A Rare Cause of Rectovaginal Fistula in Early Infancy: It is in the Genes!

Acquired Rectovaginal Fistula (RVF) is rare in infants. Interleukin10/ Interleukin 10 receptor deficiencies are monogenic disorders presenting as aggressive forms of infantile onset inflammatory bowel disease with perianal abscess and fistula. Genetic studies assist in confirming the diagnosis. We present a two month old infant with rectovaginal fistula, severe colitis, failure to thrive and recurrent infections in whom colonoscopy revealed irregular colonic ulcers, and genetic studies confirmed an IL10RB mutation. Hematopoietic Stem cell transplantation is the definitive therapy for this disorder which the child underwent. We report this infant with an acquired RVF with extraintestinal features due to IL10RB mutation to highlight the importance of thinking beyond the local anatomy and looking into the genetic domain.

Pancreatic Adenocarcinoma With Synchronous Colonic Metastases.

Metastases from pancreatic malignancy are commonly known to occur in the regional lymph nodes, liver, lung, and peritoneum. Synchronous or metachronous metastasis from the pancreas to the colon is rare, with only 6 cases reported in the literature. We report a man who was found to have adenocarcinoma on biopsies from synchronous lesions in the colon and the pancreas. The immunohistochemistry report revealed the diagnosis of a primary pancreatic malignancy with synchronous colonic metastases.

Role of bedside pancreatic scores and C-reactive protein in predicting pancreatic fluid collections and necrosis.

BACKGROUND: Acute pancreatitis is a disease with variable outcome; the course of the disease can be modified by early aggressive management in patients with severe pancreatitis. Easily calculable pancreatic scores and investigations can help to triage these patients. We aimed to determine the role of bedside index for severity in acute pancreatitis (BISAP), harmless acute pancreatitis score (HAPS), and systemic inflammatory response syndrome (SIRS) scores on day of admission and C-reactive protein (CRP) at 48 h for predicting the presence of pancreatic fluid collection (PFC) and necrosis on CT scans done at 72 h. METHODS: Of a total of 114 consecutively seen patients of pancreatitis, 64 with acute pancreatitis were enrolled in the study. All individuals had the pancreatitis predicting scores calculated at the time of admission, CRP at 48 h, and contrast-enhanced computed tomography (CECT) abdomen at 72 h from admission. RESULTS: The study population of 64 (55 male) had a mean (+SD) age of 37.7 ± 13 years. Alcohol was the most common (68.8%) etiology in these patients. Based on CECT, patients were divided into 2 groups; group 1 with 41 patients who had mild pancreatitis and group 2 with 23 patients who had pancreatic fluid collection with or without necrosis (PFCN). PFCN were seen in 19 (29.7%) of patients with 2 or more SIRS criteria, 17 (26.6%) of patients with BISAP score ≥3, and 16 patients (25.0%) with HAPS >0 respectively. All three scores were able to predict PFCN significantly. CRP >150 mg/L was noted in 23 patients and was able to predict the presence of fluid collections (p=0.0002) and pancreatic necrosis (p = 0.0004) on CT. CONCLUSION: BISAP, HAPS, and SIRS scores and CRP of 150 mg/L all correlated significantly with the occurrence of fluid collections and pancreatic necrosis on CT at 72 h. None of the scores was superior to the other in this respect.

Pancreatic ascariasis with periampullary carcinoma.

Ascarias lumbricoides infestation is endemic in tropical countries. Most infections are asymptomatic, but it can produce a wide spectrum of manifestations including hepatobiliary and pancreatic complications. There are reports of association of biliary ascariasis with bilary malignancies in the past, but same is not known for pancreatic ascariasis. We report a case of association of periampullary malignancy with pancreatic ascariasis.

Overlap syndrome: autoimmune hepatitis with primary biliary cirrhosis.

"Overlap syndrome" is used to describe variant forms of autoimmune hepatitis (AIH) which present with characteristics of AIH and primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Patients with overlap syndromes present with both hepatitic and cholestatic serum liver tests and have histological features of AIH and PBC or PSC. AIH-PBC is the most common form of overlap syndrome, affecting almost 10% of adults with AIH or PBC. Transitions from PBC to AIH-PBC overlap syndrome have also been reported. Overlap syndromes show a progressive course without treatment, and therapy is empiric. Ursodeoxycholic acid is usually combined with immunosuppressive therapy but end-stage disease requires liver transplantation. We report a case of AIH/PBC overlap with relevant discussion of literature.

Pancreas divisum and choledochal cyst.

Pancreas divisum (PD) is the most common congenital variant of the pancreas, affecting 5% to 14% of the population. The ventral duct only drains the ventral pancreas through the major papilla, whereas the majority of the pancreas drains via the dorsal duct through the minor papilla. We report the case of a 21-year-old woman with recurrent acute pancreatitis who presented with the rare finding of choledochal cyst and pancreas divisum (PD). She underwent minor papilla sphincterotomy and pancreatic duct stenting. Comparable literature findings of PD and choledochal cyst are discussed with regard to the presented case.

Brain stem auditory evoked responses and visual evoked responses in children with tubercular meningitis.

Thirty two patients between 6 months and 14 years of age with tubercular meningitis were evaluated for brain stem auditory evoked response (BAER) and Visual evoked responses (VER), within 7 days of admission. Absolute latencies and interpeak latencies were compared with values obtained from normal children. BAER abnormality was found in 56.25% and VER in 28%children, respectively. BAER abnormality correlated with Glasgow Coma Scale at admission and discharge, stage of meningitis, raised intracranial pressure, seizure activity, and poor outcome. VER abnormality correlated with abnormal fundus findings only.