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Choledochal cysts: age of presentation,symptoms, and late complications related to Todani's classification
Authors:de Vries J S  de Vries S  Aronson D C  Bosman D K  Rauws E A J  Bosma A  Heij H A  Gouma D J  van Gulik T M
Institution:From the Departments of Surgery, Pediatric Surgery, Pediatric Gastroenterology, Gastroenterology, and Pathology, Academic Medical Center and Vrije Universiteit Medical Center, Amsterdam, The Netherlands.
Abstract:Purpose: The aim of this study was to compare presentation, complications, diagnosis, and treatment of choledochal cysts in pediatric and adult patients. Methods: Forty-two patients were analyzed after subdivision into 3 groups: group A, less than 2 years (n = 10); group B, 2 to 16 years (n = 11); group C, greater than 16 years (n = 21). Results: The cysts were classified as extrahepatic (n = 33), intrahepatic (n = 5), and combined (n = 4). Seventy-six percent of patients presented with abdominal pain, (20 of 21 group C), and 57% with jaundice, (10 of 10 group A). Cholangiocarcinoma occurred in 6 patients, 4 of whom had previously undergone internal drainage procedures. Excision of the extrahepatic cyst was performed in 27 of 37 patients. Five patients, of whom, 4 had cholangiocarcinoma, were beyond curative treatment at the time of diagnosis. Six patients had died at the closure of this study, 5 of them had carcinoma. Conclusions: Presenting symptoms are age dependent with jaundice prevailing in children and abdominal pain in adults. In view of the high risk of cholangiocarcinoma, early resection and not internal drainage is the appropriate treatment of extrahepatic cysts. Patients who had undergone internal drainage in the past still should undergo resection of the cyst.
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