CHOLEDOCHAL CYSTS: OUR EXPERIENCE Wg Cdr ASWINI KUMAR PUJAHARI *, Col KARAM VIR SINGH +, Dr SRIHARSHA BANGARASWAMY H, Sqn Ldr CHIrTOOR NAGABUSHNAM SATISH 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 sphinctcroplasty. 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. MJAFI 1999; 55: 123-125 KEY WORDS:-Choledochal cyst; Hepaticojejunostomy; Sphincteroplasty.
Introduction
C
holecdochal 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 perfonned 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 i\'lethods 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 palients 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 palients had high intermittent fever and one of them was investigated as pyrexia of unknown origin. Leucocytosis was noticed in three cases. elevated serum biliru· bin in two. Uhrasound was done in all cases. computed tomography scan was done in five cases. One case was confused as pseudocyst and in olher cases dilatation of biliary tree was noticed. All cases were confirmed by ERCP, except one which was con-
fused with pseudocyst of pancreas. We explored through right subcostal incision in five cases and the case operated earlier for acute pancreatilis 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. can 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 ems from duodenojejunall1exure 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 10 injury to accessory pancreatic duct which stopped in three days with injeclion 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 I).
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
• Classified Specialist in Surgery and Surgical Gastroenterologist, + Senior Adviser (Medicine & Medical Gastroenterology). /I Post graduate Trainee in Surgery, Command Hospital Air Force, Bangalore 560 007.
124
Pujahari, et al
TABLE I S.No Age/Sex
Presentation
L
4F
Fever, jaundice, Pain
2.
5M
Acute pancreatitis (optd)
3.
56F
Flatulent dyspepsia
4.
44F
PUO
5.
82M
Recurrent pain abdomen Fever
6.
54M
Recurrent cholangitis
7.
46F
Vague pain/dyspepsia
8.
17F
Recurrent jaundice
Cholangiogram
Association
Procedure
Focal Pancreatitis head
Excision DEA
Excision BEA
, o JJ JJ tJ
Gall Stone, carcinoma gall bladder
Radical Cholecystectomy
I mrahepat ic porcelain GB
Transduodenal sphincteroplasty
Gall Stone
Cholecyslojejunostomy
BEA
Excision and BEA
Not operated
Not operated
BEA - Bilioenteric anastomosis, PUO - Pyrexia of unknown origin
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 carci-
noma 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 MJAFl. VOL 55. NO.2. 1999
125
Choledochal Cysts
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 Raux-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. REFERENCES I. Howard ER. Choledochal cysts. In : Schwartz SI, Harold
Ellis, Wendy Cowles Hussers, editors. Muingots abdomin:l! operation, 8th Ed, Appleton-Centul)'-Crofts: 1985:1789. 2. Pamella A, Lipsett MD, Henry A, Pitt MD. Paul M, Colombani MD. 10hn K Boitnott .John Cameron MD. Choledochal Cyst disease: A changing pattern of presentation. Annals of Surgery 1994:220(5): 644-52. 3. Me Whorter GL. Congenital cystic dilatation of common bile duct: report of a case with cure. Annals of Surgery 1924;8:604-62. 4. KC Tan and Howard ER. Choledochal cysts: a 14 years surgical exaperience with 36 patients. Br 1 Surg 1988;75:892-5. 5. Buckel EG, Nagorney OM. Choledochal cysts in adults life. In : Blumgart LH, editors. Surgery of liver and biliary tract. 2nd ed. Churchill Livingstone 1994: 1183. 6. Howard ER. Choledochal cyst. In: Schwartz SI. Harold Ellis, Wendy Cowles Husser, editors. MaingolS abdominal operation, 8th cd. Appleton-Century-Crofts. 1985:1793. 7. Flamingan DP. Biliary cysts. Annals of Surgery. 1975;182:
MJAFf. I'OL 55. NO. 2, /999
635-43. 8. Miyano T , Yamal
Introduction
C
holecdochal 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 perfonned 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 i\'lethods 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 palients 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 palients had high intermittent fever and one of them was investigated as pyrexia of unknown origin. Leucocytosis was noticed in three cases. elevated serum biliru· bin in two. Uhrasound was done in all cases. computed tomography scan was done in five cases. One case was confused as pseudocyst and in olher cases dilatation of biliary tree was noticed. All cases were confirmed by ERCP, except one which was con-
fused with pseudocyst of pancreas. We explored through right subcostal incision in five cases and the case operated earlier for acute pancreatilis 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. can 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 ems from duodenojejunall1exure 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 10 injury to accessory pancreatic duct which stopped in three days with injeclion 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 I).
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
• Classified Specialist in Surgery and Surgical Gastroenterologist, + Senior Adviser (Medicine & Medical Gastroenterology). /I Post graduate Trainee in Surgery, Command Hospital Air Force, Bangalore 560 007.
124
Pujahari, et al
TABLE I S.No Age/Sex
Presentation
L
4F
Fever, jaundice, Pain
2.
5M
Acute pancreatitis (optd)
3.
56F
Flatulent dyspepsia
4.
44F
PUO
5.
82M
Recurrent pain abdomen Fever
6.
54M
Recurrent cholangitis
7.
46F
Vague pain/dyspepsia
8.
17F
Recurrent jaundice
Cholangiogram
Association
Procedure
Focal Pancreatitis head
Excision DEA
Excision BEA
, o JJ JJ tJ
Gall Stone, carcinoma gall bladder
Radical Cholecystectomy
I mrahepat ic porcelain GB
Transduodenal sphincteroplasty
Gall Stone
Cholecyslojejunostomy
BEA
Excision and BEA
Not operated
Not operated
BEA - Bilioenteric anastomosis, PUO - Pyrexia of unknown origin
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 carci-
noma 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 MJAFl. VOL 55. NO.2. 1999
125
Choledochal Cysts
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 Raux-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. REFERENCES I. Howard ER. Choledochal cysts. In : Schwartz SI, Harold
Ellis, Wendy Cowles Hussers, editors. Muingots abdomin:l! operation, 8th Ed, Appleton-Centul)'-Crofts: 1985:1789. 2. Pamella A, Lipsett MD, Henry A, Pitt MD. Paul M, Colombani MD. 10hn K Boitnott .John Cameron MD. Choledochal Cyst disease: A changing pattern of presentation. Annals of Surgery 1994:220(5): 644-52. 3. Me Whorter GL. Congenital cystic dilatation of common bile duct: report of a case with cure. Annals of Surgery 1924;8:604-62. 4. KC Tan and Howard ER. Choledochal cysts: a 14 years surgical exaperience with 36 patients. Br 1 Surg 1988;75:892-5. 5. Buckel EG, Nagorney OM. Choledochal cysts in adults life. In : Blumgart LH, editors. Surgery of liver and biliary tract. 2nd ed. Churchill Livingstone 1994: 1183. 6. Howard ER. Choledochal cyst. In: Schwartz SI. Harold Ellis, Wendy Cowles Husser, editors. MaingolS abdominal operation, 8th cd. Appleton-Century-Crofts. 1985:1793. 7. Flamingan DP. Biliary cysts. Annals of Surgery. 1975;182:
MJAFf. I'OL 55. NO. 2, /999
635-43. 8. Miyano T , Yamal
