"The Innate Tail": An Unusual Cause of Rectal Bleeding in an Adult Male.

Retrorectal cystic hamartomas or tailgut cysts are infrequent congenital lesions presenting as presacral masses originating from the embryonic hindgut. They are commonly diagnosed in middle-aged women. Physicians must have a high index of suspicion to diagnose this rare tumor. We report a case of retrorectal hamartoma in a 70-year-old male presenting as rectal bleeding instead of the usual compressive symptoms. The utility of imaging modalities and the paramount importance of histopathology has been described. The treatment modality is surgical excision to prevent the potential malignant transformation.

Hamartomas císticos retrorretais ou "tailgut cysts" sao lesóes congénitas pouco frequentes que se originam do intestino posterior embrionário e que se apresentam como massas pré-sagradas. São comumente diagnosticados em mulheres de meia-idade. Os médicos devem ter um elevado grau de suspeição para diagnosticar este tumor raro. Relatamos um caso de um hamartoma retrorretal num homem com 70 anos de idade que se apresentou com retorragias e não com os sintomas mais comuns de obstrução. A utilidade dos exames de imagem e a grande importância da histopatologia foi demonstrada neste caso. A modalidade terapêutica adoptada foi a excisão cirúrgica para prevenir uma transformação maligna potencial.

Minimal Hepatic Encephalopathy in Indians: Psychometric Hepatic Encephalopathy Score and Inhibitory Control Test for Diagnosis and Rifaximin or Lactulose for Its Reversal.

Background and Aims: Psychometric hepatic encephalopathy score (PHES) is used widely for diagnosis of minimal hepatic encephalopathy (MHE). This prospective study aimed to determine the utility of the inhibitory control test (ICT) for the diagnosis of MHE. Additionally, the efficacy of rifaximin and lactulose for reversal of MHE was evaluated. Methods: A total of 180 eligible cirrhotic patients underwent testing for MHE. When PHES was ≤ -5 and ICT lures were ≥ 14, MHE was diagnosed. The 108 patients with MHE were randomized to three groups for treatment with either lactulose, rifaximin, or placebo. Treatment outcomes were measured at the end of 3 months. Results: The 108 patients with MHE diagnosed by PHES and/or ICT accounted for 60%. The diagnosis of MHE was made by both ICT and PHES positivity in 56 patients, by abnormal ICT and normal PHES in 37 patients, and by abnormal PHES and normal ICT in 15 patients. For diagnosis of MHE, ICT had sensitivity of 78.87%, specificity of 66.06% with 60.22% positive predictive value and 82.76% negative predictive value. An area under the curve value of 0.724 (95% CI: 0.653-0.788) was obtained for diagnosis of MHE. Reversal of MHE was seen in 71.42%, 70.27% and 11.11% of patients in the rifaximin, lactulose and placebo arms (p < 0.001). Rifaximin showed better tolerability compared to lactulose. Conclusions: For the diagnosis of MHE, ICT is a simple tool but has lower sensitivity and better specificity than PHES. Rifaximin is as efficacious as lactulose in the treatment of MHE and better tolerated.

Gastroduodenal Tuberculosis: A Case Series and Review of Literature.

Gastroduodenal tuberculosis is infrequently seen in day-to-day clinical practice with few cases reported in the literature. It is usually associated with features of gastric outlet obstruction. This is a case series of 4 patients with 2 of them having associated lower gastrointestinal involvement. One of them resembled a growth in the cardia of the stomach which responded to antitubercular drugs. Another had duodenal erosions with portal lymph node enlargement which responded to antitubercular drug treatment. None of the patients required surgical management. Gastroduodenal tuberculosis should be considered with a high degree of suspicion when patients present with gastric outlet obstruction or with endoscopic evidence of ulceronodular disease in areas endemic for tuberculosis.

Duodenal and Bile Duct Narrowing Due to Partial Annular Pancreas: A Rare Association.

Annular pancreas consists of a ring of pancreatic tissue partially or completely encircling the descending duodenum. It is an unusual congenital anomaly rarely detected in adult life. Gastric outlet obstruction is most common presentation mainly in 2nd or 3rd decades of life. We are reporting an unusual association of partial annular pancreas - a dilated biliary system due to distal common bile duct (CBD) stricture without features of pancreatitis or underlying malignancy which is rare and not reported previously .Our patient also had gastric outflow obstruction. This may be explained by the pancreatic tissue encircling the duodenum at the level where CBD joins the papilla causing dilated CBD and duodenal stenosis.

A Child with Debilitating Pruritus.

We describe a case of two-year-old boy presenting with debilitating pruritus, patchy alopecia and jaundice since the age of 6 months. On evaluation he had intrahepatic cholestasis with persistently raised serum alkaline phosphatase, normal Gamma glutamyl transferase and raised serum bile acid levels. His liver biopsy showed bland cholestasis and electron microscopy showed granular bile suggestive of progressive familial intrahepatic cholestasis type I. Medical therapy with ursodeoxycholic acid, cholestyramine, rifampicin with nutritional modification was successful in alleviating the symptoms and correcting the nutritional status. To our knowledge this is only the sixth case of progressive familial intrahepatic cholestasis type I reported from India. Herein we discuss the diagnostic and therapeutic hurdles that one encounters in managing progressive familial intrahepatic cholestasis and also review the literature regarding this rare disorder.

Symptomatic improvement with gluten restriction in irritable bowel syndrome: a prospective, randomized, double blinded placebo controlled trial.

BACKGROUND/AIMS: The existence of non-celiac gluten sensitivity has been debated. Indeed, the intestinal and extra-intestinal symptoms of many patients with irritable bowel syndrome (IBS) but without celiac disease or wheat allergy have been shown to improve on a gluten-free diet. Therefore, this study set out to evaluate the effects of gluten on IBS symptoms. METHODS: We performed a double-blind randomized placebo-controlled rechallenge trial in a tertiary care hospital with IBS patients who fulfilled the Rome III criteria. Patients with celiac disease and wheat allergy were appropriately excluded. The participants were administered a gluten-free diet for 4 weeks and were asked to complete a symptom-based questionnaire to assess their overall symptoms, abdominal pain, bloating, wind, and tiredness on the visual analog scale (0-100) at the baseline and every week thereafter. The participants who showed improvement were randomly assigned to one of two groups to receive either a placebo (gluten-free breads) or gluten (whole cereal breads) as a rechallenge for the next 4 weeks. RESULTS: In line with the protocol analysis, 60 patients completed the study. The overall symptom score on the visual analog scale was significantly different between the two groups (P<0.05). Moreover, the patients in the gluten intervention group scored significantly higher in terms of abdominal pain, bloating, and tiredness (P<0.05), and their symptoms worsened within 1 week of the rechallenge. CONCLUSIONS: A gluten diet may worsen the symptoms of IBS patients. Therefore, some form of gluten sensitivity other than celiac disease exists in some of them, and patients with IBS may benefit from gluten restrictions.

Most overweight and obese Indian children have nonalcoholic fatty liver disease.

 Background and rationale. Nonalcoholic fatty liver disease (NAFLD) is the most common cause of pediatric liver disease in western countries. Its prevalence in Indian subcontinent is not well studied. MATERIAL AND METHODS: In a school based cross sectional study we have screened overweight and obese children in the age group of 11 to 15 years for NAFLD. Ultrasonography, elevated serum transaminases, fibroscan were used for defining NAFLD. Dietary habits, blood pressure, serum lipid profile, blood counts and insulin resistance were recorded. The relation of fibrosis 4 score, pediatric NAFLD fibrosis index, aspartate transaminases to platelet ratio index (APRI) with fibroscan was evaluated. RESULTS: Out of 616 students screened 198 were overweight and obese. Hundred students and their parents gave informed consent for the further evaluation. The prevalence of NAFLD was 62% in overweight and obese children. Fatty liver was found in 50 % students on ultrasonography, liver stiffness (≥ 6.1 Kilopascals) in 23% and raised alanine transaminase in 30%. Hypertension, dyslipidemia, diabetes mellitus and insulin resistance were seen in 6%, 18%, 2% and 66% students respectively. Systolic hypertension, serum triglyceride, aspartate transaminase, APRI was significantly higher in the NAFLD group. On binary logistic regression only systolic hypertension was an independent risk factor for NAFLD. CONCLUSION: In conclusion NAFLD is common in asymptomatic overweight and obese Indian children. Systolic hypertension is the only independent factor associated with NAFLD. Fibroscan has limited role for screening. We recommend screening for NAFLD in this high risk group with alanine transaminases and ultrasonography.

Non-celiac Gluten Sensitivity (NCGS).

There has been increasing interest in the entity "Non-celiac gluten sensitivity" in recent years which was first of its in 1980s. This "re-discovered" disorder is characterized by intestinal and extra-intestinal symptoms which occur after ingestion of gluten containing food. The number of such patients who neither have celiac disease nor wheat allergy, but appear to benefit from gluten withdrawal is increasing substantially. However it still remains a controversial and its pathogenesis is not well understood. Lack of biomarkers is a major limitation making it difficult to differentiate it from other gluten related disorders. Recent studies have raised the possibility that, beside gluten and wheat amylase-trypsin inhibitors (ATI), low-fermentable, poorly-absorbed, short-chain carbohydrates can contribute to symptoms (at least those related to IBS) experienced by NCGS patients. In this paper we will focus on the manifestations of NCGS and evidence for the condition. Also areas of controversy, major advances and future trends will be discussed.

An Interesting Case of Non-Resolving Hepatogastric Fistula - An Unseen Cause.

Hepatic tuberculosis presents one of the rare forms of extra-pulmonary tuberculosis. It is usually secondary to infection in the lung or the gut. Tuberculous liver abscess is the rarer manifestation even in endemic areas of Mycobacterium tuberculosis. Hepatogastric fistula secondary to tuberculous liver abscess has never been reported in literature. We herein report a case of a disseminated tuberculous liver abscess complicated by hepatogastric fistula, which posed a considerable diagnostic challenge. It was treated successfully with anti tubercular drugs. Liver abscess with atypical features and non responsive to antibiotics should raise the suspicious of tuberculosis or fungal infections.

Primary hepatic amyloidosis: A case report and review of literature.

We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT levels with normal transaminases and bilirubin. On imaging she had diffuse enlargement of liver with heterogeneous contrast uptake in liver. Her viral marker and autoimmune markers were negative. Liver biopsy depicted massive deposition of amyloid in peri-sinusoidal spaces which revealed apple green birefringence on polarizing microscopy after Congo red staining. Cardiac and renal evaluation was unremarkable. Abdominal fat pad and rectum biopsy was negative for amyloid deposit. There was no evidence of primary amyloidosis as bone marrow examination was normal. Serum and urine immunofixation electrophoresis were normal. Immunoperoxidase staining for serum amyloid associated protein for secondary amyloidosis was negative from liver biopsy. We present this rare case of primary hepatic amyloidosis and review the literature regarding varied presentations of hepatic involvement in amyloidosis.

An unusual cause of overt gastrointestinal bleeding in a malnourished child.

Trichuris trichiura (T. Trichiura) is a known parasite infestation among tropical children. Humans acquire Trichuriasis infection after ingestion of embryonated eggs via contaminated food or water. Most of them are asymptomatic. A severe form of trichuris infestation is known as Trichuris dysentery syndrome (TDS). It manifests as mucous diarrhoea, bleeding, malnutrition and stunting, or even life-threatening anaemia. We are reporting an interesting case of a 7-year-old girl who presented with bloody diarrhoea for 2 years. Her growth was stunted as she was not gaining weight. Severe inflammatory bowel disease was suspected clinically. Physical examination revealed marked pallor, pedal oedema and koilonychia along with sparse and hypopigmented hair. On evaluationT. Trichiurahelminths were seen on stool examination and massive worm load was visualised directly on colonoscopy. A diagnosis of TDS was made. She was successfully treated with oral Albendazole (400 mg) and blood transfusion.

Unusual complication of amebic liver abscess: Hepatogastric fistula.

Amebic liver abscess is a parasitic disease which is often encountered in tropical countries. A hepatogastric fistula secondary to an amebic liver abscess is a rare complication of this disease and there are only a handful of reported cases in literature. Here we present a case of an amebic liver abscess which was complicated with the development of a hepatogastric fistula. The patient presented with the Jaundice, pain and distension of abdomen. The Jaundice and pain improved partially after he had an episode of brownish black colored increase in frequency of stools for 5 to 6 d. Patient also had ascites and anemia. He was a chronic alcohol drinker. Esophagogastroduodenoscopy performed in view of the above findings. It showed a fistulous opening with bilious secretions along the lesser curvature of the stomach. On imaging multiple liver abscesses seen including one in sub capsular location. The patient was managed conservatively with antiamebic medications along with proton pump inhibitors. The pigtail drainage of the sub capsular abscess was done. The patient improved significantly. The repeat endoscopy performed after about two months showed reduction in fistula size. A review of the literature shows that hepatogastric fistulas can be managed conservatively with medications and drainage, endoscopically with biliary stenting or with surgical excision.

A Family of Congenital Hepatic Fibrosis and Atypical Retinitis Pigmentosa.

Congenital hepatic fibrosis is a rare cause of portal hypertension and esophageal varices in children. We report cases of siblings with biopsy proven congenital hepatic fibrosis and with atypical retinitis pigmentosa. They presented with repeated episodes of jaundice along with progressive decrease of vision in night. They had hepatosplenomegaly and portal hypertension with esophageal varices. One of the siblings had a large regenerating nodule replacing the entire right lobe of the liver and other one developed repeated hematemesis. This constellation of diagnosis belongs to the ciliopathy group of disorders. The spectrum of ciliopathy disorders has been evolving, and it varies from mild to severe manifestations.

Killian-Jamieson Diverticulum: Cervical Oesophageal Diverticulum.

Killian-Jamieson (K-J) diverticulum is an outpouching from the lateral wall of the proximal cervical oesophagus and is less commonly encountered compared to Zenker's diverticulum (ZD). These diverticulae arise between the fibers of the cricopharyngeus muscle superiorly and longitudinal muscle of the oesophagus inferiorly. In this report we present a case of a symptomatic Killian Jamieson diverticulum and review the clinical presentation, differential diagnosis and radiological findings that distinguish it from the more common Zenker's diverticulum.