CHOLEDOCHAL CYSTS: OUR EXPERIENCE

Abstract

Eight patients of choledochal cyst have been diagnosed and six have been managed surgically. There were two children and six adults. Both the children had Type I cyst. They were managed by excision of the cysts. One lady with associated stone and carcinoma of the gall bladder underwent radical cholecystectomy with excision of segments IV and V of liver. Second lady with intrahepatic porcelain gall bladder had a choledochocele and was treated by transduodenal sphincteroplasty. Fifth patient, an 82 years old man with symptomatic gall stone with Type I choledochal cyst was subjected to just a cholecysto-jejunostomy. A 54 years old man with a Type IV cysts has undergone excision of the cyst. In patients undergoing excision of choledochal cysts, the biliary tract was reconstructed by a Roux-en-Y hepaticojejunostomy. Two of our patients never turned up for surgery. All the operated patients were asymptomatic during the follow-up period of 6 months to 24 months.

Introduction

Cholecdochal cyst may be defined as a rare congenital dilatation of common bile duct that is associated not infrequently with congenital or acquired dilatation of the intrahepatic ducts [1]. Choledochal cysts were originally described in 1723 by Vater and Ezler [2]; and the first surgical excision was performed by Whorter in 1924[3]. Even though it is rare, over 3000 cases have been reported by the year 1988 [4]. The typical patient of choledochal cyst is a female child with painful abdominal lump and jaundice. However at The John Hopkins hospital adult patients were more common [2]. Excision and bilioenteric anastomosis is the treatment of choice in extrahepatic cysts [5].

Patients and Methods

From July 95 to Jan ‘97 we had 8 patients of choledochal cyst. There were five women and three men. Age ranging from 4 years to 82 years. A detail history was taken, 6 patients gave history of pain abdomen. One case was explored for acute abdomen and found to have fat necrosis indicating acute pancreatitis with raised peritoneal fluid amylase of 22,000 IU/dl. Only two patients had clinical jaundice during admission with elevated serum alkaline phosphatase. Four patients had high intermittent fever and one of them was investigated as pyrexia of unknown origin.

Leucocytosis was noticed in three cases, elevated serum bilirubin in two. Ultrasound was done in all cases, computed tomography scan was done in five cases. One case was confused as pseudocyst and in other cases dilatation of biliary tree was noticed. All cases were confirmed by ERCP, except one which was confused with pseudocyst of pancreas. We explored through right subcostal incision in five cases and the case operated earlier for acute pancreatitis was operated through previous scar. Side to side cholecystojejunostomy was done using 2-0 continuous chromic catgut in 82 year old male patient. In all other cases, gall bladder was mobilised by fundus first method. Cystic artery was ligated, CBD taped and lifted up from the portal vein and removed from the junction to the infraduodenal part and lower end was ligated. Roux loop was made around 25 cms from duodenojejunal flexure and end to side bilioenteric anastomosis was done using 3-0 vicry in single layer over a tube after closing the end of jejunum in two layers. Abdomen was closed after placing a tube drain. One case developed peri T tube bile leak and managed by low pressure suction. Another case developed excessive discharge probably due to injury to accessory pancreatic duct which stopped in three days with injection sandostatin 50 micro grams, SC, 6th hourly. All other cases recovered well. T tube was removed after three weeks after doing T tube cholangiogram (Table 1).

TABLE 1.

S.No Age/Sex		Presentation Cholangiogram		Association	Procedure
1.	4F	Fever, jaundice, Pain		Focal Pancreatitis head	Excision BEA
2.	5M	Acute pancreatitis (optd)		—	Excision BEA
3.	56F	Flatulent dyspepsia		Gall Stone, carcinoma gall bladder	Radical Cholecystectomy BEA
4.	44F	PUO		Intrahepatic porcelain GB	Transduodenal sphincteroplasty
5.	82M	Recurrent pain abdomen Fever		Gall Stone	Cholecystojejunostomy
6.	54M	Recurrent cholangitis		—	Excision and BEA
7.	46F	Vague pain/dyspepsia		—	Not operated
8.	17F	Recurrent jaundice		—	Not operated

BEA – Bilioenteric anastomosis, PUO – Pyrexia of unknown origin

Discussion

Bile duct cysts (choledochal cyst) are an uncommon finding[5]. The classical presentation of jaundice, lump and fever is seen in 13-63% of patients [6]. Majority of patients present in childhood and only 20% present beyond 16 years of age [7]. In a recent review the mean age was found to be 4.3 years in 180 cases [8]. Increase in the adult patients is possibly due to more number of ERCP in adults in our institute. Women outnumber men in most of the studies [9]. Acute pancreatitis is seen in 30-40% of patients [10]. In our study 2 out of 8 patients had pancreatitis. In one patient choledochal cyst was confused as a pseudocyst both in ultrasound and computerised tomogram scan with only one such case report in the literature [10]. The delay in the diagnosis in adults is due to the nonspecific symptoms. [11]. In our study two adults had presented with gall stones and the third with pyrexia of unknown origin. ERCP was diagnostic in first two patients and per-operative cholangiogram in third. Cholangiocarcinoma in choledochal cyst is a known complication [12] even after excision of cyst [13] and association with carcinoma gall bladder has been reported by Tan [14]. We had a similar case of carcinoma gall bladder associated with a choledochal cyst. There are reports of anomalous pancreaticobiliary junction in choledochal cyst [15]. But it became difficult for us to interpret such anomaly in absence of an image intensifier.

Excision of the choledochal cyst and primary bilioenteric anastomosis is confirmed as the treatment of choice [16]. We had four cases of extra hepatic choledochal cyst. Two Type I cysts in children were managed by cyst excision and Roux-en-Y bilioenteric anastomosis. One woman with associated carcinoma gall bladder with gall bladder stones has undergone radical cholecystectomy involving removal of whole of extrahepatic biliary tree along with segment IV & V of liver and reconstructed by Roux-en-Y bilioenteric anastomosis 30 cms from duodenojejunal junction. Similar case of choledochal cyst and carcinoma gall bladder has also been reported from China [17]. The 4th patient found to be having a choledochocele on per-operative cholangiogram, underwent transduodenal sphincteroplasty which is one of the preferred methods in adults [18, 19]. In the fifth patient, an 82 year male with Type I choledochal cyst, minimum procedure, cholecysto-jejunostomy was done after removal of stone, in view of his age. Two of our patients never turned up for surgery after ERCP confirmation of diagnosis and were lost to follow up. All our operated patients are doing well during the follow-up period of 6 months to 24 months except one readmission for 3 days.