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PubMed2003

Smallest Todani's type II choledochal cyst.

A choledochal cyst is defined as an isolated or combined congenital dilation of the extra hepatic or intrahepatic biliary tree. Todani and colleagues proposed the five types of congenital choledochal cysts which have gained widespread acceptance. Type II choledochal cyst, a diverticulum of common bile duct, is rarest, and most reported cases of Type II were as large as several centimeters in size. We herein report the case of a small Type II choledochal cyst which was resected at pancreatoduodenectomy for carcinoma of the papilla of Vater. A 58-year-old Japanese male was referred to our hospital for the evaluation of jaundice. Preoperative cholangiogram via the percutaneous transhepatic biliary drainage tube revealed a complete obstruction at the narrow terminal segment. Furthermore, a small diverticular protrusion was demonstrated on the lower part of the common bile duct. The resected specimen showed a 2.2 x 1.7 x 1.2 cm carcinoma of the major papilla, and a deep, 2 mm in diameter and 5 mm in depth, depression on the posterior wall of the common bile duct. The anomalous pancreatobiliary duct was not seen. The deep depression was confirmed microscopically to penetrate the fibromuscular layer of the common bile duct and diagnosed as a Todani's Type II choledochal cyst. To our knowledge, the current case is the smallest Type II choledochal cyst which was completely resected.

Cholangiography↗
PubMed2005

Adult choledochal cyst.

OBJECTIVE: Choledochal cyst is a rare disease in adults. Excellent results have been achieved with proper management. However, malignancy complicating choledochal cyst still carries a dismal prognosis despite radical surgery. The aim of this study was to review the clinical course and operative results of the disease in adulthood, with emphasis on the occurrence of malignancy. METHODS: A retrospective review of adult patients who underwent surgery for choledochal cysts in a 12-year period was performed. RESULTS: Of the 25 adults, 80% were female and the median age was 30 years. A total of 32% of patients had previous drainage procedures for their choledochal cyst disease. Malignancy was noted in five patients (20%). There were significantly more males among the patients with malignancy than among those with benign disease (60% vs 10%), a greater number of older patients (median age, 39 vs 27 years) and more impaired liver function tests, but there was no difference in terms of incidence of previous drainage procedures between the two groups. There was no operative mortality; operative morbidity was 36%. No significant long-term complications were noted in the benign group but three of the five patients with malignancy died or developed recurrence within a few months after surgery. CONCLUSION: The prognosis for patients with malignancy complicating choledochal cyst remains poor despite aggressive radical surgery such as Whipple's operation or additional hepatectomy. Malignancy should be suspected in older and male patients who present with jaundice or impaired liver function tests. Total cyst excision and hepaticojejunostomy is an effective and safe treatment for patients with the common type I choledochal cyst in order to reduce the chance of subsequent development of malignancy.

Adult↗
PubMed2004

Expressions of p53 and inducible nitric oxide synthase in congenital choledochal cysts.

BACKGROUND: Choledochal cyst, an isolated defect unrestricted to the bile duct, is more appropriately regarded as the sentinel feature of a constellation of anomalies affecting the pancreatobiliary system. This study was to assess the relationship between the expression of inducible nitric oxide synthase (iNOS) and the p53 gene as well as the pathogenesis of choledochal cysts. METHODS: iNOS and p53 were detected by immunohistochemistry staining in 26 patients with congenital choledochal cysts. Histopathologically, hyperplasia of the mucosa of the cysts and the amylase level in the bile were also investigated. RESULTS: Patients with a high level of amylase in the bile had higher expression of iNOS than those with a low level of amylase. p53 protein was expressed neither in fusiform type nor in cystic type. The incidence of mucosal hyperplasia was significantly higher in the fusiform type than that in the cystic type. CONCLUSIONS: Higher expression of iNOS may participate in hyperplasia and carcinogenesis of the mucosa of choledochal cysts. The regurgitation of pancreatic juice into the biliary system might induce mucosal hyperplasia of the biliary tract and inflammatory reaction. In preventing regurgitation-caused hyperplasia and malignancy of the bi-liary tract, early surgery is important for children with congenital choledochal cysts.

Adolescent↗
PubMed1997

Malignant change in the biliary tract after excision of choledochal cyst.

BACKGROUND: Choledochal cyst is a rare congenital condition with a high risk of malignant change if untreated. The risk of malignancy after surgical excision of choledochal cyst is not known. METHODS: Forty-eight patients with choledochal cysts managed over a 21-year period were reviewed, to determine the risk of malignant change after cyst excision. Thirty-nine of 48 patients had no carcinoma at first admission; their mean(s.d.) age was 20(18) years. Thirty-seven of 39 patients underwent cyst excision and cholecystectomy followed by hepaticoenterostomy. RESULTS: Cyst excision was incomplete in 28 of the 37 patients because dilated portions of the biliary ducts remained proximally and/or distally. In these 37 patients, no carcinoma has developed in the remnant proximal hepatic duct or the terminal bile duct after mean(s.d.) follow-up of 9.1(6.4) years. In the remaining nine patients, biliary carcinoma was diagnosed at the first visit. Six patients died from recurrence with a mean(s.d.) survival time of 13(11) months, while three patients were alive and free from recurrence 2 months, 1 year and 7 years after operation. CONCLUSION: Malignant change has not been observed after total or subtotal excision of choledochal cysts in this series.

Adolescent↗
PubMed2003

Management dilemmas with choledochal cysts.

CONTEXT: Choledochal cysts are rare and of unknown cause. Their presentation is protean, with the classical triad of pain, jaundice, and mass rarely seen. The potential complications are serious, including pancreatitis, cholangitis, and cholangiocarcinoma. OBJECTIVE: To present the current experience and evidence relating to all aspects of choledochal cysts to derive appropriate management recommendations. METHODS: Review of relevant literature in the English language indexed on MEDLINE. RESULTS: The elaboration of the classification of choledochal cysts. We describe the modes of presentation and optimal investigation and summarize the current theories on etiology and malignant transformation. The results of different management strategies are presented. CONCLUSIONS: Choledochal cysts are often detected during the investigation of nonspecific symptoms, or even incidentally detected. Magnetic resonance imaging is the best imaging modality for the diagnosis and characterization of these cysts. Complete excision and hepaticojejenostomy is the management of choice.

Anastomosis, Surgical↗
PubMed1999

Choledochal cyst associated the with anomalous union of pancreaticobiliary duct (AUPBD) has a more grave clinical course than choledochal cyst alone.

OBJECTIVE: Since choledochal cyst is frequently associated with the anomalous union of pancreaticobiliary duct (AUPBD), AUPBD has been regarded to be the etiologic factor of choledochal cyst. However, the clinical significance of AUPBD an patients with choledochal cyst has not been clearly defined. Therefore, to clarify the significance of AUPBD in choledochal cyst patients, we compared the clinical features of patients with choledochal cyst according to the presence or absence of AUPBD. METHODS: Among 52 cases which were diagnosed as choledochal cyst out of 5,037 ERCP referrals between August 1990 and December 1996, we selected 44 cases, in which the pancreaticobiliary junction was clearly visualized on cholangio-pancreaticography. These cases were divided into AUPBD-present group (n = 28) and AUPBD-absent group (n = 16). Clinical features were compared between the two groups. Furthermore, in AUPBD-present group, clinical data were also analyzed according to Kimura's classification of AUPBD. RESULTS: In our study, AUPBD was associated with choledochal cyst in 28 (64%) cases. AUPBD was found only in type I and IV according to Todani's classification of choledochal cyst. There were no significant differences between the AUPBD-present group and the AUPBD-absent group in the incidence of gallstone disease, while the incidence of acute inflammation was 93% (26/28) in the AUPBD-absent group (p < 0.01). Carcinoma developed only in the AUOBD-present group (9/28, 32%) (p < 0.05). Pancreatic disorders (i.e. pancreatic stone, pancreatitis or pancreatic cancer) occurred in 12 of 28 cases in the AUPBD-present group (43%), while only in 1 of 16 cases in the AUPBD-absent group (6%) (p < 0.05). CONCLUSION: AUPBD associated with choledochal cyst may have implications not only as a possible etiologic factor but also as an important factor that may affect the clinical course, surgical planning and prognosis. In cases with choledochal cyst, we should make an effort to evaluate the presence of AUPBD.

Adolescent↗
PubMed2004

Endoscopic management of choledochal cyst.

BACKGROUND: Choledochal cyst is an uncommon anomaly of the biliary tract. Experience with this disorder, with emphasis on the role of ERCP in management, is presented. METHODS: All pertinent data for 13 patients with choledochal cyst seen between 1987 and 1998 were reviewed, retrospectively. OBSERVATIONS: Cholangitis, either alone or associated with other findings, was the main presenting feature (69.23% of patients). Bilobar Caroli's disease was the most common cyst type (46.15% of patients). ERCP was diagnostic alone in 46.15% of patients and therapeutic in 53.85%. CONCLUSIONS: Choledochal cyst is a complex condition that requires early diagnosis. Long-term management remains controversial, and guidelines for treatment require revision. Whether interventional ERCP will obviate the need for surgery or alter the malignant potential of the disorder remains uncertain.

Adolescent↗
PubMed1983

Abnormalities of the pancreatic and biliary ducts in adult patients with choledochal cysts.

A choledochal cyst in the adult patient is not just an aneurysmal dilatation of the common bile duct, but it is the most obvious manifestation of a more widespread pancreaticobiliary ductal dysplasia. Most patients have abnormalities of the pancreatic ducts and, frequently, multiple areas of dilatation involving the intrahepatic bile ducts. Of our nine patients, six had pancreatograms, five of which were distinctly abnormal. Two of the five had recurrent pancreatitis. Six patients were found to have marked stenosis of the outflow tract from the cyst. Because the mucosa lining the choledochal cyst was chronically ulcerated, anastomoses to the cyst formed strictures in seven (64%) of 11 patients, and the long-term risk of carcinoma arising in the cyst was increased. The preferred therapy was excision of the cyst with Roux-en-Y hepaticojejunostomy.

Adolescent↗
PubMed2006

Prenatal diagnosis of familial type I choledochal cyst.

Familial choledochal cysts are extremely rare. High-resolution ultrasound now allows for the antenatal diagnosis of these anomalies. After delivery, elective surgical resection should be planned; however, increases in size, deterioration of liver function, and ascending cholangitis frequently force early intervention. We report an unusual occurrence of siblings with type I choledochal cysts and review the existing literature on cause, genetics, classification, diagnosis, and management of this disease.

Child↗
PubMed1985

Fetal sonography and neonatal scintigraphy of a choledochal cyst.

A choledochal cyst, observed by ultrasound at 27 wk gestation and diagnosed at 36 wk gestation, was confirmed postpartum by [99mTc]DISIDA cholescintogram. Cystic dilatation of the common bile duct was proven by surgical excision and histological verification. Ultrasonography provides in utero diagnosis of choledochal cyst, often vital for successful management. Technetium-99m DISIDA imaging is a valuable companion for defining function of the cyst.

Adolescent↗
PubMed2003

Laparoscopic resection of type I choledochal cyst.

Type I choledochal cyst is a rare saccular or fusiform congenital dilatation of the extrahepatic biliary tract. It is usually treated by laparotomy at which the cyst is completely excised and a Roux-en-Y hepaticojejunostomy is performed to establish biliary enteric drainage. We report the laparoscopic excision and hepaticoduodenostomy of type I choledochal cyst in two girls aged 1 and 3 years. The entire procedure was performed laparoscopically using needlescopic instruments. A 7-mm telescope port was inserted at the umbilicus, a 3-mm port in the right upper quadrant, and a 6-mm port in left upper quadrant. An additional 3-mm fan-shaped liver retractor was passed through the abdominal wall without a trocar. The gallbladder and choledochal cyst was dissected and removed en bloc. The lower end of the common bile duct was closed with 5-0 polydioxanone. The duodenum was anastomosed to the common hepatic duct below the confluence of the right and left hepatic ducts. There were no intraoperative complications, and the children were asymptomatic with no episodes of cholangitis at 6 months follow-up. Pediatric surgeons trained in advanced laparoscopic techniques including intracorporeal suturing can perform laparoscopic repair of choledochal cyst safely.

Anastomosis, Surgical↗
PubMed1996

Choledochal cyst in neonates.

Common bile duct cyst, or choledochal cyst is known to be an uncommon cause of neonatal jaundice. It is usually associated with distal biliary tract obstruction and presents a clinical picture that is indistinguishable from biliary atresia. We report two cases of neonatal choledochal cyst encountered from 1992 to 1994. The infants were free from perinatal insult and presented with prolonged jaundice. In both cases, antenatal ultrasonography was unremarkable. Biochemical examinations revealed elevated levels of serum transaminases, alkaline phosphatase and direct bilirubin. Abdominal sonograms disclosed a cystic mass close to the gall-bladder. Intrahepatic duct dilation was noted in one patient. Hepatobiliary scintigraphy demonstrated accumulation of the radionuclide in the porta hepatis without subsequent intestinal activity. Cystic dilatation of the common bile duct with distal obstruction was noted during surgery. Both infants underwent cyst excision with Roux-en-Y hepaticojejunostomy and cholecystectomy. Liver biopsy in one infant showed mild proliferation of the interlobular bile duct and mild portal fibrosis. The postoperative course was uneventful. We conclude that early detection and treatment of choledochal cyst in neonates are important for preventing serious complications of biliary obstruction.

Choledochal Cyst↗
PubMedDate not supplied

Long-term results of management of type I choledochal cysts in adults.

Choledochal cysts in adults is a rare condition. The present study describes our experience with this abnormality of the biliary tree. During a 16-year period (1978-1993) eight adults with type I choledochal cyst were treated surgically in our departments. They were 4 men and 4 women with a mean age of 38.9 years (range 20-84). Symptoms, clinical findings and abnormalities in laboratory investigations included pain in all patients, history of cholangitis (n = 3), cholangitis (n = 2), acute pancreatitis (n = 1), palpable mass (n = 2), abdominal tenderness (n = 4), leucocytocis (n = 2), and increased levels of serum total bilirubin (n = 4), SGOT (n = 2), and serum alkaline phosphatase (n = 4). Diagnosis was established by intravenous cholangiography in one case, by CT-scanning in one, by ultrasonography in 5 and by intraoperative cholangiography in one. All the patients were treated surgically. Three of them underwent a Roux-en-Y choledochocystojejunostomy and one a choledochocystoduodenostomy. The other 4 patients were treated with cyst excision and Roux-en-Y hepaticojejunostomy. There were no deaths among our patients. The mean follow-up period was 6.7 years (range 1-17). So far, five episodes of mild ascending cholangitis have occurred in the patient treated with choledochocystoduodenostomy. One patient in whom a Roux-en-Y choledochocystojejunostomy was performed had 2 episodes of right upper quadrant colic pain and one episode of cholangitis. Both these patients were treated conservatively. The other 6 patients had no episodes of pain cholangitis or jaundice. In conclusion, the primary treatment of choledochal cyst type I is the excision of the cyst with Roux-en-Y hepaticojejunostomy. The Roux-en-Y choledochocystojejunostomy is indicated in cases where, for various reasons, the cyst can not be safely removed.

Adult↗
PubMed1997

Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults.

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.

Adolescent↗
PubMed2002

Choledochal cysts in adults.

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.

Adolescent↗
PubMedDate not supplied

Surgical management of choledochal cysts in adults.

BACKGROUND/AIMS: Choledochal cysts are congenital malformations of the pancreatico-biliary system. Some aspects of optimal surgical management of choledochal cysts remain controversial. The purpose of this paper is to present our series of 14 patients with choledochal cysts, analyzing surgical management and long-term results. METHODOLOGY: Between January 1975 and December 2001, 15 adult patients with choledochal cysts were treated at our Department. Sex, age, clinical symptoms, associated diseases, surgical management and postoperative morbidity and mortality were reviewed. Choledochal cysts were classified according to the Alonso-Lej classification with Todani et al.'s, modification, based on radiographic and operative findings. RESULTS: There were 15 patients, 6 males and 9 females, with an age ranging from 28 to 82 years and a mean age at the time of surgery 58.3 years. Seven patients had a solitary fusiform extrahepatic cyst (Type I), five patients had an extrahepatic supraduodenal diverticulum (Type II), one patient had a choledochocele (Type III), while two patients had a Type IVB cyst. Symptoms were vague and intermittent. Recurrent upper abdominal pain, jaundice, fever, nausea and vomiting were the most common findings, usually occurring in combination. Two patients presented with cholestatic cirrhosis. Five patients had laboratory evidence of hepatocellular dysfunction and two patients had hyperamylasemia. A variety of operations was performed such as cystoduodenostomy, cyst excision and hepaticojejunostomy, cyst excision and choledochoduodenostomy. Postoperative follow-up ranged from 30 months to 12 years in all patients except of two patients who were lost to follow-up. CONCLUSIONS: Total or partial excision of the choledochal cysts is the optimal treatment because of the lower incidence of postoperative complications and the better survival rate after the operation.

Adult↗
PubMed1989

[Congenital hepatic fibrosis associated with choledochal cyst].

The purpose of this work is to report the case of a one-year and eleven-month-old female child who presented with an asymptomatic hepatic mass; on x-ray examination a well limited round tumor was seen as well as renal cysts. A choledochal cyst was found upon laparotomy; the liver biopsy exhibited congenital hepatic fibrosis. This association is not clearly defined in the literature review made, making this perhaps the first well-documented case with this combination. We analyze the pathogenic mechanisms of both disorders and conclude that choledochal cyst is probably not related to hepatorenal fibropolycystic disease; this could be a casual association in our patient.

Choledochal Cyst↗