft tissue thickening consistent with cellulitis. Ocular globe, postseptal space, ocular lenses, extraocular muscles, optic nerve sheath, lacrimal glands, and retro-bulbar fat appeared normal with no involvement from infection (Figure ).
Blood cultures returned negative after 5 days of incubation. Serum sample was sent for microbial cell-free DNA test also known as meta-genomic testing. It showed 459 DNA molecules per microliter (MPM) of human adenovirus D in the serum sample with normal range of <10 MPM. The patient was started on topical steroids, and he responded well with significant improvement in signs and symptoms within first 3 days. Antibiotics were discontinued, and the patient was discharged home with no sequelae at the follow-up visit.


A 71-year-old-woman admitted with acute low back pain of 8 weeks that became severe 3 days before admission. Pain is limited to left lower costal margin with no midline or paravertebral pain. There was tenderness at thoracolumbar junction and over lower ribs and left paraspinal muscles. Reflexes and motor power were normal in four limbs. No cranial nerves affection.
MRI (magnetic resonance image) spine showed D8-9 (Dorsal) spondylodiscitis with soft tissue collection. Patient underwent surgical debridement and fixation form D6-D7 to D10-D11 with fusion D8-9, through direct dorsal approach. Fixation of transpedicular screws diameter 6.5 length 35mm in dorsal and 60mm diameter, length 40 mm in lumbar, Medtronic. decompression then debridement of D8-9-disk level bilaterally.
On day 3 postoperatively, patient had progressive dyspnea and CXR (Chest x-ray) showed complete opacification of the left lung (Figure ). Urgent chest ultrasound showed massive left pleural effusion with Plankton sign (Figure ). Chest tube, pigtail size 8 French, was inserted at intercostal space to drain cloudy milky fluid (Figure ). 2.5 liters of milky fluid was drained after 24 hours. Pleural fluid showed high triglyceride 800 mg/mL (10.10 mmol/L); total protein was 36.8 g/L and total cholesterol 180 md/dl (4.8 mmol/L).
Chylothorax was confirmed. The patient was kept NPO (Nothing per mouth), TPN (Total parenteral nutrition) started, and somatostatin infusion. Left pleural effusion was decreased in subsequent days.
Follow-up CT (computerized tomography) of the chest showed—Multiple spinal fixation screws in the D6 TO D10 vertebral bodies. Left D6 screw was seen extending via the pedicle into the mediastinum, just medial to the descending aorta and touching the esophagus. Left D8 screw is not visualized. Both D9 and D10 screw on the left side is seen penetrating the anterior vertebral cortex extending into the prevertebral soft tissue. Multiple bony fragments were noted in the left half D8 vertebral body.
The chest tube was removed after 5 days with drainage less than 50 mL/d for 2 days. A follow-up chest ultrasound showed minimal effusion. The patient was discharged home in a stable condition.


A 55 year-old man with a history of myocardial infarction and ischemic cardiomyopathy status-post ICD placement 8 years prior presented to an outside hospital with bacteremia. His original ICD placement was in 2005, with a Guidant FineLine atrial lead and a Guidant dual coil ICD lead. In 2008, an additional Guidant atrial lead was placed. He was transferred to our tertiary care center after 8 weeks of IV antibiotics and evidence of a large 5cm x 2cm, mobile tricuspid valve vegetation associated with the ICD lead (Figure ) seen on trans-Esophageal Echocardiogram (TEE). Blood cultures were positive for Staphylococcus hominis.
The patient was taken to the hybrid OR, placed under general anesthesia, and underwent pulse generator and lead extraction, along with AngioVac removal of RV and RA vegetations (Figure ). Significant purulent fluid was found in the device pocket and tracking along the leads. The RA FineLine lead was extracted with a 14F laser sheath. However, adhesions made extraction difficult and the lead tip was fractured and was subsequently removed using a femoral approach with a snare. The AngioVac system was then used to remove the large vegetation via right internal jugular and right femoral vein cannulation. The patient was heparinized to an ACT >250. The AngioVac cannula was advanced into the RA, and 1.5-2L/min flow was achieved. The 14F laser sheath was used to extract the second atrial lead and a 16F laser sheath and a mechanical rotational tool was then required to extract the ICD lead.
Post-procedure the patient remained febrile and with leukocytosis to 23,000. Post-procedure TEE showed persistent valve vegetation and a chest CT scan showed evidence of sub-massive pulmonary emboli. The patient had worsening fevers, chills, pleuritic pain, and dyspnea despite antibiotics. Right heart catheterization revealed normal right heart pressures and borderline pulmonary hypertension with a mean pulmonary artery pressure of 27 mmHg. Pulmonary angiography showed proximal filling defects of bilateral lobar and segmental branches. Percutaneous embolectomy was attempted but was unsuccessful as a wire was unable to be passed beyond the thrombus.
The patient was therefore considered for pulmonary endarterectomy surgery. He underwent bilateral pulmonary endarterectomy under cardiopulmonary bypass and deep hypothermic circulatory arrest (287 minutes of bypass time, 23 minutes of arrest time on right side, 20+18 minutes of arrest on left) (Figure ). The tricuspid valve and right ventricle vegetations were also removed and the septal leaflet of the tricuspid valve repaired with a Gortex neochord. His post-operative course was uneventful with resolution of symptoms and improved hemodynamics with a Pulmonary Vascular Resistance of 146, down from 316 dyne*sec/cm5. He was discharged on antibiotics and 6 months of anticoagulation. He has done well in follow-up and a new ICD system was later successfully placed at his referring institution.


The first patient was an 86-year-old woman with a history of hypertensive cardiomyopathy with severe mitral regurgitation and atrial fibrillation on anticoagulant therapy with warfarin. She presented to the Emergency Department after experiencing a sudden onset of shortness of breath on mild exertion and peripheral edema.
At admission, the patient was afebrile, eupneic in oxygen therapy at rest. Thoracic physical examination revealed decreased breath sounds and crackles in the lower lung lobes, bilaterally.
Blood investigations revealed a total leucocyte count of 9140/mcL (normal 4000–11,000/mcL), Hb 11 g/dL (normal 13–18 g/dL), C-reactive protein (CRP) 15.8 mg/dL (normal < 0.50 mg/dL), Procalcitonin (PCT) 0.32 ng/L (normal < 0.5 ng/L), fibrinogen 707 mg/dL (normal 200–400 mg/dL), creatinine 1.23 mg/dL (normal 0.7–1.20 mg/dL), INR 2.36 (normal 0.82–1.19). Chest CT revealed bilateral pleural effusion with passive atelectasis of the lower lobes. Patient was diagnosed with COVID-19 on April the 8th, 2020, on the basis of RT-PCR testing that detected SARS-CoV-2. The patient was admitted to our Geriatric department and began to receive hydroxychloroquine, intravenous ceftriaxone and doxycycline, as per local protocol, observing a good clinical response and the decrease of inflammation markers. Weaning and discontinuation of oxygen therapy was possible once the patient was stable with satisfactory oxygen saturation. The anticoagulation (AC) therapy was maintained, however, we decided to switch AC therapy from warfarin to low molecular-weight heparin (LMWH) enoxaparin 6000 U BID.
Suddenly, 5 days after admission, she started complaining of pain and loss of movement of her left leg, which appeared swollen. Venous compression ultrasound (CUS) and evaluation of D-dimer excluded deep vein thrombosis, but a Chest computed tomography (CT) scan with contrast revealed an 11 × 8 × 14 cm hematoma in the left thigh (Fig. ). Laboratory tests showed a fall in hemoglobin from 11 g/dL to 7.8 g/dL and, despite the absence of active arterial extravasation, prophylactic embolization of small branches of superficial and deep femoral artery was performed. Blood tests at that time also showed alterations of coagulation system, with reduced prothrombin time, increased fibrinogen and international normalized ratio (INR), despite a platelet count within normal limits. Interleukin-6 levels were 67,9 pg/mL (normal < 12.7 pg/mL). Overall, the patient was transfused 3 unit of packed blood cells, which raised her hemoglobin to 10.1 g/dL. Table shows the patient’s routine blood test during the hospitalization. In the following days, the patient’s hemoglobin remained stable and the hematoma showed no evidence of further expansion. The patient recovered completely and was discharged shortly after the event.


A 29-year-old female in her second pregnancy, with a history of tetralogy of Fallot or ventricular septal defect with pulmonary atresia, had undergone 4 surgeries during her childhood, including a modified Blalock-Taussig shunt, patch repair of ventricular septal defect, and a right ventricle (RV) to pulmonary artery (PA) conduit. At 20 weeks’ gestation during her first pregnancy, she developed severe symptomatic stenosis of the RV to PA conduit with a Doppler gradient of 98 mmHg, thus requiring a 22-mm balloon dilatation and Genesis XD stent as a temporizing measure until definitive repair could be undertaken postpartum. However, she was lost to follow-up after delivery of her first child and did not undergo postpartum repair of her RV to PA conduit stenosis.
The patient presented to our clinic during her second pregnancy at 21 weeks of gestation. Thus far, her pregnancy had been uncomplicated and prenatal testing to date had found no abnormalities in fetal development. On presentation, she admitted to mild dyspnea on exertion at a distance of 20 yards. On examination, the patient was tachycardic and normotensive with normal oxygenation. She was euvolemic with hemoglobin of 13.5 g/dL and creatinine of 0.7 mg/dL. Echocardiogram showed normal left ventricular ejection fraction (LVEF) of 60% to 65%, with mild right atrial (RA) dilatation, evidence of RV hypertrophy, and an estimated RV pressure of 82 mmHg. The PA conduit was severely stenosed, with a mean gradient of 52 mmHg and unrestricted pulmonic valve insufficiency. Our assessment determined her modified World Health Organization (WHO) pregnancy risk classification to be II-III (moderately increased risk of maternal mortality and moderate morbidity).
She was seen again in clinic at 34 weeks’ gestation. Her symptoms and repeat echocardiogram were unchanged from the previous visit. However, at 36.5 weeks’ gestation, 2-dimensional echocardiogram done in preparation for delivery identified severe conduit stenosis with a mean gradient of >75 mmHg, peak gradient of 98 mmHg with a peak velocity of 5 m/s, and unrestricted pulmonic valve insufficiency. The patient's RV pressures at the time also exceeded 71 mmHg. Despite the severe restenosis and unrestricted insufficiency of her PA conduit, her symptoms remained New York Heart Association (NYHA) class II. The plan was to admit the patient prior to cesarean section for assessment of heart failure and bilateral tubal ligation at 37 weeks’ gestation.
As planned, she was admitted at 37 weeks for delivery and was cared for by a multidisciplinary team involving obstetrics, ACHD specialists, heart failure specialists, obstetric anesthesia, maternal fetal medicine, and the neonatal intensive care unit.
The patient underwent a successful low transverse cesarean section and bilateral tubal ligation under epidural block with an estimated blood loss of 1,100 mL. Postdelivery echocardiogram showed normal LVEF of 55% to 60%, RA and RV dilatation with RV hypertrophy, and no change in RV pressure or valve gradient. The RV to PA conduit stenosis had decreased to a peak velocity of <4.8 m/s and a mean gradient of <56 mmHg with unrestricted insufficiency.
The patient had an uncomplicated postoperative course, only requiring some gentle hydration, and was discharged 3 days postpartum. She was scheduled for follow-up for severe conduit stenosis at 6 weeks postpartum to discuss management options.


A 9-year-old, non-ambulatory intact male Shih Tzu dog presented to our clinic 1 day after the peracute onset of neurological signs. Upon physical examination, the dog weighed 6.5 kg, had a pulse rate of 86 per minute, a respiratory rate of 32 breaths per minute, and a rectal temperature of 38.4°C. On neurologic examination, a tetraplegia was identified with lower motor neuron (LMN) symptoms, including decreased to absent spinal reflexes, flaccid paralysis, and muscle atrophy, whereas his mental status was alert and all cranial nerve examinations were normal. Laboratory examinations did not show any remarkable abnormalities.
Initially, diseases that cause acute flaccid tetraparesis, such as acute fulminating myasthenia gravis, polyradiculoneuritis, tick paralysis, and botulism, were suspected. During hospitalization, electrophysiologic examinations and neostigmine challenging tests were performed to differentiate between neuromuscular diseases. Electromyography and repetitive motor nerve stimulation tests conducted to evaluate denervation of the muscle and peripheral motor system, especially on the quadricep femoris muscle, were both normal. In addition, when injected with neostigmine methylsulfate (0.04 mg/kg subcutaneously; Dai Han Pharm. Co., Ltd, Seoul, South Korea), which is an acetylcholinesterase inhibitor, no improvement was observed. Tick paralysis was ruled out because the dog had not visited an endemic area and an engorged tick was not found on his body. Furthermore, although we did not test for botulinal neurotoxin or botulinal neurotoxin-antibody titer, botulism was also excluded because of no exposure to spoiled food. However, polyradiculoneuritis could not be completely excluded because we could not rule out the idiopathic type.
MRI examination of the spinal cord was performed using a 0.3-Tesla unit (Airis II, Hitachi, Japan) 2 days after the initial clinical signs. General anesthesia was induced with intravenous administration of propofol (6 mg/kg; Provive, Myungmoon Pharm. Co., Ltd, Seoul, South Korea) and maintained by inhalation of 2.0 to 2.5% isoflurane (Terrell, Piramal Critical Care, Bethlehem, PA, USA) in a circle rebreathing circuit. T1-weighted, T2-weighted, and fluid-attenuated inversion recovery (FLAIR) images were obtained using transverse, sagittal, and dorsal planes. The main lesion was identified between the C3-C6 spinal cord parenchyma through hyperintensity in the T2-weighted and FLAIR images, as well as hypointensity to isointensity in the T1-weighted images (). Additionally, mild to moderate compressions of the spinal cord parenchyma between the L1 and L5 level were also identified. After administration of gadolinium-diethylenetriamine pentaacetic acid (0.1 mmol/kg, IV; Omniscan™, GE Healthcare (Shanghai) Co., Ltd, China), post-contrast T1-weighted images were taken, but they exhibited no enhancement. For cerebrospinal fluid analysis, the total nucleated cell count was 465 cells/μL (reference range <5 cells/μL) with neutrophilic pleocytosis (neutrophil 91%, lymphocyte 7%, and monocyte 2%) and protein concentration was 30 mg/dL (reference range <25 mg/dL). Polymerase chain reactions for infectious agents were all negative (Bartonella spp., Blastomyces dermatitidis, Coccidioides spp., Cryptococcus spp., Histoplasma capsulatum, Canine distemper virus, West Nile virus, Borrelia burgdorferi, Neospora spp., and Toxoplasma gondii). Based on the medical history, signalment, clinical assessments, and laboratory findings, although bacterial myelitis was not completely ruled out due to the absence of a CSF culture, the dog was tentatively diagnosed with non-infectious myelitis or FCE with concurrent lumbar intervertebral disc disease (IVDD).
Initially, the dog was treated with prednisolone (1 mg/kg twice daily; Solondo®, Yuhan, Seoul, South Korea) and pentoxifylline (15 mg/kg twice daily; HARIN sustained-release Tab., Celltrion, Inc., Incheon, South Korea) immediately after MRI examination. On the third day of hospitalization, the dose of prednisolone was increased to 2 mg/kg twice daily. However, because there was a loss of deep pain perception in the left forelimb on the 4th day of hospitalization, we decided to reexamine with MRI using a 3-Tesla unit to identify the presence of an acute ischemic spinal disease, such as FCE, using DWI and ADC maps on the 6th day of hospitalization. Using an echo planar sequence, we acquired sagittal sliced DWI sections with b values of 0 and 1,000 s/mm2 along all three axes, with the following parameters: repetition time, 4,164 ms; echo time, 107 ms; flip angle, 90°; number of slices, 10; slice thickness, 3 mm; slice gap, 0 mm; field of view, 150 × 150 mm; and acquisition matrix, 140 × 140. A high signal intensity was identified between the C4-C6 level corresponding to the lesion observed on the previous MRI on the sagittal DWI (). A region of interest was manually drawn on the hyperintensity area of DWI and copied to the ADC map at the corresponding level. The ADC map was computed based on a pixel-by-pixel basis from the DWI. It was calculated using OsiriX MD v10.0 (Pixmeo Sarl, Geneva, Switzerland) and showed a low value (0.54 × 10−3 mm2/s; ADC value of normal spinal cord, 0.93 × 10−3 mm2/s) (). Based on these results of the 3-T MRI, in addition to the poor response to the immunosuppressive treatment, we strongly suspected the dog had FCE. On the 7th day of hospitalization, the dose of prednisolone was decreased to 1 mg/kg twice daily. The loss of deep pain perception in all the limbs was identified, suggesting a poor prognosis, and the dog suddenly died due to respiratory difficulty, suspected to be caused by ascending myelomalacia which led to intercostal and diaphragmatic paralysis on the 9th day of hospitalization (10 days after the initial clinical signs). The owner donated the dog for necropsy.
Necropsy revealed necrotic regions in the area where the lesion was observed on MRI (). Furthermore, a necrotic region was also found at the C2 level that was not identified through MRI. A histopathological examination revealed the presence of a fibrocartilaginous embolus in a small artery at the C5 level (). There was also liquefaction necrosis of the gray matter with almost a total loss of the central gray column. Associated with this area of necrosis were accumulating numbers of Gitter cells with fewer neutrophils. In the surrounding white matter tracts, dorsally, laterally, and ventrally, there were variable numbers of degenerated and swollen axons. Similar lesions were present at both sites although marked at C5 and mild at C2. Based on these findings, the dog was definitively diagnosed with FCE presumed to have died due to ascending myelomalacia.


A 48-year-old female with past medical history (PMHx) of smoking presented for a left sphenopalatine ganglion implant for intractable chronic cluster headaches. Her home medications included melatonin and sumatriptan. Her pre-op vitals were stable. For pre-induction, she received midazolam (2 mg), followed by induction with remifentanil (150 mcg), lidocaine (50 mg), propofol (140 mg), and rocuronium (30 mg) for induction. Maintenance of anesthesia included a remifentanil infusion at 0.1–0.125 mcg/kg/min combined with desflurane. Soon after incision, the patient experienced an episode of bradycardia (45 bpm) and hypotension (88/51 mmHg). Surgical stimulation was stopped with recovery of both bradycardia and hypotension 2 minutes later. Throughout the case, the patient’s oxygen saturation and end tidal carbon dioxide was maintained appropriately (SpO2 > 97%, EtCO2 30–40 mmHg). Post operatively her vital signs were stable. She was given sumatriptan in the PACU for a cluster headache. This patient did not possess risk factors for TCR other than maxillary nerve proximity and manipulation. Of note, this patient returned for explantation of her sphenopalatine ganglion implant 3 years later due to paresthesias, malocclusion, and persistent cluster headaches. It was removed without intraoperative complication.


A 68-year-old male with PMHx of cluster headaches, hyperlipidemia, hypertension, right bundle branch block, and saddle embolus (2013) status post 6 months of Xarelto, presented for right sphenopalatine ganglion implant placement. His home medications included aspirin, metoprolol, telmisartan, and zolmitriptan. Pre-op vitals were stable. The patient was induced with remifentanil (50 mcg), lidocaine (50 mg), propofol (150 mg), and rocuronium (100 mg). His intubation was complicated by a grade 4 view upon initial direct laryngoscopy attempt, followed by a second successful attempt with a grade 2 view with the glidescope. Maintenance of anesthesia was accomplished with a remifentanil infusion at 0.1–0.125 mcg/kg/min along with desflurane. Intraoperatively, the patient experienced two episodes of bradycardia. The first episode of bradycardia (38 bpm) occurred during introduction of the introducer device into the pterygopalatine fossa. The episode resolved after the device was removed. There was reoccurrence 13 minutes later (34 bpm) during reinsertion of the device. Both episodes occurred before stimulation of the fixed device suggesting the cause for the TCR events was mechanical in origin. No pharmacologic intervention was used. Throughout the case, the patient’s oxygen saturation and end tidal carbon dioxide were maintained appropriately (SpO2 > 95%, EtCO2 30–36 mmHg). Post-operative vitals were stable. One risk factor for TCR was his home beta blocker, otherwise there was no evidence of light anesthesia, hypercapnia, and hypoxemia.


A 55-year-old female with PMHx of asthma, diabetes, hyperlipidemia, seasonal allergies, and smoking, presented for left sphenopalatine ganglion implant placement. Her home medications included atorvastatin, gabapentin, glyburide, montelukast, nortriptyline, rizatriptan, and topiramate. Pre-op vitals were stable. For induction, she received remifentanil (100 mcg), lidocaine (50 mg), propofol (130 mg), and rocuronium (30 mg) for induction with a remifentanil infusion at 0.1–0.15 mcg/kg/min combined with desflurane for maintenance. The patient experienced an episode of bradycardia (41 bmp) upon insertion of the introducer device. She was treated with glycopyrrolate (0.2 mg) with recovery and adjustment of the introducer device. The patient’s oxygen saturation and end tidal carbon dioxide were maintained appropriately (SpO2 > 98%, EtCO2 31–34 mmHg). Post-operative vitals were stable. The patient had no risk factors for TCR other than mechanical sources related to the surgery.


A 66-year-old female with PMHx of anxiety, cluster headaches, depression, diverticulitis, gallbladder disease, hepatitis, and reflux, presented for right sphenopalatine ganglion implant placement. Her home medications included aspirin, sumatriptan, topiramate, and verapamil. Pre-operative vital signs were stable. For pre-induction, she received midazolam (2 mg), followed by induction with remifentanil (100 mcg), lidocaine (50 mg), propofol (50 mg), and rocuronium (30 mg). Anesthesia was maintained with remifentanil boluses of 39.375 mcg every 5 min combined with desflurane. There were no intraoperative events concerning for TCR.


A 47-year-old female with PMHx of cluster headaches, hypothyroidism, and kidney stones, presented for left sphenopalatine ganglion implant placement. Her home medications included excedrine, levothyroxine, lithium carbonate, sumatriptan, trazadone, and verapamil. Pre-operative vital signs were stable. For pre-induction, she received midazolam (2 mg), followed by induction with remifentanil (100 mcg), lidocaine (50 mg), propofol (200 mg), and rocuronium (50 mg). Maintenance of anesthesia included a remifentanil infusion at 0.1–0.125 mcg/kg/min combined with desflurane. No events concerning TCR occurred.


A 46-year-old male with PMHx of anxiety, cluster headaches, chronic right shoulder pain, reflux, and skin cancer, presented for left sphenopalatine ganglion implant placement. His home medications included diazepam and verapamil. Pre-operative vital signs were stable. For pre-induction, he received midazolam (2 mg). For induction, he received remifentanil (100 mcg), lidocaine (50 mg), propofol (150 mg), and rocuronium (50 mg). Maintenance of anesthesia included a remifentanil infusion at 0.15 mcg/kg/min combined with desflurane. There were no intraoperative events concerning TCR.


A 70-year-old female with PMHx of cluster headaches, hypertension, and hypothyroidism, presented for right sphenopalatine ganglion implant. Home medications included levothyroxine, olmesartan, and verapamil. Pre-op vital signs were stable. For pre-induction, she received midazolam (2 mg), followed by induction with remifentanil (50 mcg), lidocaine (50 mg), propofol (120 mg), and rocuronium (40 mg). Anesthesia was maintained with a remifentanil infusion at 0.1–0.15 mcg/kg/min combined with desflurane for maintenance. No events concerning TCR occurred.


A 33-year-old male with PMHx of depression, seizures, diverticulitis, rhabdomyolysis secondary to statin use, and cluster headaches, presented for left sphenopalatine ganglion implant placement. His home medications included gabapentin, lithium carbonate, melatonin daily, sumatriptan, testosterone, topiramate, and verapamil. Vital signs pre-operatively were stable. For pre-induction, he received midazolam (2 mg), followed by induction with remifentanil (150 mcg), lidocaine (50 mg), propofol (150 mg), and rocuronium (40 mg). Maintenance of anesthesia included a remifentanil infusion at 0.1–0.125 mcg/kg/min combined with desflurane. There were no intraoperative events concerning for TCR.


A 36-month-old female child weighing 8.9 kg and with a height of 82.4 cm was admitted to the Unité Nutritionnelle et Thérapeutique (UNT) of the Centre Hospitalier Universitaire Pédiatrique de Bangui (CHUPB, the main referring pediatric hospital in the Central African Republic (CAR)) for hospitalization due to unexplained severe acute malnutrition (SAM). Clinical and physical examinations upon admission on 16 April 2012 revealed purulent otorrhea in the right ear, which had started approximately nine days earlier, and asthenia with muscle atrophy accompanied by oedema of the lower limbs, in addition to abdominal soreness associated with a diarrheal syndrome for at least the past three weeks. The treatment for SAM was initiated with F75 therapeutic milk during the acute phase followed by F100 therapeutic milk, rehydration with an oral rehydration solution (ReSoMaL), and probabilistic antibiotic therapy with ceftriaxone and amoxicillin to stabilize the health status of the patient. At the same time, HIV and malaria tests were carried out according to the CAR national protocol for malnourished children. The patient was determined to be HIV-seropositive, but the malaria test was negative. The measured T CD4+ cell count was 469 cells/mm3. The viral load was not determined. An antiretroviral therapy based on zidovudine/lamivudine/nevirapine (AZT/3TC/NVP) was initiated according to the national CAR protocol for HIV care. The presence of mild conjunctival jaundice and hepatomegaly combined with a slight increase in transaminases led to screening for infections with hepatitis B and C viruses 18 days after admission. These tests were negative. Unpublished results of screening for infection by the hepatitis A virus in the population of children under the age of five attending the Institut Pasteur de Bangui for their biological examinations have shown that 100% of these children had anti-hepatitis A antibodies (IgG), indicative of past contact with the hepatitis A virus. We hence decided to not screen for the presence of hepatitis A virus in this patient. In contrast, ELISA tests (DialPro kit for ELISA test, Italy) revealed the presence of the anti-hepatitis E virus (HEV) antibody (IgG), a marker of a prior infection, but the anti-HEV antibody (IgM), a marker of an ongoing infection, was negative. In addition, the amplification, using real-time and conventional RT-PCR techniques, of HEV RNA from the patient’s blood was positive and sequencing indicated the presence of HEV subtype 3c (GenBank accession number KP404612, ). Liver enzyme levels were measured using a Pentra 400 device (Horiba, Montpellier, France) and only the alanine aminotransferase (ALT) level was slightly higher than normal. Other parameters, such as the brachial perimeter (116) and the weight/height index (z score of −2), indicated an overall alteration of the health status of the patient. The patient died on 9 May 2012, three weeks after her admission to UNT, following continuous deterioration of her general health status, despite the treatments she received. The general health status of the patient, as well as the clinical and physical parameters, were indicative of stage four of the WHO clinical staging classification for HIV/AIDS. Since malnutrition is often a factor in immune depression, the degree of immunosuppression in this patient is likely to have been particularly severe. The family setting was surveyed and no concomitant infections with the HEV3 genotype were found in any of the other family members following RT-PCR testing; however, several family members were positive in the HEV IgG test. There were free-roaming cats and dogs, domestic pigs, goats, and chickens in and around the house of the patient, but blood and/or stools from these animals could not be taken due to the armed conflict that led to the displacement of populations and the dispersal or slaughter of these animals.


A 17-year-old girl presents at the removable prosthetics department. Her chief complaint was the unsightly retained deciduous teeth.
On examination, the patient had a short stature with lameness at walk. A cone-shaped thorax was noticed with an ability to bring the shoulders under the chest. The patient's father and brother also carry the same genetic defect. The transmission mode was recessive ().
The facial examination revealed an enlarged nose base, cheeks sagging, an unsupported upper lip, and a decreased occlusal vertical dimension (OVD). The intraoral examination showed generalized chronic periodontitis with tartar deposits in the lower anterior region. All the remaining teeth, in both the maxillary and mandibular arch, were deciduous except the four first molars were permanent. Mobility of the lower temporary incisors and canines was noticed. The upper anterior teeth were absent. The left maxillary molar was decayed (Figures and ). The maxilla was hypoplastic with a relative prognathism of the mandible. An anterior and lateral crossbite was diagnosed ().
Casts were made and articulated at the patient's correct OVD. Registration was recorded with an interocclusal bite (Moycowax®, Aluwax®). An interocclusal rest space of 6 mm was evaluated ().
The orthopantomogram revealed the presence of many unerupted supernumeraries and permanent teeth that had not achieved their root formation, especially in the maxillary premolar regions. The second upper left molar had a suprabony situation. Only its crown was formed. The lower permanent incisors achieved their root formation. They present a right eruption pathway (). A cone-beam computed tomography examination was performed. It showed a distorted and incomplete root formation of the permanent teeth. The two definitive upper central incisors present a juxta-bone situation with a right eruption pathway. However, only its crowns were formed. The lower permanent incisors had submucosal situation ( and ). The frontal radiograph showed the presence of multiple wormian bones, a widened sagittal suture, and an underdevelopment of the maxilla and paranasal sinuses (). The left clavicle was hypoplastic with a deformation of the vertebral column in the thorax X-ray ().
Upon a scanning electron microscopic examination, the extruded deciduous teeth showed normal structures either for the enamel, dentine, or pulp (Figures and ).
After obtaining the patient's consent, the lower incisors and canines were extracted (). The patient had an incomplete root development of her permanent unerupted teeth with a generalized chronic periodontitis that did not allow orthodontic intervention. The treatment was oriented to the prosthodontic option. The patient had one permanent tooth in each hemiarch. Moreover, there was an important interocclusal rest space. These conditions could not allow the exploitation of the remaining permanent teeth as abutments for fixed prostheses. The use of implants was also eliminated because the patient refused a surgical extraction of the supernumerary teeth in the implant site. In addition, there was a risk of bone fragility given the high number of supernumerary and unerupted teeth to be extracted.
The decision was to make a complete removable overdenture at the maxilla and a partial removable denture at the mandible. The deciduous teeth were used as abutments for a complete prosthesis.
Individual trays were fabricated using diagnostic casts. Master impressions were recorded in polysulfide material (Surflex®). Occlusal registration was performed at the correct reestablished OVD. The mounted prosthetic teeth were checked and approved by the patient. The two removable prostheses were polymerized, fitted, and adjusted in the patient's mouth. Retention and stability of prostheses were excellent. The patient was very pleased and satisfied with her new appearance ( and ).
The patient consulted every 2 months for a check-up. She has been followed for 4 years. The deciduous canines with mobility were extracted, and the prostheses were relined. Although the primary lower incisors and canines were avulsed, the corresponding permanent teeth did not erupt.


An 82-year-old Hispanic woman with a past medical history of hypertension presented to the emergency department with malaise for three days. She also experienced nausea, vomiting, fatigue, lower extremity pain, shortness of breath, cough, and oliguria. No diarrhea, weight loss, history of malignancy, or kidney disease were reported. Her vital signs were normal, and her physical examination was unremarkable. The results of initial labs revealed low platelets with concomitant elevated creatinine, lactic acid dehydrogenase, international normalized ratio, and activated partial thromboplastin time; her haptoglobin level was undetectable (Table ). A peripheral smear showed red blood cell schistocytes consistent with microangiopathic hemolytic anemia. Urinalysis showed microscopic hematuria and proteinuria. She was admitted with the diagnosis of microangiopathic hemolytic anemia and acute kidney injury. A non-tunneled dialysis catheter was placed and she underwent plasma exchange and was given steroids. Stool collection for Escherichia coli O157:H7 testing was not feasible as this patient did not have a bowel movement during hospital admission. She was later found to have an extensive lower extremity DVT, and heparin was administered. A workup for malignancy was ordered along with computed tomography (CT) scan of the chest/abdomen/pelvis. The CT scan revealed a pulmonary fibrosis pattern and multifocal areas of consolidation in the upper lung (Figure ). Tumor markers (CEA, CA 19-9, CA 125, AFP) were negative. Rituximab was started as the patient’s ANCA results were positive. Her platelet count and creatinine levels improved significantly following treatment (Table ).
A kidney biopsy was done following the resolution of her thrombocytopenia. Light microscopic examination of the kidney specimen revealed 23 glomeruli, of which six were globally sclerotic, one demonstrated early glomerular capillary thrombosis, and another demonstrated a cellular crescent (Figure ). There was no evidence of vasculitis, but moderate arteriosclerosis was present. Mild interstitial fibrosis and tubular atrophy were found in the tubulointerstitium, involving 10% to 15% of the renal cortical tissue. Electron microscopy showed no electron-dense immune-type deposits or organized deposits. Immunofluorescent staining (IgG, IgA, IgM, C3, C1q, kappa, and lambda) of the glomeruli were negative.
During the course of treatment, this patient developed an acute bowel perforation not amenable to treatment and expired.


We report a case of a hypertensive and diabetic 70-year old Caucasian male who presented with a four-week history of occipital headaches, confusion, and right-sided facial twitching. These are accompanied by a decline in cognition and difficulty with short-term memory. The patient has been taking atorvastatin, losartan, and metformin for his metabolic derangement. This patient had an unremarkable family history and no previous bleeding episodes. Prior dental appointments and hemostatic challenges in the past were as well uneventful.
Examination revealed mild confusion (GCS 14, E4V4M6) but otherwise unremarkable physical and neurological findings. Review of systems were equally normal. Initial CT Brain scans have shown an iso-dense intra-axial lesion causing compression of the left foramen of Munro and subsequent hydrocephalus. Magnetic Resonance Imaging indeed confirmed a thalamic mass in which the appearance is in keeping with an infiltrative glioma (Figure ). A biopsy with septum pellucidotomy was contemplated to ascertain the diagnosis and address the hydrocephalus.
Prior to the surgical procedure, pre-operative blood chemistry showed an isolated elevation in the activated partial thromboplastin time (aPTT) [43.5; NV:28-34]. Prothrombin time (PT) and platelet counts were within normal limits. Other hematologic investigations were therefore undertaken (Table ).
In the absence of a bleeding history coupled with the above results, the patient was hence diagnosed with a severe avWS. A single dose of 60g intravenous immunoglobulin (IVIg) was administered with respect to the patient’s ideal body weight. Subsequent assays have shown improvement in the vWF antigen, vWF RiCof and Factor VIII levels (Table ). The patient underwent surgery and histopathology has subsequently confirmed a GBM (WHO IV - High Grade). Due to the persistence of this patient’s cognitive impairment and symptomatology despite the initial intervention, adjuvant therapy has been disregarded and best supportive care was considered. The patient was monitored continuously by the Hematology department upon discharge. Unfortunately, further investigations for autoimmune disorders and other myeloproliferative disorders which could have as well been associated with the aVWS were not anymore undertaken as the patient was deemed for palliation.


An 80-year-old male presented to the emergency department with complaints of abdominal pain and fevers. The patient’s past medical history is significant for bladder cancer. One month ago, he underwent a radical cystoprostatectomy with ileal conduit placement. Prior to surgery, he was treated with neoadjuvant nivolumab and ipililumab. He stated that over the past two weeks, he had been having abdominal pain and fevers. The fevers were gradual in onset, intermittent, with a max temperature of less than 100.4°F. Along with the fevers, he complained of left flank and groin pain, aching, which worsened from positional changes. He was taking Tylenol for the pain and fevers for the past two weeks. He denied any signs of hematuria, dysuria, nausea, vomiting, or diarrhea. He denied any recent travels or sick contacts. The patient reported adequate output from the ileal conduit. The patient denied any nausea, vomiting, diarrhea, constipation, or shortness of breath at this time. Vitals were within normal limits and the temperature was 100°F on presentation. We performed routine labs, blood cultures, urinalaysis from the ileal conduit, and urine cultures (Table ).
A computed tomography of the abdomen and pelvis with contrast showed the presence of a large lymphocele (Figure ).
We empirically started him on ceftriaxone. The patient also underwent a drain placement and fluid cultures from the lymphocele were sent. Two days later, blood cultures and fluid cultures showed the presence of S. lugdunensis with susceptibilities (Table ).
Ceftriaxone was stopped and vancomycin was started. Patient was subsequently discharged two days later with 2-week course of intravenous vancomycin. Patient had resolution of his symptoms after two weeks and had negative blood cultures.


A 42-year-old male with a past medical history of Type II diabetes mellitus presented to the emergency department with a chief complaint of left knee pain starting one week prior. Vital signs on admission showed a blood pressure of 97/66 mmHg and a heart rate of 100 beats per minute. The initial body temperature was 98.7 degrees F followed by a subsequent rise to 102.8 degrees F after 24 hours. The physical exam was remarkable for left knee edema and erythema with a 5 cm lump in the prepatellar region that was warm and tender to palpation. Prior to presentation, the patient was on metformin 1 gram every 12 hours but was non-adherent. He was not taking any renin-angiotensin-aldosterone system (RAAS) inhibitors or statin therapy. Initial workup revealed serum creatinine level of 0.9 mg/dL and blood urea nitrogen (BUN) level of 19 mg/dL associated with a blood glucose of 610 mg/dL, a bicarbonate level of 24 mEq/L, hyponatremia at 126 mEq/L (corrected sodium of 134 mEq/L) with anion gap of 12. Potassium was 4.7 mEq/L. Magnetic resonance imaging (MRI) of the left knee showed pre-patellar abscess with bursitis (Figure ).
The patient was hospitalized for septic bursitis and intravenous fluids with empirical antibiotic therapy of vancomycin and piperacillin-tazobactam at the doses of 1.25 grams every 12 hours and 4.5 grams every six hours respectively, were initiated. Drainage of the abscess was performed with an elevated count of polymorphonuclear leucocytes (PMN). The tissue culture was negative for any microorganism growth. Increase of serum creatinine and BUN levels to 1.4 mg/dL and 29 mg/dL, respectively, occurred on day three of hospitalization suggesting acute kidney injury, which normalized after intravenous fluids. Infectious diseases service was consulted on day 12 of hospitalization given frequent out of range serum levels of vancomycin trough and concerns of the patient regarding repeated monitoring of blood level of vancomycin. Therefore, it was recommended to switch vancomycin to daptomycin 400 mg IV daily. To that point, laboratory findings did not show a single episode of hyperkalemia. On day 13 of hospitalization, 24 hours after daptomycin was started, the serum level of potassium increased to 6.3 mEq/L without electrocardiographic changes. Oral binding resin was given with a subsequent decrease of potassium levels to 5.1 mEq/L 12 hours later (Figure ).
Common causes of isolated hyperkalemia were considered. Among them, heparin was discontinued and tighter finger stick glucose controls were made with a goal of <180 mg/dL. Potassium levels were again registered elevated on the second and third day (days 10 and 11 after hospitalization) after daptomycin was initiated (Figure ). Aldosterone level of 4.8 ng/dL (reference range: <23.2 ng/dL), a renin level of 0.732 ng/mL/hour (reference range 0.167-5.380 ng/mL/hour) and a fraction excretion of urine sodium (FeNa) calculated of <1% with normal chloride levels of 102 mEq/L excluded the possibility of renal tubular acidosis (RTA) type 4. Plasma potassium level was started to be measured instead of serum potassium to rule out pseudo-hyperkalemia caused by laboratorial artifact.
Daily potassium monitoring showed again an increment of potassium level after daptomycin infusion on three more occasions and the patient was again treated with oral binding resin three more times (Figure ). On day 14 after daptomycin initiation, CPK had a significant increase from the normal range measured two days prior to 4,537 mg/dL and the diagnosis of rhabdomyolysis was made.
Algorithms like the World Health Organization-Uppsala Monitoring Centre (WHO-UMC) system and the Naranjo algorithm are commonly used to assess the likelihood that an adverse reaction was caused by a medication. We used the Naranjo algorithm, which is less prone to subjective variations compared to other systems []. The modified Naranjo algorithm asks 10 questions related to the probability for a drug to be the cause of the adverse reaction and provides a maximum score of 13 points. Scores from 1 to 4 points make the reaction possible, from 5 to 8 points probable, and above 9 points make it definite. Based on the Naranjo likelihood algorithm, it was probable that daptomycin could be the main factor.
Daptomycin was discontinued and vancomycin was reinitiated. Once daptomycin was discontinued and intravenous fluids were provided, no new potassium derangements were registered throughout the rest of his hospital stay and CPK levels returned to normal levels with no further elevations. The patient completed a four-week-course of antibiotics with vancomycin and cefepime. No new episodes of hyperkalemia were registered and the patient was discharged home without complications.


A three-year-old Saudi girl, with no known chronic illness, presented on the 2nd of October 2017 at the Emergency Room at Prince Mohammed Bin Abdulaziz Hospital, Ministry of National Guard Health Affairs, Medina, Saudi Arabia. She was accompanied by her parents, with a bilateral profuse non-stopping epistaxis lasting for a few hours. There was no bleeding from other orifices, she was conscious, alert, oriented, and calm, with normal vital signs (temperature 36.60 C, respiratory rate 24/minute, oxygen saturation 96% on room air, heart rate 110 beats/minute, and blood pressure 121/77 mmHg), not dysmorphic, and was not cyanosed or distressed. She was bleeding actively (epistaxis) and there were multiple ecchymoses over her upper and lower extremities (Figure , ) and abdomen with different colors and sizes. There was no hepatosplenomegaly or lymphadenopathy. Other systemic examinations were unremarkable. There was no history of trauma or drug ingestion. Another systemic review was unremarkable. After vomiting a large amount of digested blood, becoming paler and symptomatic with increased tachycardia, her hemoglobin started to drop from 11.5 g/dl to 8 g/dl (other complete blood count (CBC) indices remained normal), so she was transfused with packed red blood cells, fresh frozen plasma, as well as recombinant Factor VII, which were administrated to treat the symptomatic anemia and to stop the severe uncontrolled epistaxis. The Ear Nose and Throat team were consulted and they found multiple bleeding vessels, which were cauterized locally. Almost all bleeding completely stopped six hours post-admission.
The patient is a product of a full-term pregnancy, with a spontaneous vaginal delivery, no neonatal intensive care unit (NICU) admission, not known to have any chronic illness, not on any medication, and never received a blood transfusion before. There was a history of prolonged wound healing and profuse bleeding due to minimal injuries at the age of six months. Her parents are cousins and she has two siblings, all are healthy, with no family history of bleeding disorders.
Glanzmann thrombasthenia (GT) had initially been suspected, as this type of platelet dysfunction is common in our region. An initial bleeding-related work-up was done including CBC. The coagulation profile showed a normal platelet count, normal partial thromboplastin time (PTT), international normalized ratio (INR), normal Factor XIII, and the von Willebrand factor (VWF) panel was normal (Table , ). Flow cytometry was done to rule out GT, which was normal. ITGA2B, ITGB3, and VWF gene sequencing were done and no relevant variant was detected. A platelet aggregation study (more than one reading) revealed a normal response to ristocetin and arachidonic acid but had an abnormal response to collagen, adenosine diphosphate (ADP), and epinephrine (Table ). The peripheral blood smear was normal.
The results of a genetic study (performed at Bioscientia Human Genetics, Ingelheim, Germany) identified a homozygous RASGRP2 VUS c.956 C>T p. (pro 319leu) which confirmed the diagnosis of BDPLT18, and based on this final genetic result, we recommended checking all the family members for the same gene. We found that all the other family members are heterozygous carriers of the same familial RASGRP2 VUS.