Severe reflux esophagitis and multiple congenital defects: A case report.

INTRODUCTION: Gastroesophageal reflux disease is a common and troublesome condition. This paper reports a rare case of gastroesophageal reflux disease caused by ectopic biliary drainage accompanying the absence of a pyloric channel and duodenal bulb in a female patient with multiple underlying malformations. PATIENT CONCERNS: A 24-year-old female presented with acid regurgitation and abdominal pain for one month. She was born two weeks premature and with blindness of the right eye. Cardiac murmur was detected in the physical examination. DIAGNOSIS: Gastroendoscopy was performed, and a class D reflux esophagitis and ectopic papilla complicated with the absence of a pyloric channel and duodenal bulb were found. Doppler echocardiography further confirmed the defects of atrial and ventricular septa. Trio-based whole exome sequencing was performed on the proband and her family to find the potential association of multiple variations. However, no putative pathogenic mutations were found. INTERVENTIONS: The patient received proton pump inhibitors and prokinetic treatment and underwent surgical repair of septal defects. OUTCOMES: The symptoms were quickly relieved, and the patient was kept stable upon follow-up. CONCLUSION: The combination of an absent pylorus and ectopic papilla is a rare cause of reflux esophagitis. Unusual gastrointestinal anatomical variations may be accompanied by other malformations. Though no remarkable mutation were detected in this case, sequencing is an efficient technique worth full consideration.

Pancreatobiliary maljunction: association with gallbladder cancer

Background: Pancreatobiliary maljunction is a rare disease characterized by the junction of the pancreatic and biliary ducts outside of the duodenal wall, which normally results in a large common duct. As a result, there is a greater risk of acute pancreatitis and cancer of the gallbladder and biliary tract. Case report: We present the case of a 43-year-old female diagnosed with a pancreatobiliary maljunction and an associated stenosis of the bile duct, secondary to an episode of acute pancreatitis. She underwent several endoscopic retrograde cholangiopancreatography procedures over the course of three years, without improvement of the stenosis, and therefore a surgical approach was taken. Prior to the surgical intervention, magnetic resonance imaging showed the presence of an 11-mm polyp in the gallbladder. A histological study of the surgical sample identified intramucosal adenocarcinoma over a tubular adenoma of the gallbladder. Discussion: Pancreatobiliary maljunction can be considered as a premalignant entity due to the risk of developing cancer of the biliary tree and gallbladder. Therefore, these patients should undergo a prophylactic intervention, despite being asymptomatic

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Ectopic papilla of Vater in duodenum bulb: A hospital-based study.

The major papilla of Vater can be ectopically present in the stomach, pyloric canal, duodenal bulb, and third or fourth portion of the duodenum. In this study, we determined the clinical significance of ectopic papilla of Vater by endoscopic retrograde cholangiopancreatogram (ERCP).A retrospective study was conducted by reviewing the medical records of 6133 patients receiving ERCP from 1988 to 2011. The diagnosis was confirmed if both the common bile duct (CBD) and the main pancreatic duct (PD) drained into the same opening, either by ERCP or magnetic resonance cholangiopancreatography.Eight patients with major papilla of Vater in the duodenal bulb were identified among 6133 patients receiving ERCP from 1988 to 2011, with an incidence rate of 0.13%. The mean age was 67 years and patients were predominantly male. Duodenal bulb deformity was noted in all patients and three of them had shallow gastric and/or duodenal ulcers. Hook-shaped CBD configuration was seen only in half of our cases. Three patients with CBD stones were treated successfully after endoscopic sphincterotomy or papillary balloon dilation.Ectopic orifice of papilla is a rare finding of ERCP. Opacification of both the CBD and main PD from the same opening is an essential criterion for diagnosing an ectopic papilla of Vater in the duodenal bulb.

Quiste de colédoco mixto (tipo I y II) asociado a malformación de la unión pancreatobiliar. Descripción de un caso y revisión de la literatura / Mixed choledochal cyst (type I and II) associated with a malformation of the pancreatobiliary junction. A case report and review of the literatura

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A study on the human pancreaticobiliary duct system and ampulla region with their clinical considerations.

INTRODUCTION: The purpose of this study is to present the variations of the pancreatic duct system and hepatopancreatic ampulla so that an awareness of these variations may help in surgical planning and prevention of ductal injury. METHODS: Forty human pancreatic specimens were used to study the duct pattern of the pancreas and the formation and mode of termination of the pancreatic ducts. Also, the patency of the main pancreatic ducts, length of the hepatopancreatic ampulla, and length of the main pancreatic duct were measured. RESULTS: The main pancreatic duct was patent in 37 out of 40 specimens, 2 specimens showed the embryonic type of the duct system, and one had the duct obliterated at its duodenal end. The mode of termination of the main pancreatic and common pancreatic ducts was divided into four types. Seventy-five percent specimens belonged to type I, followed by type II in 22.5 % and type III in 2.5 % of specimens. The length of the main pancreatic duct ranged from 11 to 21.2 cm, the average being 17.3 cm, and the length of the hepatopancreatic ampulla varied from 1 to 10 mm (1 to 2 mm-5 %, 3 to 8 mm-55 %, and more than 8 mm in 15 % of specimens), and it was absent in 25 %. CONCLUSION: Awareness of these anomalies may help in surgical planning and prevention of undue ductal injury.

Periampullary duodenal duplication cyst masquerading as a choledochocele.

Enteric duplication cysts are rare congenital anomalies of unclear etiology. While they can occur anywhere in the gastrointestinal tract, they typically occur in the ileum or ileocecal region and very rarely in the duodenum. Here, we report a case of a periampullary duodenal duplication cyst in a 13-year-old male who presented with clinical and laboratory evidence of small bowel obstruction, hepatitis, and pancreatitis. Based on radiologic imaging, the patient was thought to have a type III choledochal cyst (choledochocele) within the duodenal lumen. Intraoperative findings and postoperative pathological evaluation, however, revealed that the lesion was a duodenal duplication cyst masquerading as a choledochal cyst. Interestingly, the duplication cyst was communicating with the common bile duct, simultaneously causing biliary and small bowel obstruction.

Surgery or endoscopy to treat duodenal duplications in children.

BACKGROUND/PURPOSE: Gastrointestinal duplications (duodenal duplications [DDs]) are a rare congenital malformation generally located in or adjacent to the medial border of the duodenal wall. The goal of therapy is surgical excision. Conservative endoscopic management represents an alternative option. AIM: The aim of the study was to highlight the role of endoscopic ultrasound (EUS) in guiding the endoscopic or surgical treatment of DD. METHODS: Between 2002 and 2010, 6 patients (2 male; mean age, 7.83 years; range, 2-18 years), all with recurrent acute pancreatitis, were diagnosed with DD by ultrasound and magnetic resonance imaging. Endoscopy was always performed together with EUS (Olympus UM-3R 20-MHz radial miniprobe, Tokyo, Japan). An endoscopic section of the common duodenal-DD wall, using a precut needle or sphincterotome, was chosen by EUS when the biliary tree was not involved in the DD. Otherwise, surgery with duodenotomy and complete opening of the common wall was used. RESULTS: After EUS evaluation, endoscopic treatment was successfully performed in 4 patients, 2 of whom required surgical treatment. Bleeding occurred in 1 patient after endoscopic resection and in 1 patient after surgery. The mean follow-up time without pathologic signs was 3.3 years (range, 0.25-8). CONCLUSIONS: Endoscopic ultrasound can effectively guide surgical or endoscopic therapies. Bleeding is a possible complication.

A rare case of periampullary carcinoma with ectopic ending of Vater's ampulla.

A 71-year-old woman was referred to our department complaining of painless progressive jaundice for the last 3 mo. Magnetic resonance imaging and magnetic resonance cholangiopancreatography (MRCP) showed the ectopic hepatopancreatic ampulla draining into the fourth part of the duodenum adjacent to the duodenojejunal flexure; the irregular morphology of the duodenojejunal flexure likely due to a soft tissue mass. Laparotomy confirmed the presence of the abnormal ampulla of Vater located at the fourth part of the duodenum and a soft tissue tumor about 6 cm x 5 cm x 5 cm with a peduncle adjoining the ampulla. Resection of the tumor, including some peripheral tissue, and a Roux-Y loop anastomosis choledochojejunostomy were performed. Pathological examination indicated an intestinal villous adenoma accompanied by severe dysplasia and focal canceration. Periampullary carcinoma with ectopic ending of the Vater's ampulla into the fourth part of the duodenum is rather rare. The embryonic genetic background of this anomaly has not yet been fully explained. It is worth mentioning that MRCP is useful for demonstrating anomalies and anatomic variants of the biliary tract system and pancreatic duct.