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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.

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

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.

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.

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.

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.

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.

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 evaluation T. Trichiura helminths 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.

A 40-year-old male patient presented with distension of abdomen since last 4 years. The distension was progressive and more in upper abdomen. He also developed umbilical hernia since 1 year. There was no jaundice or bleeding. On examination, massive hepatomegaly till right iliac fossa with multiple nodular swellings was felt. The computed tomography of abdomen showed hepatomegaly of 36 cm in size reaching up to pelvis (Fig. 1a). There were multiple variable sized non-enhancing hypodense cystic lesions seen in liver. A 3.4 × 3.5 cm defect was seen in anterior abdominal wall in umbilical region with liver cysts as its content (Fig. 1b). Right kidney was seen in midline in the pelvis facing posteriorly. Both kidneys were enlarged with irregular lobulated contour with cystic lesions (Fig. 1c). The free fluid is seen in abdomen and pelvis. This is a case of polycystic liver and kidney disease with portal hypertension with ascites. Ultrasonography of the family members were normal. The patient was started on salt restricted diet and diuretics. The umbilical hernia was reduced and abdominal binder was applied. The polycystic liver disease occurs as an extra-renal organ involvement in polycystic kidney disease. Rarely its presentation can be isolated. The cystic transformation of liver is secondary to ductal plate malformation and abnormal fluid secretion by cholangiocytes [1]. This can be managed with medications and genetic counseling. The surgical options are fenestration but recurrence is high, segmental hepatic resection if the cysts are localized and liver transplantation with or without kidney transplantation [2].

nodular painless lesions all over his body since 2 months. He had undergone emergency colostomy 1 year previously for intestinal obstruction due to an ulceroinfiltrative mass in the descending colon. Histopathology showed a mucinous adenocarcinoma (signet-ring type). The patient underwent left hemi-colectomy and received 6 sessions of 5-fluorouraciland oxaliplatin-based adjuvant chemotherapy for the tumor (T3N2M0). On physical examination, the patient was pale and had firm-to-hard non-tender nodules of varying sizes over the scalp, face, abdomen, back, and arms (Fig. A, B); the maximum nodule size was 8×8 mm. On microscopic examination, the nodules showed a basket-weave pattern of the stratum corneum, with follicular plugging and increased basal layer melanization. The dermis was infiltrated, showing a narrow green zone and foamy cells with a mucin-like substance (Fig. C, D; alcian blue staining, C: ×40, D: ×10). Imaging did not show any metastatic lesions in the liver or lungs. What is the diagnosis for this lesion?

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.