A 63 year old Caucasian female with ampullary pancreatic cancer s/p pancreaticoduodenectomy in 2016 was admitted to our cancer center with complaints of fever (temperature 103° F) and abdominal pain. She was found to have multiple liver abscesses. Her blood culture grew out Shewanella algae. We herein report on this rare human bacterial infection.
A suspected insulinoma with the unexpected histopathological finding of a nesidioblastosis – A case report and literature review
Introduction: Nesidioblastosis is a hyperplasia of the beta cells that causes persistent hyperinsulinemic hypoglycemia, unfortunately, it is difficult to diagnose through imaging studies. Case presentation: A 68-year-old woman with palpitations, diaphoresis, dizziness and alterations in the level of consciousness that improve with food intake. Laboratories: glucose 35.0 mg/dl, insulin 12.5 mUI/ml, proinsulin 14.1 pmol/L and peptide C 2.55 ng/ml. Octreoscan reveals an abnormal epigastric uptake area and the tomography shows a focal hypervascular lesion in pancreatic body of 12×11 mm. A distal pancreatectomy was performed without palpating the tumor in transoperative period, and a diffuse nesiodioblastosis was reported in the histopathological study. The patient persists with hypoglycemia and an additional pancreatic resection was performed, resecting 90% of the organ. Although an insulinoma was not located in the piece, an area surrounded by connective tissue was observed where islet hyperplasia was more accentuated. Discussion: The pathophysiology of nesidioblastosis is unknown, its main differential diagnosis is insulinoma and this should be suspected when the tumor can not be identified. Most agree that a pancreatectomy that involves 60-80% of the total organ can control glucose levels with low risk of diabetes and pancreatic insufficiency. Conclusion: The nesidioblastosis should be suspected when a hyperinsulinemic hypoglycemia is difficult to control and when a tumor can not be identified. The extension of the pancreatectomy should be individualized and if an insulinoma is not localized and a nesidioblastosis is suspected, an intraoperative histopathological examination with frozen section evaluation for the margins could determine the extent of pancreatectomy.
Pleomorphic adenoma is a benign tumor of the salivary gland with the highest incidence rate among all salivary gland tumors in the general population. Even though it’s common, ectopic presentation of the same is rare. When it happens, it presents in the head and neck region. We discuss in this paper a rare case of ectopic pleomorphic adenoma on the plane of the cheek.
We report a near fatal rectal perforation due to a phosphate enema in an elderly male. The presentation in septic shock within 4 hours of the enema is quite rare. Early recognition and prompt management are essential for a good outcome. A defunctioning colostomy is standard for these cases but we recommend a distal rectal washout since intraluminal faeces in a loaded rectum could be a cause of ongoing sepsis. Although enemas are commonly used for constipation in the elderly, suppositories and oral preparations should be used preferentially where appropriate.
Methemoglobinemia – A review and recommendation for management – Acute methemoglobinemia with hemolytic anemia following suicidal use of aniline based substance
Methemoglobinemia is a rare disease classification related with congenital or acquired (usually iatrogenic) hemoglobin oxygenation disorder. Despite the fact that number of potentially methemoglobin forming agents is very long, methemoglobinemia is still a case-report of patients admitting to the Emergency Departments. The patient was brought after consuming resin hardener for suicidal purposes from the Center of lower reference to the hospital Emergency Department with suspected burns of the gastrointestinal tract. The patient presented cyanosis, dyspnoea and brown-colored urine on admission.
Sarcoidosis and tuberculosis are chronic granulomatous diseases with predominant pulmonary involvement. Simultaneous occurrence of sarcoidosis and tuberculosis is a rarely encountered clinical entity. We present two Sri Lankan males with coexisting bacteriologically confirmed tuberculosis and histologically diagnosed sarcoidosis. Careful and timely commencement of anti-tuberculous treatment followed by steroids treatment for sarcoidosis lead to improvement of both conditions. These cases are reported to describe the dilemmas in diagnosis and management of this underrecognized clinical entity.
Classic Neuroimaging Features in a Case of Congenital Muscular Dystrophy [Fukuyama Variant] – a Rare Cause of Infantile Hypotonia in an Indian Male
The aetiological diagnosis in an infant with hypotonia is a challenging task for a clinician due to variable and long list of differentials . It could be due to an insult within the central nervous system (CNS) or less commonly result from a peripheral defect at neuro-muscular level and other miscellaneous causes (rickets, hypothyroidism) . Most commonly it is a central hypotonia where the muscular weakness is absent or not profound. In Indian scenario, it is mostly idiopathic central hypotonia, followed by HIE (cerebral palsy) . In cases of cerebral palsy, neuro-imaging reveals the severity of affliction. The peripheral aetiologies like congenital myopathies, congenital myasthenia, infantile botulism etc. are rather rare occurrences. Infants with congenital muscular dystrophy have muscular dystrophy, central neural affliction and involvement of multiple systems (skeletal, cardiovascular, respiratory, ocular etc.). It is a rare disease with studies in Italian population showing point prevalence of 0.563 per 100,000 total population . Unlike HIE, the affliction in CMD is multi-system and it is an inherited disease with variable penetration, the distinction between the two is important to a clinician for the management. CMDs generally have early fatal outcome. So, early diagnosis is important for prognostication, supportive treatment and genetic counselling. The neuro-imaging findings of CMD clearly stand out from the rest aetiologies and can guide a clinician to go in early , for an invasive test like muscle biopsy which is the gold standard diagnostic test .
Atypical carcinoid in the anterior mediastinum is a rare neoplasm. In our case the huge mediastinal carcinoid was treated with surgery using a median sternotomy and clamshell incision because of the extension of the tumor. We describe our difficulties under the surgery and take a look at the literature.
Here, we report a case of hepatic sinusoidal obstruction syndrome(HSOS) of a 30 years old female patient with Ulcerative colitis(UC) who was being treated by mesalazine. The patient was admitted to the emergency unit by harsh and cramping abdominal pain, she had increased AST-ALT-Bilirubine and GGT levels in the blood laboratory analysis. In the Portal venous Doppler US; Splenomegaly and coarse granular appearance in liver parenchymal echogenecity were observed. In the abdominal CT, Splenomegaly was confirmed. In the Dynamic Abdominal MRI, pathognomonic liver finding which was the patchy contrast-enhancing reticular appearance, was visualized and the exact diagnosis was handled by true-cut biopsy. The aid of Dynamic Abdominal MRI to the early diagnosis of HSOS was also demonstrated in this case.
Background: Although multiple reports exist in the literature of patients with 15q11.2 deletion syndrome, the variability of the phenotype has made clinical delineation difficult. Neuro-developmental scores have not been previously reported. We present clinical findings in a group of 16 patients referred to our center for evaluation and management of neurodevelopmental difficulties. Methods: All patients were seen in our center between 2005 and 2016. They were seen by a clinical geneticist and their diagnosis of 15q11.2 deletion syndrome was confirmed by a microarray analysis. Bayley Scales of Infant Development was done and provided a mental developmental index and a motor developmental index. The data collected was then compared to previous reports in the literature of patients with 15q11.2 deletion syndrome. Results: The reviewed group consisted of 10 males and 6 female patients between the ages of 3 and 15 years. The most common clinical findings included developmental delay (94%), hypotonia (88%), ADHD (75%), anxiety (50%), feeding difficulties (44%), autism (31%), dolichocephaly (25%), 2-3 toe syndactyly (19%), seizure activity (19%), and congenital heart disease (13%). Additional findings included prominence of the metopic suture, epicanthal folds, micrognathia, ankle torsion, incontinence, and sleeping difficulties. Our developmental evaluation by the Bayley Scales of Infant Development indicated an average Mental Developmental Index of 75 (NL = >85) and Motor Developmental Index of 75 (NL = >85) in the patients less than 3 years old. Close to 43% of patients had an occipitofrontal circumference (OFC) greater than or equal to the 97th. Conclusion: The data provided here intends to expand the phenotype of the 15q11.2 deletion syndrome. Neurodevelopmental scores have not been previously reported in 15q11.2 del syndrome which were found to be in the mild developmental delay range.