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.