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An 11-year-old girl presented to our emergency department with a 2-day history of nausea, vomiting and headache. Her medical history included varicella infection at 5 years of age. Physical examination revealed a deviation of the left lip commissure, incapacity for total occlusion of the right eye and asymmetry of the wrinkles in the forehead (House-Brackmann Facial Nerve Grading Scale (HB FGS) V). She had crusting vesicular lesions on the right ear pinna (Figure 1,2 and 3). Meningeal signs were negative. CT scan and blood analysis were unremarkable. Cerebrospinal Fluid (CSF) showed an elevated white cell count (210 u/L) but normal glucose and protein levels. Based on clinical and analytical findings, Ramsay Hunt Syndrome complicated with viral meningitis was diagnosed at this point. The patient started oral prednisolone (60 mg/day), intravenous acyclovir (1500 mg/m2 /day) and physical rehabilitation. Subsequent molecular testing of CSF detected Varicella Zoster Virus (VZV) RNA by reverse transcriptase PCR. The follow-up showed disappearance of the earache and vesicular rash, as well as the improvement of facial paralysis, becoming a grade III HBFGS (Figure 4,5 and 6). The one-year follow-up showed no relapse of the syndrome (Figure 7 and 8). Ramsay-Hunt Syndrome is a rare disease characterized by facial paralysis, inner ear dysfunction, periauricular pain, and herpetiform vesicles (zoster oticus). The reported incidence in children is 2.7/100,000 and it´s caused by the reactivation of latent VZV in the geniculate ganglion [1-3].
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A 61-year-old man with mitral valve prolapse presented with 1-week history of fever, palmar rashes and pain over the right thenar eminence. On admission, he was found to have a pansystolic murmur and haemorrhagic macules over his palms. A painful, non-pulsatile swelling was noted at the right thenar eminence. Echocardiography revealed a 0.6 x 0.4cm vegetation on the anterior mitral valve leaflet and severe mitral regurgitation. Magnetic resonance imaging (MRI) revealed an irregular intramuscular collection between the right 1st and 2nd metacarpals. Computed Tomography (CT) and MRI revealed scattered infarcts in the liver, kidney, spleen, and brain. Two sets of blood cultures were positive for cloxacillin-sensitive Staphylococcus aureus. Fever resolved following IV Cloxacillin.
On the 3rd-week of antibiotic treatment, the right thenar eminence swelling had enlarged and was pulsatile (Figure 1 and 2). Ultrasound and MRI confirmed the presence of right radial artery aneurysm (Figure 3 and 4). Surgical excision and repair excision was performed. Mycotic aneurysms of the peripheral arteries are rare and can lead to significant complications e.g. haemorrhage and limb loss. To the best of our knowledge, there are very limited reports regarding transformation of myositis to mycotic aneurysm. Thus, early detection and intervention are crucial to ensure optimum outcome.
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A 34-year-old gentleman presented with reduced effort tolerance and palpitation for one-week duration. He had underlying thalassemia intermedia diagnosed at the age of 7 years old. Clinically, he was pale with frontal bossing, nasal bridge depression, prominent maxillary bones and hepatosplenomegaly. His hemoglobin level was 4.5g/dl and he required an admission for transfusion. A chest radiograph showed multiple ribs expansion bilaterally, indicates features of extramedullary hematopoiesis. The computed tomography of thorax showed diffuse bony changes with medullary expansion, cortical thinning and coarse trabeculation involved the ribs and paravertebral consistent with extramedullary hematopoiesis. His average hemoglobin level for the past few years were between 4g - 7g/dl indicating that he had been under-transfused contributing to his prominent extramedullary hematopoiesis features.
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Gillespie syndrome is a rare, congenital disease that typically presents in childhood with cerebellar ataxia, aniridia, and intellectual disability. Since the first case was reported in the literature in 1965, recent studies have shown that the mutations in Gillespie patients occur in the 1,4,5- trisphosphate receptor type 1 (ITPR1) gene. In our study, we present the first case of genetically diagnosed Gillespie syndrome in a child who does not present with aniridia. This child of first-degree consanguineous parents presents with a novel homozygous mutation in the ITPR1 gene. In addition, her other symptoms are mild when compared to known Gillespie syndrome cases in the literature, and this is the first reported case of Gillespie syndrome in Lebanon and the Middle East. This underscores the importance of genetic testing when diagnosing neurological syndromes, and the potential for multivariable presentation of patients with such syndromes.
Keywords: Gillespie syndrome; Aniridia
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A 53-year-old woman presented with sudden, painless loss of vision in the left eye and vision of counting fingers. She was a known diabetic and hypertensive on medical management. Left eye retinal examination revealed retinal whitening at the posterior pole along with cherry red spot at the macula and presence of emboli called Hollenhorst plaques in the superotemporal and inferotemporal branches of the retinal arteries (arrows). Immediate ocular massage was done to dislodge the emboli but visual improvement was minimal. Carotid doppler and a full cardiac work-up was advised which was found to be normal. Hollenhorst plaques are cholesterol emboli, the most common form of emboli causing branch or central retinal artery occlusions. They are yellow, refractile and originate from carotid or aortic atherosclerotic plaques.
Hollenhorst plaques are important as they can be predictive of an impending ischemic stroke especially in the elderly. Immediate carotid doppler should be ordered in these patients to assess carotid artery stenosis as well as a cardiological work-up. If the lesion is ulcerated and causing more than 70% stenosis, the patient should be for stenting to an interventional radiologist [1].
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A 54-year-old man with no prior medical care presented with acute vision loss in the left eye. Visual acuity was hand motion and blood pressure were 220/136. Examination demonstrated severe disc and macular edema, retinal whitening, venous sheathing, and intraretinal hemorrhages (Panel A). Fluorescein angiography revealed venous leakage and non-perfusion (Panel B). The patient underwent empiric intravitreal foscarnet. After infectious/inflammatory etiologies were ruled out, he received 3 monthly intravitreal bevacizumab injections for presumed central retinal vein occlusion. At 3 months, there was complete resolution of sheathing (Panel C) but with persistent macular ischemia (Panel D) resulting in counting fingers vision.
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We report on two cases who suffered from an unstable type 2 odontoid fracture following a skiing and a domestic fall accident. Prior to anterior screw fixation surgery both patients presented without neurologic deficits but postoperatively developed a bilateral me-dial medullary syndrome (MMS), including an incomplete tetraparesis, impaired sensation of position and movement as well as tactile discrimination and paralysis of the tongue muscle with deviation to the paralyzed side. MRI showed a typical heart-shaped ischemic lesion in the medial medulla bilaterally. The search for aetiologic factors was uneventful in both patients except for severe atherosclerosis. MMS has not been reported after ante-rior screw fixation of an odontoid type 2 fracture. Due to the close proximity of the ischemic area to the surgical site we here propose the perioperative mechanical manipulation of the upper cervical spine during surgery as a new etiology for MMS.
Keywords: Bilateral medial medullary syndrome; Type 2 odontoid fracture; Anterior screw fixation; Stroke etiology
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A 94-year-old patient presented to the emergency department with retrosternal and epigastric pain; he was diabetic and hypertensive, in therapy with oral anticoagulants and with a history of prostate cancer, without on-going cytotoxic drug treatment. He had no fever or dyspnea. By a routine check-up performed four months ago, no biochemical tests or blood count abnormalities were found.
Complete blood count performed on a haematological analyser registered a newly elevated WBC count (48.7 x 109/L), a mild anemia (Hb = 12.7 g/dL) and thrombocytopenia (39 x 109/L). In the SWDF channel for differential leukocyte count there was an anomalous WBC scattergram, with unseparated cell clusters and specific instrumental flags.
The morphological examination of the peripheral blood smear, performed with an automated digital morphology analyzer, showed the presence of numerous small to medium-sized lymphocytes (27.5%), with scant cytoplasm and "clod" nuclear chromatin, and the presence of some nuclear shadows, suggesting a diagnosis of chronic lymphocytic Leukemia (CLL). Surprisingly, it was also noted the presence of some medium to large-sized elements (25.0%), with immature chromatin, monocytoid aspect, basophilic cytoplasm sometimes vacuolized and evident nucleoli.
A comprehensive flow-cytometry (FC) antigen panel was used. Acquisition of data was performed using a flow cytometer. FC analysis revealed a subset of mature clonal B-cell population with the classical immunophenotype of CLL (CD19+, CD20+dim, CD5+, CD200+, CD23+ and clonal kappa+ light chain with weak expression) and a second pathological blast cells population was found, characterized by a monocytic differentiation, with lack of expression of mature monocytic antigens (CD14 and CD300) and aberrant overexpression of CD56.
WBC count doubled after 2 days, with the increase of both populations (respectively, 34.2% lymphocytes and 39.0% blasts). The patient, unfortunately, had a poor prognosis and he died three days after hospitalization, for acute kidney injury and heart failure.
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Epithelioid Trophoblastic Tumor is a rare form of gestational trophoblastic neoplasia only reported in approximately 110 cases in literature. We present the case of a 50 years old woman incidentally diagnosed with an ETT on the site of surgical repair of a ventral incisional hernia after a Caesarean section.
Keywords: Epithelioid trophoblastic tumor; ETT; Ventral incisional hernia; Gestational trophoblastic neoplasia; GTN
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In an attempt to overcome the physiological and visible signs of facial aging, scientists and formulators diligently work to elucidate strategies for its intervention. It has been demonstrated that the natural slowing in the production of endogenous glycosaminoglycans, hyaluronic acid (HA) in particular, leads to an acceleration in cutaneous aging [1]. Additionally, the normal stratum corneum or barrier function that is key to proper moisture retention tends to worsen with age in some ethnic groups [2-6]. Hyaluronic acid is one of the naturally occurring glycosaminoglycans in human skin that shows an impressive ability to bind to and hold 1000 times its weight in moisture within the skin [7]. It is therefore a logical strategy to develop products to deliver essential hyaluronic acid into the skin to mitigate the negative effects of a loss of HA in dry and aging skin. The PCA SKIN Hyaluronic Acid Overnight Mask, a combination of HA of varying molecular weights, was tested for efficacy in-vivo for both immediate and eight hours moisture increase in human skin.
Keywords: Hyaluronic acid; Aging skin; Moisture mapping; Moisturization; Corneometer; Facial mask
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Introduction: Pneumothorax, pneumomediastinum and pneumopericardium are three clinical conditions in which air is present abnormally within the pleural space, mediastinal peri-bronchial and perivascular areas and pericardial sac respectively. Reports from around the world mentioned COVID-19 inducing the above entities in adult patients either separately (pneumothorax alone) or in combination (pneumomediastinum and pneumopericardium).
Case description: We present the first pediatric case worldwide of a 1-year-old previously healthy girl presenting to the emergency department of Rafik Hariri University Hospital, Lebanon with tachypnea and cyanosis. CT scan of the chest was done and revealed severe massive bilateral pneumothorax, pneumomediastinum and pneumopericardium.
Discussion: COVID-19 is no more the easy-going infection for pediatric population as was thought previously upon the emergence of the pandemic. Many children have been reported to develop multi-organ failure requiring intensive care. A new entity to be feared in pediatric patients is the alveolar rupture into the intrathoracic spaces which may occur even prior to establishment of mechanical ventilation.
Conclusion: High suspicion for severe COVID-19 complications must be present when facing a clinically unstable patient during the COVID-19 pandemic. Rapid intervention must be established to avoid clinical deterioration of the patient.
Keywords: Pneumothorax; Pneumomediastinum; Pneumopericardium; SARS CoV-2; Pediatrics
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Sigmoid stricture secondary to repeated episodes of complicated diverticulitis has an incidence of 0.09%. Our patient is a 36-year-old Caucasian male who presented with a significant history of worsening abdominal pain for over 11 years. He was repeatedly treated with a non-operative approach that consisted of antibiotics during multiple hospital admissions. A sigmoidoscopy was attempted by his gastroenterologist, but the scope was unable to pass the sigmoid, increasing the concern for stricture and potential large bowel obstruction. After a complete course of antibiotics, he was referred to our surgical team. Confirmation with an abdominal computed tomography (CT) scan showed that the inflammatory mucosa had subsided. A colonoscopy was then conducted by the surgical team and biopsies were obtained. A one-stage laparoscopic sigmoid resection with colorectal anastomosis was performed.
Keywords: Diverticulitis; Diverticulosis; Sigmoid stricture; Case report
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Two separate cases of 10-day old new-borns presenting to the Emergency Department with superficial chemical burns to the skin around the umbilical area secondary to improper application of silver nitrate in primary care. In both cases, the new-borns had been seen in primary care on day 9 of life due to concerns from parents regarding the appearance of the umbilical cord. In each case, the umbilical cord had separated on day 6 of life. At the time of assessment in primary care, the umbilical cord appeared pink and moist. The appearance was mistaken for an umbilical granuloma and consequently treated with topical silver nitrate.
The umbilical cord typically separates within the first week of life but can take up to 14 days, whereas umbilical granulomas appear a few weeks after the separation of the cord. An umbilical granuloma is an overgrowth of granulation tissue that appears as a soft, pink nodule with associated serous discharge. In each case, the new-borns presented 3 days after the umbilical cord separated making the diagnosis of umbilical granuloma unlikely. In both cases, silver nitrate was used as first line treatment for a suspected umbilical granuloma. Cases of silver nitrate burns have been well documented over the past two decades. In each case, silver nitrate was applied without the use of Vaseline to the surrounding area which helped precipitate the chemical burns. Furthermore, parents were not advised to keep the nappy below the level of the umbilicus following silver nitrate application. Lastly, topical application of salt crystals is an effective and safe treatment for umbilical granuloma with a 100% resolution rate in a recent study by Bagadia et al. Given the risks associated with silver nitrate, it may prove safer to use salt crystals as first line treatment for suspected umbilical granulomas.
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A 62-year-old man with a history of hypertension and symptomatic bradycardia requiring a permanent pacemaker came in with worsening shortness of breath associated with decreased exercise tolerance and orthopnea. The examination was remarkable for pan systolic murmur at the apex, jugular venous distention, and decreased breath sounds in the right lung base. Chest radiograph (Figure 1) revealed cardiomegaly and a pleural-based rounded mass in the right pleural space inferiorly. Computed tomography (Figure 2) showed a loculated cystic mass (label A) (13 x 9 x 10 cm) measuring slightly above water density and apical bullae. An echocardiogram revealed severe left atrial and left ventricular dilation with an ejection fraction of 55% and prolapse of the anterior leaflet of the mitral valve (MV) with severe mitral regurgitation (MR). He was started on intravenous loop diuretics for congestive heart failure due to MR with improvement in symptoms. Later he underwent Mitra-Clip placement with improvement in MV function.
The loculated cystic mass in the lung was found to be Pulmonary Plombage which was placed in 1993 due to recurrent pneumothorax. Plombage (extra-pleural pneumonolysis) is a surgical treatment method used to treat cavitary lesions of the lung in patients with pulmonary tuberculosis (TB) during 1930-50s before the introduction of anti-TB chemotherapy by creating a cavity surgically under the ribs in the chest wall and filling the space with inert material [1]. The materials used in this procedure are acrylic balls, fat, wax, rubber, and mineral oil (oleothorax), where the latter was used in our case.
Rarely, Plombage is used to seal a large bronchopleural fistula that causes refractory pneumothorax, with only one reported case in the literature [2]. We present another case of Pulmonary Plombage used to treat refractory pneumothorax. Plombage of the pleural cavity connecting to the pleural space can be effective in treating refractory pneumothorax.
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Background: In the setting of severe acute respiratory syndrome coronavirus 2 (SARSCoV?2) infection, data from autopsy in subjects who died at home during lockdown are scarce. We here report a forensic autopsy series of coronavirus disease 2019 (COVID-19)-related out-of-hospital cardiac arrest (OHCA).
Methods and Results: Between March and April 2020, four COVID-19-related OHCA were autopsied at the Institute of Legal Medicine of the metropolitan area of Lyon (France). Autopsies of 3 individuals reported natural death by acute respiratory failure implicating SARS-CoV-2 with typical COVID-19 pulmonary aspect of gross findings and pulmonary microscopy findings, i.e. diffusely congestive edematous lungs with peripheral thrombi and diffuse alveolar damage (DAD) at different stages of inflammatory reaction. For one individual, autopsy concluded of violent death due to suicidal acute alcohol intoxication in a patient that could no longer endure COVID-19 lockdown. No significant lesions were found in the heart.
Conclusions: We report here OHCAs of non-cardiac cause directly implicating COVID?19 at various stages of SARS-CoV-2-related DAD. This autopsy remains of interest during this epidemic, both legally and medically to better understand the pathogenic processes of this infectious disease.
Keywords: Autopsy; COVID-19; SARS-CoV-2 infection; Cardiac arrest
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Introduction: Multisystem inflammatory syndrome in children (MIS-C) is the dangerous complication in the pediatrics population associated with Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2). It leads to the injury of several organs and impairment of their function. Treatment protocols have been placed to avoid the long-term consequences of this syndrome which may be severe enough to cause death of the affected patient.
Case description: A 50 days old boy with no previous medical conditions presented to the hospital with respiratory distress and had a negative reverse transcription polymerase chain reaction (RT-PCR) test for novel coronavirus and his family denied contact with a COVID-19 infected individual. The patient clinical status deteriorated and was not responding to antibiotics and nebulizers. This raised our suspicion for MIS-C so a SARS-CoV-2 serology was performed and were found out to be positive. Treatment started but the patient deteriorated rapidly and ended up intubated on mechanical ventilation then passed away after a cardio-pulmonary arrest.
Discussion: During the COVID-19 pandemic, respiratory complaints must be considered to be associated with coronavirus infection and must not be ruled out by just performing a PCR test from a nasopharyngeal sample. Knowing that MIS-C usually do not appear during the acute phase of coronavirus infection but rather after the resolving of the infection.
Conclusion: This case raises the necessity of performing serology for SARS-CoV-2 for a patient presenting with respiratory complaints and with non-resolving multi-organ inflammatory injury. Also, no age group can be considered safe and protected from complications of coronavirus infection. This is why serious precautions must be applied when a suspected or infected individual is dealing with other people.
Keywords: MIS-C; SARS-CoV-2; Pediatrics; Serology
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A 64 year old lady presented for routine mammogram at which a left breast lump was isolated. Subsequent biopsy of this lesion surprisingly showed Amyloid light chain (AL) kappa type amyloidosis. Further investigations did not show any evidence of systemic amyloidosis on bone marrow trephine, Serum amyloid P component scintigraphy, echocardiogram or serum free light chain assay and thus she was diagnosed with localised breast AL amyloidosis. Interestingly, she clinically displayed mild macroglossia which can be associated with Sjogren’s syndrome which has an established association with amyloid deposition of the breast. Here we display images of the initial screening mammogram and the subsequent diagnostic breast biopsy. Breast amyloidosis is a very rare phenomenon and has only been reported as individual case reports or small case series. It is usually reported in conjunction with additional haematological malignancies, thus making this case of localised Breast AL Kappa type Amyloid deposition even more unusual.
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