Journal of Indian Association of Pediatric Surgeons, Vol. 10, No. 2, April-June, 2005, pp. 86-88
Biliary ductal and vascular anomalies associated with choledochal cyst
Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi
Code Number: ip05021
ABSTRACTNineteen patients operated for choledochal cyst over a 7-year period were reviewed retrospectively. Cyst excision and Roux -en -Y hepaticojejunostomy was done in all patients but one; one patient was managed with cyst excision and an antiperistaltic hepatico-appendico-duodenostomy. About one-third of patients (6/19) had biliary ductal/vascular anomalies identified at surgery. These included anomalous right hepatic artery (n = 3), primary ductal stricture (n = 2), and aberrant right hepatic duct (n = 1). A high degree of awareness of biliary ductal and vascular anomalies is required on the part of the operating surgeon to facilitate accurate intraoperative diagnosis as well as successful reconstructive surgery.
Keywords: Choledochal cyst
Biliary duct anomalies are well known; they often result in significant intraoperative complications and postoperative morbidity if unrecognised at the time of surgery. Recently, a few authors have noticed a high degree of association between biliary anomalies and choledochal cysts. A retrospective analysis of records of 19 patients operated for choledochal cysts over a period of 7 years (April 1998-March 2005) was done to determine the incidence of biliary duct and vascular anomalies in association with choledochal cysts.
The records of 19 patients operated for choledochal cysts during 7-year
period were reviewed retrospectively; two patients who were diagnosed to
have forme fruste choledochal cyst have not been included in this series.
There were 11 females and 8 males. Their ages ranged from 4 months to 13
years with five of them presenting in infancy, five presenting in the age
group 1-5 years, another five presenting between 5 and 10 years of age
and four presenting above 10 years of age. Abdominal pain was the commonest
complaint and was present in 79% (15/19) of the patients; jaundice
was seen in 63% (12/19) of the patients, whereas a palpable abdominal
lump was noticed only in 21% (4/19) of the patients. None of the
patients had the classical triad of symptoms.
RESULTSAssociated biliary/vascular anomalies were found in close to one-third (32%) of the patients. Primary ductal stricture was seen in two patients, aberrant right hepatic duct was seen in one patient, and anomalous right hepatic artery was found in three patients. All but one of these anomalies were not known preoperatively and all were diagnosed intraoperatively. One patient with anomalous right hepatic artery had excessive intraoperative haemorrhage due to injury to this vessel, which was repaired using 8-0 Gore-Tex using operating loop. The patient with aberrant right hepatic duct also sustained injury of the anomalous duct; which was diagnosed and managed intraoperatively. Postoperatively this patient had biliary leak, which responded well to percutaneous drainage.
One patient had massive blood loss of an unsuspected bleeding diasthesis (due to wrong preoperative laboratory reports) intraoperatively and died immediate postoperatively of shock and DIC. Another patient, who had infantile variety choledochal cyst with proximal ductal stricture, died of bronchopneumonia 2 months after surgery. One patient needed re-exploration, revision anastomosis, flexible choledochoscopy and removal of intrahepatic biliary sludge and calculi, 4 years after the initial surgery; since then the patient has been doing well. Other 16 patients have been doing well with no clinical or biochemical evidence of cholestasis. In all, 14 patients have had a long-term follow-up ranging from 6 months to 6 years.
DISCUSSIONMany authors agree that ′normal biliary anatomy′ is seen in less than 50% of patients undergoing biliary tract procedures. The various anomalies mentioned in the literature include primary ductal stricture, aberrant hepatic duct, anomalous hepatic arteries, low confluence of hepatic duct and a very small bile duct.
Primary Ductal Strictures often require hilar anastomosis, redo surgeries due to persistence of symptoms because of missed proximal stricture or anastomotic strictures, and ductoplasties. Todani et al. have described primary ductal strictures at the hilum, at the confluence of right and left hepatic ducts, and at the junction of hepatic duct with cystic duct., They have classified these strictures as simple strictures and membranous strictures. In their series, one patient had a left hepatic duct stricture, which was ignored initially. Postoperatively, the patient developed recurrent episodes of cholangitis for which the patient was investigated and re-explored 15 years after the primary surgery. He underwent excision of the atrophic left lateral segment.
Coexistence of a type 1 infantile variety of choledochal cyst with distal biliary stricture has been frequently mentioned in the literature, but association of ductal stricture proximal to choledochal cyst has not been reported hitherto. One such case was treated; he was misdiagnosed as biliary atresia preoperatively.
An aberrant hepatic duct (AHD), although rare, is a significant accompaniment to choledochal cysts., An AHD is an extra-segmental or sub-segmental bile duct that often drains a part of the right hepatic lobe. It may join the biliary system at any level such as the cystic duct, gall bladder, right hepatic duct, common hepatic duct or common bile duct. The incidence of AHD varies from 1 to 31% in different operative and autopsy series. Greenhoze and Lily successfully used vascularised appendicular graft to bridge the incidental right accessory hepatic duct to Roux-en-y- hepaticoportoenterostomy. Todani et al. have suggested anastomosing these with one of the main ducts and then performing hepaticojejunostomy, while Narsimhan et al. believed in creating a separate biliary enteric anastomosis with the aberrant hepatic duct., Awareness of this entity is important in order to avoid intraoperative injury or accidental ligation. When identified, they should be included in the anastomosis. If the AHD is damaged or goes unrecognised at surgery, postoperative bile leak may result in biliary peritonitis, biliary fistula, or abscess formation. If the AHD is accidentally ligated and remains unrecognised, it may result in infection of the lobe and subsequent atrophy of the obstructed segments with compensatory hypertrophy of the unaffected segment. Ligation of small sized AHD may be a viable option, however, in large AHDs, reconstructive surgery is recommended by most.,
Caution to identify the presence of anomalous arterial supply cannot be overemphasized, since it is the only way to avoid injury or accidental ligation - both of which can result in a major surgical disaster. The aberrant artery running in front of the common hepatic duct can be easily displaced behind the jejunal loop at the time of reconstruction.
To conclude, biliary and vascular anomalies are frequently seen in association with choledochal cysts. They have tremendous implications as they are seldom diagnosed preoperatively, cause significant intraoperative complications and require a high degree of awareness on part of the operating surgeon, to facilitate accurate intraoperative diagnosis as well as successful reconstructive surgery.
Copyright 2005 - Journal of Indian Association of Pediatric Surgeons