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Anomalous Pancreaticobiliary Junction

Arora, Ankur; Mukund, Amar; Thapar, Shalini; Alam, Seema

Journal of Pediatric Gastroenterology and Nutrition: February 2014 - Volume 58 - Issue 2 - p e13
doi: 10.1097/MPG.0b013e31825a2a71
Image of the Month

Institute of Liver and Biliary Sciences, New Delhi, India.

Address correspondence and reprint requests to Ankur Arora, MD, DNB, Institute of Liver and Biliary Sciences, New Delhi 110070, India (e-mail:

Received 15 February, 2012

Accepted 12 April, 2012

Submissions for the Image of the Month should include high-quality TIF endoscopic images of unusual or informative findings. In addition, 1 or 2 other associated photographs, such as radiological or pathological images, can be submitted. A brief description of no more than 200 words should accompany the images. Submissions are to be made online at, and will undergo peer review by members of the NASPGHAN Endoscopy and Procedures Committee, as well as by the Journal.

The authors report no conflicts of interest.

A 3-year-old child with recurrent pancreatitis was diagnosed as having congenital choledochal cyst (type IVA of Todani classification). Three-dimensional magnetic resonance cholangiopancreatography (Fig. 1A and B) showed marked dilatation of the intra- and extrahepatic bile ducts (black arrow) with overdistended gallbladder. The main pancreatic duct (dotted arrow) was also dilated with intraductal filling defects representing stones (thin white arrow). The bile duct was seen joining the pancreatic duct anomalously at an acute angle with a long common channel of approximately 20 mm in length (thick arrow) consistent with anomalous pancreaticobiliary junction (APBJ). A cholangiopancreatogram (Fig. 1C) following intraductal stone removal (with guidewire within the pancreatic duct) confirmed an APBJ.



APBJ refers to union of the pancreatic and bile ducts outside the duodenal wall resulting in a long common channel (usually >15 mm) (1). ABPJ is seen in up to 90% of cases of congenital choledochal cysts and is associated with increased risk of pancreatitis and cholangiocarcinoma presumably secondary to biliopancreatic reflux (1,2). Magnetic resonance cholangiopancreatography with 3 dimensions is considered an easy and accurate imaging method for diagnosing and depicting APBJ, its associated findings, and potential complications (3). Endoscopic retrograde cholangiopancreatography may be helpful for obstructive symptoms, but the definitive therapy is surgical. A timely diagnosis and early surgery can yield a good prognosis with fewer complications. Total resection of the extrahepatic bile duct with hepaticoenterostomy is considered the preferred treatment in children with APBJ with choledochal cyst (4).

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1. Kamisawa T, Takuma K, Anjiki H, et al. Pancreaticobiliary maljunction. Clin Gastroenterol Hepatol 2009; 7 (11 suppl):S84–S88.
2. Funabiki T, Matsubara T, Miyakawa S, et al. Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy. Langenbecks Arch Surg 2009; 394:159–169.
3. Kim MJ, Han SJ, Yoon CS, et al. Using MR cholangiopancreatography to reveal anomalous pancreaticobiliary ductal union in infants and children with choledochal cysts. AJR Am J Roentgenol 2002; 179:209–214.
4. Ono S, Fumino S, Iwai N. Diagnosis and treatment of pancreaticobiliary maljunction in children. Surg Today 2011; 41:601–605.
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