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- W2092189074 abstract "INTRODUCTION Congenital duodenal atresia is frequently associated with other anomalies such as trisomy 21, annular pancreas and congenital heart disease (1-4). Biliary and pancreatic tract anomalies, such as biliary atresia, a dual pancreatic duct or anomalous bile ducts communicating between the proximal and distal ends of the duodenum, have infrequently been observed in patients with duodenal atresia (4,5). However, whether there is an association between duodenal atresia and choledochal cyst is still controversial. To our knowledge, only four such cases have been documented. This report describes the fifth case with a review of the literature to emphasize that such an unusual coexistence of the two anomalies may, at least in part, relate to the development of an anomalous pancreaticobiliary ductal junction (APBDJ). CASE REPORT A female baby born by Caesarean section at 41 weeks of gestation weighed 2,450 grams. The pregnancy was complicated by polyhydramnios. The results of prenatal sonography suggested the presence of proximal gastrointestinal obstruction. After birth, she was admitted to the pediatric intensive care unit and an abdominal radiograph showed a “double-bubble” sign, compatible with the diagnosis of duodenal atresia (Fig. 1). She underwent duodenoduodenostomy for correction of type III duodenal atresia associated with an annular pancreas on the third day after birth. She recovered uneventfully after the operation and did well for 7 years.FIG. 1: Plain abdominal radiograph taken immediately after birth demonstrates the classic “double-bubble sign” consistent with the diagnosis of duodenal obstruction.At 7 years of age, she was admitted to evaluate intermittent moderate to severe abdominal pain lasting for 1 week. Physical examination revealed tender epigastrium without palpable mass and jaundice. Laboratory data showed abnormal liver and pancreatic function tests with elevated serum levels of aspartate aminotransferase (130 U/L; normal range, 0-37 U/L), alanine aminotransferase (60 U/L; normal range, 1-40 U/L), direct/total bilirubin (0.42/1.2 mg%; normal range, 0-0.4/0.2-1.4 mg%), amylase (242 U/L; normal range, 27-137 U/L) and lipase (246 U/L; normal range, <190 U/L). Abdominal sonography revealed a dilated biliary tree. A tentative diagnosis of choledochal cyst was made. Subsequent computerized tomography and magnetic resonance cholangiopancreatography confirmed the diagnosis. During surgery, a huge and elongated gallbladder and a dilated common bile duct were found (Fig. 2). Intraoperative cholangiography demonstrated an Alonson Lej type IV choledochal cyst with APBDJ and a long common channel distal to the junction of pancreatic duct and common bile duct (Fig. 3). Excision of the choledochal cyst and the long tortuous gallbladder and hepaticoduodenostomy (Fig. 4) were performed. The patient recovered well after the second operation.FIG. 2: A, a photograph taken during operation shows a huge and elongated gall bladder (GB) and a dilated common bile duct (CBD) connected by a cystic duct (CD). The biliary dilatation is extended into intrahepatic bile ducts demonstrated by intraoperative cholangiography. B, schematic presentation of the photograph.FIG. 3: Intraoperative cholangiography shows Alonson Lej type IV choledochal cyst (CC): both extra-hepatic and intra-hepatic bile are dilated diffusely. A long common channel (arrowhead) distal to the junction of pancreatic duct (PD, arrows) and the common bile duct is demonstrated. D, duodenum; GB, gall bladder; IHD, intrahepatic duct; PD, pancreatic duct.FIG. 4: Schematic presentation of reconstructive procedure: the choledochal cyst is dissected at the level of the proximal end of common hepatic duct (CHD) and most of the choledochal cyst is excised. An end-to-side hepaticoduodenostomy was performed between CHD and the dilated first portion of duodenum (D1).DISCUSSION The association of duodenal atresia or stenosis and choledochal cyst was first described by Bailey et al. in 1993 who found one case among 128 patients with intrinsic and extrinsic duodenal obstruction (3). The anatomic details of this case were not clearly depicted. Grosfeld and Rescorla reported another possible case (4,5), in whom duodenojejunostomy was performed for duodenal obstruction (either atresia or stenosis) with annular pancreas after birth. Choledochal cyst was detected 13 years later after repeated episodes of pancreatitis. This patient underwent a second operation to correct choledochal cyst with good recovery. Okada et al. (6) reported the first two definite cases of duodenal atresia associated with choledochal cyst in 1993 in Japan. In the first case, a male infant, the duodenal atresia was associated with an annular pancreas. Twelve years after diamond-shaped duodenoduodenostomy for duodenal atresia, he presented with abdominal pain. A type IV choledochal cyst with common bile duct stone was demonstrated by computerized tomography and corrected by Roux-Y-choledochojejunostomy. Intraoperative cholangiography revealed APBDJ with a long common channel. The second patient was a female infant, who underwent the same procedure for duodenal atresia and annular pancreas. Three years later, she presented with abdominal pain. Computerized tomography also showed a type IV choledochal cyst. Roux-Y-choledochojejunostomy was performed and intraoperative cholangiography confirmed the presence of APBDJ and a long common channel. Komuro et al. (7) reported the third case in 2000. This was a male infant with multiple anomalies, including type III duodenal atresia without annular pancreas, imperforate anus, cardiac anomalies, tracheobronchial stenosis and brain atrophy. In contrast to the two previously reported cases, choledochal cyst was found during surgery but was left untreated during surgical correction of duodenal atresia. Seven months later, he underwent choledochoduodenostomy for cholangitis. APBDJ was found on the intraoperative cholangiogram. Unfortunately, this patient died of respiratory failure caused by tracheobronchial stenosis. In 2002, Sugimoto et al. (8) described the fourth case, a female infant who was initially treated with duodenoduodenostomy for type III duodenal atresia associated with an annular pancreas. She was readmitted for abdominal pain 8 months later. Abdominal sonography revealed a choledochal cyst. A long common channel was demonstrated on the intraoperative cholangiogram and the patient underwent hepaticoduodenostomy with uneventful recovery. In our patient, a type III duodenal atresia with an annular pancreas at birth was later associated with APBDJ with a long common channel. Taken together, three of five cases were type III duodenal atresia. Excluding Case 3, the other four cases were associated with an annular pancreas. APBDJ was demonstrated on the intraoperative cholangiogram in all five cases (Table 1).TABLE 1: Summary of five cases with concomitant duodenal atresia and choledochal cystThe association of duodenal atresia with choledochal cyst is not by chance alone. In addition to a more common association of annular pancreas, duodenal atresia may be associated with other anomalies of the pancreatic and biliary ducts, including anomalous bile duct communicating between the proximal and distal ends of the duodenum (9). Because duodenal atresia most commonly involves the papilla of Vater and the common duct is frequently opened to the intraluminal web, Boyden et al. (10) hypothesized that local “traffic jam” induced by the entry of the hepaticopancreatic and accessory pancreatic ducts might hinder recanalization of the duodenum at this point. This postulation was supported by observations from Gourevitch (11) suggesting that the band of tissue joining the proximal and distal duodenum frequently represents a biliary canal rather than an atretic duodenum. Accordingly, errors in embryological development may lead to duodenal atresia with annular pancreas and APBDJ and APBDJ leading to choledochal cyst (12-13). The observation that choledochal cyst with APBDJ may occur as a result of sinistral dislocation of the ventral pancreas may also help to explain the simultaneous development of type III duodenal atresia and choledochal cyst in three of the five cases. (14-15)." @default.
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- W2092189074 title "Is there an Association Between Duodenal Atresia and Choledochal Cyst?" @default.
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