A case of biliary atresia with pancreaticobiliary maljunction

نویسندگان

  • Kosuke Endo
  • Akiko Yokoi
  • Yasuhiko Mishima
  • Akihiko Tamaki
  • Keiichi Morita
  • Yuichi Okata
  • Chieko Hisamatsu
  • Hiroaki Fukuzawa
  • Makiko Yoshida
  • Yoshinobu Akasaka
  • Kosaku Maeda
چکیده

BACKGROUND The pathogenesis of biliary atresia (BA) is still unknown. There are several reports on the etiology of BA, including pancreaticobiliary maljunction (PBM). We experienced a case of Kasai type IIIa BA with PBM, in which we found elevation of pancreatic enzymes in the gallbladder. We evaluated whether PBM is related to the pathogenesis of BA based on our findings. CASE PRESENTATION The patient was born at 40 weeks of gestation. His body weight at birth was 2850 g. At the age of 4 days, he had an acholic stool and was referred to our hospital. Abdominal ultrasonography showed that triangular cord sign was negative. The gallbladder was isolated with a diameter of 19 mm, and it contracted in response to oral feeding. His ultrasonographic findings were atypical for BA, but his jaundice did not improve. Therefore, we performed an operation at the age of 56 days. Intraoperative cholangiography showed a common bile duct and pancreatic duct and a common channel patent, while the common hepatic duct or intrahepatic duct was not visualized. Bile in the gallbladder contained colorless fluid, which showed elevated lipase level (34,100 IU/L). We performed Kasai portoenterostomy under the diagnosis of Kasai type IIIa BA with PBM. The patient's postoperative course was uneventful, and he was discharged on day 30 after the operation. Histopathological evaluation showed that the lumens of the common bile duct and cystic duct were patent. However, the common hepatic duct was closed, and only bile ductules with diameters of less than 50 μm were isolated. Infiltration of lymphocytes was detected in the porta hepatis. No apparent inflammation was observed around the cystic duct, which was constantly exposed to pancreatic juice because of reflux through PBM. CONCLUSIONS Reflux of pancreatic juice through PBM might not be an etiological factor for BA, but might be associated with patency of the common and cystic bile ducts in Kasai type IIIa BA.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Pitfalls of MRCP in the diagnosis of pancreaticobiliary maljunction.

CONTEXT Magnetic resonance cholangiopancreatography (MRCP) is useful for examining the pancreatic duct system in patients with acute pancreatitis instead of using endoscopic retrograde cholangiopancreatography (ERCP), as ERCP-induced pancreatitis represents a serious problem. However, we present here a case of idiopathic acute pancreatitis in which MRCP suggested pancreaticobiliary maljunction,...

متن کامل

Inflammatory Polyp in the Common Bile Duct with Pancreaticobiliary Maljunction

A 63-year-old woman was admitted because of epigastric pain and obstructive jaundice. Endoscopic retrograde cholangiopancreatography and intraductal ultrasonography revealed a 25-mm papillary nodule in the middle to inferior portion of the common bile duct (CBD). Pancreaticobiliary maljunction (PBM) was also identified. Contrast-enhanced computed tomography also showed an enhanced nodule in the...

متن کامل

Synchronous primary gallbladder and pancreatic cancer associated with congenital biliary dilatation and pancreaticobiliary maljunction

INTRODUCTION Synchronous double cancer of the gallbladder and pancreas that is associated with congenital biliary dilatation (CBD) and pancreaticobiliary maljunction (PBM) is extremely rare. PBM is frequently reported in Asia, particularly in Japan. We report a surgical case of synchronous double cancer in a patient with primary gallbladder and pancreatic cancer. PRESENTATION OF CASE A 72-yea...

متن کامل

Hilar cholangiocarcinoma accompanied by pancreaticobiliary maljunction without bile duct dilatation 20 years after cholecystectomy: report of a case.

Pancreaticobiliary maljunction (PBM) is associated with the occurrence of biliary cancer due to pancreatobiliary reflux. From the perspective of carcinogenesis, the treatment for PBM is controversial. We herein report a case of hilar cholangiocarcinoma 20 years after the occurrence of gallbladder cancer. A 75-year-old man was referred to our hospital regarding an obstructive jaundice and bile d...

متن کامل

Annular pancreas concurrent with pancreaticobiliary maljunction presented with symptoms until adult age: case report with comparative data on pediatric cases

BACKGROUND Annular pancreas (AP) concurrent with pancreaticobiliary maljunction (PBMJ), an unusual coexisted congenital anomaly, often presented symptoms and subjected surgical treatment at the early age of life. We reported the first adult case of concurrent AP with PBMJ presented with symptoms until his twenties, and performed a literature review to analyze the clinicopathological features of...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 3  شماره 

صفحات  -

تاریخ انتشار 2017