Anomalous Pancreatobiliary Ductal Union Presenting as Recurrent Acute and Chronic Pancreatitis in Children and Adolescents With Response to Endotherapy.

Introduction Anomalous pancreaticobiliary duct union (APBDU) is defined by the abnormal position of the junctional union of the common bile duct and the pancreatic duct, outside the duodenal wall above the influence of sphincter of Oddi, associated with choledochal cysts and biliary malignancies. APBDU may rarely present as recurrent acute pancreatitis (RAP) or chronic pancreatitis (CP). We aimed to study the prevalence of patients with APBDU presenting as RAP or CP and their response to endotherapy. Methods A retrospective audit of the prospectively maintained endoscopy database at our institute between January 2018 and November 2020 was conducted to identify cases of APBDU presenting as RAP or CP. Details of investigations, endoscopic retrograde cholangiopancreatography (ERCP) findings, and follow-up till six months were noted. Results We identified 26 cases of APBDU, of which five (19.2%) cases presented as RAP or CP. Of these five patients, two had RAP, while three presented with CP (median: 11 years; range: 4-25 years). Magnetic resonance cholangiopancreatography (MRCP) showed APBDU in three patients. One patient with RAP had a Komi type IIIB anomaly. Another patient with RAP had a rare anomaly with absent ventral PD, with the bile duct communicating and draining through the dorsal duct. Two patients with CP had a long common channel with Komi IIA anomaly. One patient with CP had IIIC2 anomaly. Pancreas divisum was noted in three patients, all of whom underwent minor-papilla sphincterotomy. Successful pancreatic stent placement was performed in all patients. Over one year of follow-up, patients with CP had a significant decrease in pain as measured by the visual analog scale. Those with RAP had no further episodes of pancreatitis. Conclusion APBDU is a rare cause of RAP and CP in young patients, occasionally missed on MRCP. RAP and CP caused by APBDU show good response to endotherapy.

Outcomes and predictors of response to endotherapy in pancreatic ductal disruptions with refractory internal and high-output external fistulae.

Backgrounds/Aims: Endoscopic retrograde cholangiopancreatography (ERCP) remains the primary treatment for a subset of patients with pancreatic fistulae. The objective of this study was reporting outcomes of ERCP and predictors of resolution in patients with pancreatic fistulae refractory to conservative therapy. Methods: Retrospective review of patients who underwent ERCP and pancreatic stent placement for pancreatic fistula not responding to medical therapy was performed. Clinical features, laboratory parameters, radiological features and pancreatogram findings were noted. Clinical resolution of fistula was the primary outcome measure. Results: Sixty-eight patients underwent ERCP for high-output pancreatic fistula (Mean age 34.1 years, 91.1% males, 35/68 chronic pancreatitis, 52.9% alcohol etiology). Internal fistulae (pancreatic ascites, pleural effusion, or pericardial effusion) were seen in 55 (80.9%) patients and external fistula in 13 (19.1%) patients. Technical success for ERCP was 92.6% (63/68). Leak was seen in 98.4% (62/63). The most common leak site was body (69.8%). Multiple leak sites were seen in 23.1%. Pancreatic stricture was found in 36.5%. In 44 (69.4%) patients, stent was placed beyond the site of the leak. Resolution at six weeks was achieved in 76.4% (52/68). On univariate and multivariate analyses, placement of stent beyond site of leak was significantly associated with resolution of high-output fistulae (3/41 [7.3%] vs. 5/19 [26.3%], p = 0.03; odds ratio: 6.5, 95% confidence interval: 1.211-34.94). Conclusions: In our experience, ERCP was successful in 76% of patients with pancreatic fistulae refractory to conservative therapy. Stent placement beyond the site of leak was associated with higher resolution of fistulae.

Normal Values of High-resolution Anorectal Manometry of Healthy Indians.

Background/Aims: High-resolution anorectal manometry (HRAM) measures anal sphincter function and anorectal co-ordination. This study aims to provide normal data for HRAM and evaluate the effect of gender, age, and body mass index (BMI) on anorectal functions in healthy Indian subjects. Methods: HRAM was performed on 93 healthy volunteers using a 20-channel, water-perfused catheter. We evaluated anorectal pressures, rectal sensation, and balloon expulsion time. Measurements were recorded during rest, squeeze, and simulated defecation (push). Results: Median anal resting pressure (88 mmHg vs 94 mmHg, P = NS), anal squeeze pressure (165 mmHg vs 147 mmHg, P = NS) were not significantly different between males and females. Rectal pressure (70 mmHg vs 54 mmHg, P = 0.024) and anal pressure (82 mmHg vs 63 mmHg, P = 0.008) during simulated evacuation without rectal distention, were higher in males. The threshold for the first sensation was lower in females (40 mL vs 30 mL, P = 0.021) but desire to defecate (105 mL vs 90 mL, P = NS) and maximum tolerable volume (160 mL vs 140 mL, P = NS) were not significantly different in males and females. Anal residual pressure (median mmHg 83 vs 71 mmHg, P = 0.025) was higher in subjects < 40 years of age. Maximum anal squeeze pressure (185 mmHg vs 165 mmHg, P = 0.024) and maximum rectal pressure (75 mmHg vs 62 mmHg, P = 0.032) during push higher in BMI < 23 kg/m2. Conclusions: The present study provides normal data for the Indian population that can be used for comparison and further work. Age, gender, and BMI affect anorectal parameters in HRAM and should be considered while reporting.

Gonadal dysfunction in male patients with Budd Chiari syndrome and its reversibility with treatment.

BACKGROUND AND AIMS: Budd Chiari syndrome (BCS) commonly affects adolescents and adults. With improved survival, important quality-of-life parameters such as sexual life and fertility become more relevant. This study was aimed to assess the gonadal function in male patients with BCS and the effect of treatment on gonadal function. METHODS: Thirty male patients with newly diagnosed BCS were prospectively assessed for the presence of gonadal dysfunction. Erectile function was assessed using standardized International Index of Erectile Function questionnaire (IIEF). Follicular stimulating hormone (FSH), luteinizing hormone (LH), sex hormone-binding globulin (SHBG), estradiol, total testosterone (TT), calculated free testosterone (cFT), calculated bioavailable testosterone (cBT), sperm count, and sperm motility were compared at baseline and at 6 months of treatment for the assessment of gonadal function. RESULTS: Sixteen (53.3%) out of 30 patients were sexually active at the time of study and 5/16 (31%) had erectile dysfunction. Hypogonadotropic hypogonadism (HH) was the most common pattern seen in 50% cases followed by hypergonadotropic hypogonadism (HyH) in 23% cases. 27% patients had eugonadism. At 6 months of treatment, 60% of patients in HH group became eugonadal as compared to only 14% in HyH group. Proportion of patients with erectile dysfunction reduced (5/16 vs 1/16) after 6 months of therapy. The improvement in sperm count and sperm motility was not significant. CONCLUSION: Gonadal dysfunction is common in male patients with BCS. HH remains the most common type of hypogonadism BCS and the type which improves significantly after treatment.