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      Choledochal cyst: A review of 79 cases and the role of hepaticodochoduodenostomy

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          To analyze our experience of choledochal cysts. The results of complete excision of cyst and hepaticodochoduodenostomy have been analyzed in particular.

          Material and Methods:

          Period of study: January 1993 to August 2010. Apart from basic investigations, we did ultrasonography (USG) of abdomen, contrast-enhanced computerized axial tomography scan (CECT), endoscopic retrograde cholangiopancreatography (ERCP), operative cholangiogram, hepatic isotope scan (HIDA), magnetic resonance cholangiopancreatogram (MRCP) and choledochoscopy. The patients were divided into six groups according to the management done and the results analyzed.


          The total number of cases was 79 (Male : Female = 26 : 53). The mean age of presentationwas 5.18 years. The most common mode of presentation was abdominal pain in 86% cases. Types of cysts - Type I: 63; Type IV: 12; Forme fruste: 1; Caroli's disease: 1; Cyst with atresia in the lower end of common bile duct: 2. Two patients of group A (cyst excision and Roux-en-Y hepaticojejunostomy) had recurrent pain and cholangitis. One patient required revision for stricture. In group E ( n=53) (excision of cyst and hepaticodochoduodenostomy), three patients developed occasional epigastric pain and they responded to omeprazole. One patient developed anastomotic stricture and was lost to follow up. One patient of cyst with biliary atresia with biliary cirrhosis died.


          In choledochal cyst, complete excision of cyst and good bilioenteric anastomosis with wide stoma should be done. Hepaticodochoduodenostomy with wide stoma is a simple, quick procedure with preservation of normal anatomy and physiology and minimum complications. It also avoids multiple intestinal anastomoses and so should be the preferred approach.

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          Most cited references 18

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          Surgical treatment of choledochal cysts.

           H Pitt,  P A Lipsett (2002)
          Biliary cystic disease is uncommon in Asia and very rare in Europe and the Americas. Patients with biliary cysts may present as infants, children, or adults. When patients present as adults, they are more likely to have stones in the gallbladder, common duct, or intrahepatic ducts and to present with biliary colic, acute cholecystitis, cholangitis, or gallstone pancreatitis. With increasing age at presentation, the risks of intrahepatic strictures and stones, segmented hepatic atrophy/hypertrophy, secondary biliary cirrhosis, portal hypertension, and biliary malignancy all increase significantly. Factors to be considered when performing surgery on patients with biliary cystic disease include: (1) age, (2) presenting symptoms, (3) cyst type, (4) associated biliary stones, (5) prior biliary surgery, (6) intrahepatic strictures, (7) hepatic atrophy/hypertrophy, (8) biliary cirrhosis, (9) portal hypertension, and (10) associated biliary malignancy. In general, regardless of age, presenting symptoms, biliary stones, prior surgery or other secondary problems, surgery should include cholecystectomy and excision of extrahepatic cyst(s). With respect to the distal bile duct, the surgical principle should be excision of a portion of the intrapancreatic bile duct with care to not injure the pancreatic duct or a long common channel. Resection of the pancreatic head should be reserved for patients with an established malignancy. With respect to the intrahepatic ducts, surgery should be individualized depending on whether (1) both lobes are involved, (2) strictures and stones are present, (3) cirrhosis has developed, or (4) an associated malignancy is localized or metastatic. When the liver is not cirrhotic, hepatic parenchyma should be preserved even when strictures and stones are present. If cirrhosis is advanced, hepatic transplantation may be indicated, but this sequence of events is unusual. If a malignancy has developed, oncologic principles should be followed. Whenever possible, resection of a localized tumor including adjacent hepatic parenchyma and regional lymph nodes should be performed.
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            Influence of age on the presentation and outcome of choledochal cyst.

            The aim of this study was to identify the most appropriate timing for surgery in newborns with choledochal cysts. The clinical and histological data of 8 newborn cases of choledochal cysts (newborn group) were compared with 45 cases that were diagnosed later than the newborn period (late group). The mean and standard deviation of age at diagnosis was 0.4+/-0.4 months and 43.4+/-37.8 months in the newborn and late group, respectively. The age at operation was significantly earlier in the newborn group than in the late group (4.9+/-4.5 months v54.7+/-47.0 months). Although no significant difference in the time that elapsed between the operation and the onset of symptoms between the newborn and late groups (4.5+/-4.7 months v11.3+/-21.1 months), the serum bilirubin level (4.6+/-3.8 mg/dL v1.8+/-3.4 mg/dL) and the grade of liver fibrosis (2.0+/-0.8 v1.1+/-0.8) were significantly higher in the newborn group than in the late group. The newborn group should be considered as a special group of patients with different clinical course and pathology than those of the late group, which necessitate the early surgical intervention to prevent progression of liver fibrosis.
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              Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults.

              The aim of this study was to review the cases 200 children and 40 adults who had cyst excision combined with hepaticoenterostomy (CEHE) for choledochal cyst, with particular emphasis on post-CEHE complications and their surgical management. Patients who had CEHE at the age of 15 years or less were defined as children, and those aged 16 years or older were defined as adults. The mean age when patients became initially symptomatic was 3 years in children and 26 years in adults. Eleven adults became symptomatic as children (< or = 15 years of age). The mean age of CEHE in children and adults was 4.2 years and 35 years, respectively. The time interval between the onset of initial symptoms and CEHE was significantly less in children than in adults (P < .0001). Of the 200 children, 176 had primary CEHE, and 24 had secondary CEHE converted from cystoenterostomy or other biliary surgery. Seventy children had intraoperative cyst endoscopy, which enabled us to examine the proximal intrahepatic bile ducts for stenosis and debris, and to wash out debris, protein plugs, and stones from the intrapancreatic ducts. Of the 40 adults, 22 had primary CEHE, 18 had secondary CEHE. The mean follow-up period was 10.9 years in children and 10.7 years in adults. The number of patients with post-CEHE complications in children and adults was 18 (9.0%) and 17 (42.5%), respectively. The post-CEHE complication rate in children was significantly lower than in adults (P < .0001). The 18 children had 25 post-CEHE complications such as cholangitis, intrahepatic bile duct stones, pancreatitis, stone formation in the intrapancreatic terminal choledochus or pancreatic duct, and bowel obstruction. Twenty-seven post-CEHE complications developed in the 17 adults including 2 cases of cholangiocarcinoma. There were no post-CEHE complications in the 70 children who had intraoperative cyst endoscopy. No stone formation was seen in the 145 children who had CEHE at the age of 5 years or less. Eight stone formations were seen in seven (12.7%) of the remaining 55 children aged over 5 years. Stones developed in seven (17.5%) adults. The incidence of post-CEHE stone formation in children aged 5 years or less was significantly lower than in other children and adults (P < .0001). Reoperation was required in 15 children: revision of hepaticoenterostomy in 4, percutaneous transhepatic cholangioscopic lithotomy (PTCSL) in 1, excision of intrapancreatic terminal choledochus in 2, endoscopic sphincterotomy of the papilla of Vater in 1, pancreaticojejunostomy in 1, and laparotomy for bowel obstruction in 6. Ten adults required reoperations: revision of hepaticoenterostomy in 2, PTCSL in 2, left hepatic lobectomy in 1, endoscopic sphincterotomy in 2, exploratory laparotomy in 2, and adhesiolysis in 1. The authors conclude that early diagnosis followed by CEHE is the treatment of choice for choledochal cyst, and intraoperative cyst endoscopy is recommended as a valuable adjunct to CEHE.

                Author and article information

                J Indian Assoc Pediatr Surg
                Journal of Indian Association of Pediatric Surgeons
                Medknow Publications (India )
                Apr-Jun 2011
                : 16
                : 2
                : 54-57
                Department of Pediatric Surgery, N R S Medical College, Kolkata - 700 014, India
                [1 ]Department of Pathology, Institute of Postgraduate Medical Education & Research, Kolkata - 700 020, India
                Author notes
                Address for correspondence: Dr. Biswanath Mukhopadhyay, 7E, Dinobandhu Mukherjee Lane, Sibpur, Howrah - 711 102, West Bengal, India. E-mail: drbmukhopadhyay@
                Copyright: © Journal of Indian Association of Pediatric Surgeons

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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