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Intact Choledochal Cyst With Spontaneous Common Hepatic Duct Perforation: A Spectrum of Congenital Biliary Canal Defects?

Kurbet, Santosh B.; Prashanth, Gowda P.; Patil, Vishwanath D.; Wali, Rajashekhar M.

Journal of Pediatric Gastroenterology and Nutrition: January 2015 - Volume 60 - Issue 1 - p e1
doi: 10.1097/MPG.0b013e318287c5b1
Image of the Month

KLE University's Jawaharlal Nehru Medical College, Belgaum, Karnataka, India.

Address correspondence and reprint requests to Gowda P. Prashanth, MD, KLE University's Jawaharlal Nehru Medical College, Belgaum, Karnataka, India (e-mail:

Received 20 November, 2012

Accepted 14 January, 2013

The authors report no conflicts of interest.

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.

A 3-month-old girl admitted for lower respiratory infection developed constipation and abdominal distension 3 days later. On examination, she had mild abdominal distension with absent bowel sounds. Abdominal sonography showed a normal biliary tract and pancreas. Computed tomography of abdomen revealed minimal localized fluid collection in the right upper quadrant. Abdominal paracentesis done 24 hours later revealed bile-stained ascitic fluid. Exploratory laparotomy on the same day revealed intact cystic dilation of common bile duct (type 1). Operative cholangiography revealed leakage of contrast from the distal common hepatic duct with a 4-mm perforation that was sutured securely (Fig. 1). Complete excision of the cyst, cholecystectomy, Roux-en-Y hepatojejunostomy, and jejunojejunostomy were performed (Fig. 2).





Both spontaneous bile duct perforation and common hepatic duct perforation are rare in children (1). The unusual combination of common hepatic duct perforation coexisting with an intact choledochal cyst has not been reported previously. Clinical presentations of spontaneous perforation of bile duct and choledochal cyst are similar. Our case suggests a common underlying pathogenesis, and supports the theory that these 2 separate entities are related to weakness of the biliary canal wall during embryogenesis with pancreaticobiliary malunion (2,3). Pathobiology of spontaneous biliary perforation could be similar to that of choledochal cyst as is evident in the present case.

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1. Evans K, Marsden N, Desai A. Spontaneous perforation of the bile duct in infancy and childhood: a systematic review. J Pediatr Gastroenterol Nutr 2010; 50:677–681.
2. Yamataka A, Kato Y, Mityano T. Biliary tract disorders, and portal hypertension. In: Holcomb GW, Murphy JP, eds. Ashcraft's Pediatric Surgery. 5th ed. Philadelphia: Elsevier Saunders; 2010:566–571.
3. Sai Prasad TR, Chui CH, Low Y, et al. Bile duct perforation in children: is it truly spontaneous? Ann Acad Med Singapore 2006; 35:905–908.
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