Choledochal Cyst Excision Research Articles

A large stone detected in Roux-en-Y jejunal limb 20 years after excision of congenital choledochal cyst

A 30-YEAR-OLDWOMAN was transferred to our hospital with pain in the right hypochondriac region. The patient had undergone excision of an extrahepatic cyst and Roux-en-Y reconstruction for a congenital choledochal cyst (Todani type IVA) at 10 years of age. A magnetic resonance imaging scan demonstrated scattered tiny stones in the dilated intrahepatic bile duct. In addition, a huge low-intensity, oval-shaped mass approximately 8 cm in diameter was also detected in the dilated jejunal limb (Fig 1). Percutaneous transhepatic biliary drainage was performed for cholangitis; bile cultures were positive for Klebsiella. Serum tumor markers were within normal limits. During surgical exploration, a huge stone measuring 8.0 cm in diameter in the jejunal limb (Fig 2), and 92 intrahepatic stones were found. Because the jejunal limb above the level of transverse mesocolon was slack and long, we performed a resection of the jejunal limb including the previous hepaticojejunal anastomosis, removal of the stones, and re-hepaticojejunostomy. No jejunal obstruction was found during the operation. Biliary stenosis was present in the remnant hilar bile duct, which presented inflammation and fibrosis histologically. The stones both in the intrahepatic bile duct and the jejunal limb were calcium bilirubinate stones. The postoperative course was uneventful.

Pancreatitis complicated with dilated choledochal remnant after congenital choledochal cyst excision

We describe here three cases of pancreatitis after congenital choledochal cyst excision. In these three cases, the choledochal remnant in the pancreas head was markedly dilated, probably because of an incomplete resection of the cyst at the primary operation, and an increase in intraluminal pressure of the pancreatic duct caused by a dynamic obstruction by a protein plug or a pancreatic calculus. Complete cyst excision, including the choledochal wall in the pancreas, is therefore strongly recommended.

Experience of diagnosis and treatment of carcinoma arising from congenital biliary duct cyst

To summarize the experience in diagnosis, prevention and treatment of carcinoma arising from congenital biliary duct cyst. The clinical and pathological data of 185 patients with congenital biliary duct cyst admitted to Chinese PLA General Hospital were analyzed retrospectively. Among 185 patients, twenty-seven cases had carcinomas arising from congenital biliary duct cyst, and the frequency of malignant transformation was 14.6%, which closely related to the age (P < 0.001). The incidences of malignancy for different age groups were: 0 for 0-9 age group, 5.1% for 0-19, 9.1% for 20-29, 16.2% for 30-39, 26.7% for 40-49, 33.3% for 50-59, and 50% for over 60, respectively. Six patients had the history of cyst-enterostomy. Abdominal pain, fever, jaundice and weight loss were the main clinical manifestations. Abdominal ultrasonography, CT, MRI or magnetic resonance cholangiopancreatography, MRCP and endoscopic retrograde choledochopancreatography (ERCP) were the main diagnostic methods. For twenty patients (74.1%), a definite diagnosis was made preoperatively, but it's hard to make an early diagnosis. Nine patients (33.3%) underwent curative resection. Congenital biliary duct cyst is a premalignant lesion, and the incidence of carcinogenesis increases remarkably with age. The most effective method for prevention of carcinogenesis in choledochal cyst is complete excision of choledochal cyst during childhood, and the prognosis is poor for patients with biliary malignancy.

Is Early Excision of Choledochal Cyst in Neonate Necessary?

Is Early Excision of Choledochal Cyst in Neonate Necessary?

Laparoscopic-assisted total cyst excision of choledochal cyst and Roux-en-Y hepatoenterostomy

Purpose The aim of this study was to describe the technical experience and outcome in laparoscopic-assisted total cyst excision of choledochal cyst with Roux-en-Y hepatoenterostomy. Methods Thirty-five patients with choledochal cyst were studied. Their age ranged from 3 months to 9 years (average age, 3.6 years). The choledochal cysts were cyst type in 33 cases and fusiform type in the other 2 cases. Four trocars were utilized with 3- to 5-mm instrumentation. Under laparoscopic guidance, the gallbladder and the dilated bile duct were excised completely. The Roux-en-Y jejunojejunostomy was performed extracorporeally by exteriorizing the jejunum through the extending umbilical incision (1.5 to 2 cm), and an end-to-side hepaticojejunostomy was carried out intracorporeally by the hand suture methods. Results Average duration of operation was 4.3 hours (range, 3.5 to 7.6 hours), intraoperative blood loss was 5 to 10 mL, and 8 of the 35 patients had associated hepatic ductal stenosis and underwent laparoscopic excision of the cyst and ductoplasty. In 1 of the 8 cases, bile leak was noticed from day 1 through 26 postoperatively. The postoperative course was uneventful in the other 34 patients with a hospital postoperative stay ranging from 3 to 6 days. There were no postoperative complications in the 3-month to 1 1 2 -year follow-up. Conclusions Laparoscopic-assisted total cyst excision with Roux-en-Y hepatoenterostomy is feasible for the treatment of choledochal cyst in children.

Duodenogastric reflux following biliary reconstruction after excision of choledochal cyst

Duodenogastric reflux (DGR) was assessed in patients surgically treated for choledochal cyst, with emphasis on two different biliary reconstruction methods: Roux-en-Y hepaticojejunostomy (HJ) and hepaticoduodenostomy (HD). Gastric bile monitoring with the Bilitec device revealed excessive DGR in patients in the HD group. Endoscopic findings demonstrated mild to moderate gastric mucosal erosion in patients after HD. In contrast, neither DGR nor gastritis was found in patients after HJ. This preliminary study suggests that HJ, rather than HD, should be recommended as a method of biliary reconstruction for pediatric patients with choledochal cyst. Careful observation of DGR should be continued in patients who have undergone HD.

TYPE II CHOLEDOCHAL CYST A RARE CAUSE OF OBSTRUCTIVE JAUNDICE AND IN A PATIENT WITH CELIAC DISEASE

53 yo patient with history of iron deficiency anemia due to celiac disease presented with jaundice and abdominal pain radiating to the back. Upon examination patient was grossly jaundiced, had right upper quadrant tenderness but no palpable mass was felt. Laboratory values were total bilirubin was 11.2 mg/dl, direct bilirubin 7.8 mg/dl, ALT 143 mg/dl, AST 128 mg/dl, Alk. Phos 218 mg/dl, WBC 13.4K with no left shift. Abdominal CT and RUQ ultrasound revealed a subhepatic cystic mass and no gallstone or biliary dilation was noted. ERCP revealed complete obstruction of CBD by external compression 3cm proximal to the ampulla. A sphinterotomy was done and a guidewire was advanced beyond the distorted and obstructed CBD, during advancement the guidewire curled in the choledochal cyst in the opposite direction of the gallbladder. As there was no evidence of perforation diagnosis of type II choledochal cyst was made. A plastic stent positioned above the choledochal cyst. Placement of the stent relieved the external compression of the cyst. Drainage established and patient rapidly improved. Choledochal cyst excision and Roux-en-Y anastomosis was performed electively. There was no evidence of malignancy in the pathology specimen. Discussion: A choledochal cyst is defined as an isolated or combined congenital dilation of the intrahepatic or extrahepatic biliary tree. Todani and colleagues proposed the five types of congenital cysts. Incidence of choledochal cysts is 1 in 13,000 to 15,000 in Western countries. Type II choledochal cyst, a dicerticulum of common bile duct, is the rarest form. Classic presentation triad is obstructive jaundice, right upper quadrant abdominal pain and palpable abdominal mass. Celiac Disease is a genetically-determined chronic inflammatory intestinal disease induced by an environmental precipitant, gluten. Celiac Disease is very common effecting about one in 250 people. Celiac Disease is associated with many autoimmune diseases including IDDM, Autoimmune thyroiditis, ulcerative colitis, autoimmune hepatitis and malignancies especially lymphoma. Considering the rarity of choledochal cyst and frequency of Celiac Disease association is likely of a casual nature, however this is the first case report of type II choledochal cyst in a patient with celiac disease.

Laparoscopic biliary reconstruction

Background Biliary reconstruction represents a relatively untested frontier in laparoscopy. Methods Retrospective review of all patients who underwent laparoscopic biliary operations at Legacy Health System from 1998 to 2003. Results Seven patients underwent laparoscopic biliary reconstruction. Indications included benign calculous disease in 4 patients, benign stricture on 1 patient, choledochal cyst in 1 patient, and malignant biliary obstruction in 1 patient. Operations performed included choledochoduodenostomy, hepaticojejunostomy, stricturoplasty, choledochal cyst excision with hepaticojejunostomy, and cholecystojejunostomy. Median operative time was 300 minutes. Median hospital stay was 4 days. One perioperative complication of a bowel obstruction required reoperation. Median follow-up was 15 months. One patient died of metastatic cancer 8 months after surgery. All other patients are symptom free with no signs of stricture or recurrent biliary obstruction. Conclusions Laparoscopic biliary reconstruction represents a viable treatment option in carefully selected patients.

Intracorporeal electrohydraulic lithotripsy for intrahepatic bile duct stone formation after choledochal cyst excision

An 18-year-old girl who had undergone excision of a choledochal cyst and Roux-en-Y hepatico-jejunostomy at another hospital when 23 months old was referred to our department because of recurrent cholangitis. Radiological investigations showed stones lying in minimally dilated, right posterior intrahepatic bile ducts (IHBD). At laparotomy, the hepatico-jejunostomy site was incised, and a flexible endoscope inserted into the IHBD. Multiple stones packed in the IHBD were easily fragmented using an electro-hydraulic lithotripsy (EHL) device inserted through the endoscope, and removed. There were no EHL-related complications, and her postoperative progress was uneventful. She is currently well with no episodes of cholangitis after a follow-up period of 3 years. EHL is a simple, effective alternative method for removing IHBD stones after choledochal cyst excision. To the best of our knowledge, this is the first report of EHL being used to remove stones that developed in the IHBD after choledochal cyst excision.

Anomalous choledocho-pancretic ductal junction in a choledochal cyst: A case report

Choledochal cysts are rare congenital anomalies, mostly detected in adults. Pathogenesis of these cysts seems to be in anomalous junction between pancreatic and common bile duct, above the papillary sphincter and outside of the duodenal wall. The absence of the sphincter above the junction is followed by reflux of the pancreatic juice into the bile duct leading to dilatation and fibrous changes of bile duct wall. A 38-year-old female is presented in whom a choledochal cyst was found 11 years earlier, during the operation performed for obstructive jaundice, when cystojejunostomy with Roux-en Y Jejunal limb was carried out. In February 1990, she was admitted to our Institution forjaundice and biliary colic. The patient was reoperated. Operative cholangiography showed an anomalous pancreatobiliary junction, choledochal cyst, dilated cystic duct and moderate dilatation of intrahepatic bile ducts. Cholecystectomy, desanastomosis with partial excision of choledochal cyst, and retrocolic choledochojejunostomy with the same Roux-en-Y jejunal limb were performed. Total excision of choledochal cyst was too risky due to chronic inflammatory changes in the hepatoduodenal ligament. Postoperative recovery was uneventful and the patient remained symptom-free so far.

Experience of Congenital Choledochal Cyst in Adults: Treatment, Surgical Procedures and Clinical Outcome in the Second Affiliated Hospital of Harbin Medical University

This study was undertaken to analyze and evaluate the diagnosis and principal treatment methods for congenital choledochal cyst, focusing on various surgical procedures and clinical outcome. A comprehensive, retrospective study was conducted on 72 adult patients who presented with choledochal cyst from 1985 to 2002. Surgical procedures were cyst excision with hepaticojejunostomy in 25 cases for type I or type IV-B, extrahepatic cyst excision with hepaticojejunostomy in 8 cases for type IV-A, extrahepatic cyst excision with modified hepaticojejunostomy in 2 cases for type IV-B, non-cyst excision with or without hepaticojejunostomy in 27 cases for types I, II, IV-A, IV-B. The early postoperative morbidity and mortality rate were 16.1% (9/62) and 6.5% (4/62) respectively, and the complication rate related to surgical procedure was 30.6% (19/62). The incidence of cholangiocarcinoma with non-cyst excision or non-operated congenital choledochal cyst was 10.8% (4/37). One patient died of primary hepatocellular carcinoma after cyst excision with hepatojejunostomy. In conclusion, our results showed that complete exci-sion of choledochal cyst for types I, II, and IV-B and complete excision of extra-hepatic choledochal cyst from the hepatic hilum in type IV-A with hepaticojejunostomy or modified hepaticojejunostomy are the treatment of choice for choledochal cyst in adult patients.

Recommendations for preventing complications related to Roux-en-Y hepatico-jejunostomy performed during excision of choledochal cyst in children

Purpose: The aim of this study was to review the outcome of a protocol the authors proposed for preventing Roux-en-Y hepatico-jejunostomy (RYHJ) related complications. Methods: In 1986 the authors adopted the following protocol for improving RYHJ to minimize risks for RY-related complications: (1) end-to-end anastomosis if possible; (2) if end-to-side anastomosis is unavoidable, the common hepatic duct should be anastomosed as close as possible to the closed end of the blind pouch (BP); (3) careful selection of the vascular supply to the RY jejunal limb; (4) length of the RY jejunal limb should not be predetermined, but individualized; (5) approximate the native jejunum to the RY limb. The authors reviewed 92 children who had surgery for CC (mean age at surgery, 4.1 years) at our institution between 1986 and 2002 to assess the effectiveness of their recommendations. Results: After the adoption of the authors’ recommendations for RYHJ, they have experienced no RYHJ-related complications after a mean follow-up period of 7.6 years (range, 4 months to 16 years). Conclusions: RYHJ in children is different from that in adults, because the RY limb or BP can grow and elongate as the child grows. The authors’ recommendations appear to have successfully prevented RY-related complications up to the time of this review, and they highly recommend their use.

Clinical and Operative Findings of Choledochal Cysts in Neonates and Infants Differ From Those in Older Children

Significant differences in the clinical and pathological presentation of choledochal cysts between infants and older children have been noted. We developed a new management strategy according to these differences. Between 1990 and 2001, we managed 34 cases of type I choledochal cyst. Nineteen patients were more than 1 year old. Fifteen patients underwent surgery before the age of 1 year, and eight before the age of 2 months. One patient was diagnosed prenatally. All patients underwent choledochal cyst excision and hepaticojejunostomy. Symptoms and signs, and findings from physical examination, laboratory tests, and pathology were recorded and analysed. Follow-up periods ranged from 6 months to 11 years. Eight cases who were less than 1 year old suffered from painless jaundice, and choledochal cysts in this group usually ended as a blind pouch. Their cystic amylase and lipase concentrations were within normal limits. Biliary cirrhosis was diagnosed by liver biopsy in three of these patients. In contrast, 17 of the 19 patients who were more than 1 year old had abdominal pain, and 15 of their cysts were connected to the pancreatic duct. Cystic amylase and lipase concentrations were often elevated. All patients had an excellent outcome. Diseases with different pathogeneses may cause different clinical manifestations and pathology of choledochal cysts in infants and older children. Some patients develop biliary cirrhosis in the early months of life. Our findings show that it is safe and imperative to treat neonates and infants with choledochal cysts as soon as possible, as delayed surgery may cause severe complications.

P-103 Intracorporeal electro hydraulic lithotripsy for intrahepatic bile duct stones after choledochal cyst excision

P-103 Intracorporeal electro hydraulic lithotripsy for intrahepatic bile duct stones after choledochal cyst excision

Choledochal cysts in adults.

Choledochal cyst is rarely diagnosed in adulthood. When complicated by biliary tract malignancy, the disease has a distinct presentation and carries a dismal prognosis despite radical surgical resection. Retrospective study. Tertiary referral center. A retrospective study was performed on 30 adult patients who presented with choledochal cyst from January 1, 1989, to December 31, 2000. The clinical presentation, management, and outcome of patients with and without biliary tract malignancy. Nine patients (30%) had biliary tract malignancy complicating choledochal cyst (group A). Compared with 21 patients without malignancy (group B), group A patients had a significantly higher incidence of previous internal drainage operations for choledochal cyst (P =.049) and presentation with cholangitis (P =.03). Four patients in group A underwent pancreaticoduodenectomy and 3 received a palliative biliary drainage operation. The overall median survival of patients in group A was 12 months. Complete excision of choledochal cyst and Roux-en-Y hepaticojejunostomy were performed for all patients in group B, among whom 2 underwent concomitant hemihepatectomy. The operative morbidity and mortality were 14% and 0%, respectively, and there were no long-term complications with a median follow-up of 66 months. Biliary tract malignancy complicating choledochal cyst in adults should be suspected in patients with a history of internal drainage of choledochal cyst and presentation with cholangitis. Complete excision of choledochal cyst with Roux-en-Y hepaticojejunostomy is the treatment of choice for patients without malignancy and can be performed with low operative morbidity and absence of long-term complications in adult patients.

Choledochal Cysts in Adults—Invited Critique

Hypothesis: Choledochal cyst is rarely diagnosed in adulthood. When complicated by biliary tract malignancy, the disease has a distinct presentation and carries a dismal prognosis despite radical surgical resection. Design: Retrospective study. Setting: Tertiary referral center. Patients: A retrospective study was performed on 30 adult patients who presented with choledochal cyst from January 1, 1989, to December 31, 2000. Main Outcome Measures: The clinical presentation, management, and outcome of patients with and without biliary tract malignancy. Results: Nine patients (30%) had biliary tract malignancy complicating choledochal cyst (group A). Compared with 21 patients without malignancy (group B), group A patients had a significantly higher incidence of previous internal drainage operations for choledochal cyst (P=.049) and presentation with cholangitis (P=.03) Four patients in group A underwent pancreaticoduodenectomy and 3 received a palliative biliary drainage operation. The overall median survival of patients in group A was 12 months. Complete excision of choledochal cyst and Roux-en-Y hepaticojejunostomy were performed for all patients in group B, among whom 2 underwent concomitant hemihepatectomy. The operative morbidity and mortality were 14% and 0%, respectively, and there were no long-term complications with a median follow-up of 66 months. Conclusions: Biliary tract malignancy complicating choledochal cyst in adults should be suspected in patients with a history of internal drainage of choledochal cyst and presentation with cholangitis. Complete excision of choledochal cyst with Roux-en-Y hepaticojejunostomy is the treatment of choice for patients without malignancy and can be performed with low operative morbidity and absence of long-term complications in adult patients.

Development of intrahepatic biliary stones after excision of choledochal cysts

Background: The incidence of intrahepatic cholelithiasis and cholangitis has not yet been well studied postoperatively in patients with choledochal cysts. Methods: One hundred three patients with choledochal cysts had operative cholangiography, underwent standard excision of a choledochal cyst with Roux-en-Y hepatico-jejunal anastomosis, and were at a mean follow-up of 12½ years. The incidence of intrahepatic bile duct stones was analyzed according to the 3 morphologic types of intrahepatic bile duct observed at initial operative cholangiography: type 1, no dilatation of the intrahepatic bile ducts; type 2, dilatation of the intrahepatic bile ducts but without any downstream stenosis; and type 3, dilatation of the intrahepatic bile ducts associated with downstream stenosis. Initially, there was no evidence of intrahepatic bile duct stones in any of the 103 patients. Results: Among 50 type 1 patients, intrahepatic cholelithiasis developed in only 1 patient (2%). Among 43 type 2 patients, 1 patient (2%) had intrahepatic cholelithiasis, and 2 (5%) had postoperative cholangitis. Among 10 type 3 patients, 4 (40%) had intrahepatic cholelithiasis (P <.01), and 3 (30%) had postoperative cholangitis. Time intervals between the initial surgery and the first identification of intrahepatic stones ranged from 3 to 22 years. Conclusions: One of the major causes of formation of intrahepatic cholelithiasis has been clarified; patients with intrahepatic biliary dilatation with downstream stenosis can get intrahepatic bile duct stones long after excision of a choledochal cyst. J Pediatr Surg 37:165-167. Copyright © 2002 by W.B. Saunders Company.

Cisto de colédoco em criança

We report the case of a one-year and two-months-old child with a choledochal cyst tYpe I of Alonso-Lej and Todani's classification , diagnosed through abdominal ultrasound. The surgical treatment was cholecistectomy and choledochal cyst excision with Roux-in-Y hepatic- jejunostomy. Minor complications were observed during early postoperative recovery. Long-term flow-up has been uneventiful, with overall improvement.

Tc-99m DISIDA Scan Findings in Two Large Choledochal Cysts in the Same Patient

The authors describe a 5-day-old neonate with two large choledochal cysts arising from the hepatic duct and pancreatic portion of the common bile duct. She underwent Tc-99m DISIDA hepatobiliary scintigraphy for two large cystic masses in the liver and right abdomen that were detected on prenatal ultrasound. The scan revealed delayed filling of the two cysts with bile, indicating large choledochal cysts. She was treated with excision of the lower choledochal cyst, cholecystectomy, and choledochojejunostomy. Histologic analysis confirmed a choledochal cyst. The upper intrahepatic choledochal cyst was left alone, with no surgical intervention.

Management of accessory hepatic ducts in choledochal cysts

This report describes the surgical management of 2 children with fusiform choledochal cysts who had accessory hepatic ducts (AHD) identified during excisional surgery for fusiform choledochal cysts (CC). Two children presenting with a triad of recurrent jaundice, fever, and abdominal pain were investigated and found to have type 1 choledochal cyst. Preoperative imaging and intraoperative cholangiography missed the AHD in both cases. In one of the patients, the main and the accessory ducts were separated by the right hepatic artery. In both the patients the accessory ducts were reconstructed successfully into a Roux loop along with the main common hepatic duct. Follow-up studies showed no evidence of biliary tract obstruction or atrophic changes in the liver. There was satisfactory uptake and drainage on hepatic scintigraphy. During excision of CC, AHD may be encountered. These may be missed on preoperative imaging. AHD may have a close relationship with neighboring vascular structures in the porta. Accessory hepatic ducts should be anticipated, identified, and reimplanted into the Roux loop during excisional surgery. J Pediatr Surg 36:1092-1093. Copyright © 2001 by W.B. Saunders Company.