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.     

Radiologic approach to choledochal cysts

Choledochal cysts, being uncommon and having a nonspecific presentation, require a high index of suspicion for their diagnosis. In this series of 11 patients, the correct diagnosis was established in all using a combination of real-time ultrasound (US) scanning (9 positive) and endoscopic retrograde cholangiopancreatography (ERCP; 10 positive). Real-time US is suitable as the initial imaging modality and ERCP is complementary.     

Surgical treatment of choledochal cysts

Biliary cystic disease is uncommon in Asia and very rare in Europe and the Americas. Patients with biliary cysts may present as infants, children, or adults. When patients present as adults, they are more likely to have stones in the gallbladder, common duct, or intrahepatic ducts and to present with biliary colic, acute cholecystitis, cholangitis, or gallstone pancreatitis. With increasing age at presentation, the risks of intrahepatic strictures and stones, segmented hepatic atrophy/hypertrophy, secondary biliary cirrhosis, portal hypertension, and biliary malignancy all increase significantly. Factors to be considered when performing surgery on patients with biliary cystic disease include: (1) age, (2) presenting symptoms, (3) cyst type, (4) associated biliary stones, (5) prior biliary surgery, (6) intrahepatic strictures, (7) hepatic atrophy/hypertrophy, (8) biliary cirrhosis, (9) portal hypertension, and (10) associated biliary malignancy. In general, regardless of age, presenting symptoms, biliary stones, prior surgery or other secondary problems, surgery should include cholecystectomy and excision of extrahepatic cyst(s). With respect to the distal bile duct, the surgical principle should be excision of a portion of the intrapancreatic bile duct with care to not injure the pancreatic duct or a long common channel. Resection of the pancreatic head should be reserved for patients with an established malignancy. With respect to the intrahepatic ducts, surgery should be individualized depending on whether (1) both lobes are involved, (2) strictures and stones are present, (3) cirrhosis has developed, or (4) an associated malignancy is localized or metastatic. When the liver is not cirrhotic, hepatic parenchyma should be preserved even when strictures and stones are present. If cirrhosis is advanced, hepatic transplantation may be indicated, but this sequence of events is unusual. If a malignancy has developed, oncologic principles should be followed. Whenever possible, resection of a localized tumor including adjacent hepatic parenchyma and regional lymph nodes should be performed.     

Type IV-A choledochal cysts: a challenge

Background/Purpose

Type IV-A choledochal cysts are characterized by congenital cystic dilatation of the biliary tree extending to involve the intrahepatic biliary channels also. A single-center experience of the management of type IV-A choledochal cysts is presented.

Methods

Thirty-five out of 105 (33%) patients with choledochal cysts, who underwent surgery at a tertiary care center in northern India from January 1989 to December 2002, were found to have a type IV-A (Todani’s classification) cyst. The mean age of the patients was 24 years (range, 3 months to 60 years); 17 patients in the group were adults and 22 were females. Presenting features were abdominal pain, jaundice, cholangitis, and abdominal lump, in various combinations.

Results

Excision of the extrahepatic part of the cyst and a wide bilio-enteric anastomosis was achieved in 32 (91%) patients, while internal drainage of the cyst was necessitated in 3 patients, for technical reasons: collaterals due to portal hypertension (1 patient) and dense adhesions (2 patients). Six (17%) patients developed postoperative complications: 3 had bilio-enteric anastomosis leaks, with 2 requiring a percutaneous proximal biliary diversion; 2 had intraabdominal bleeds requiring re-exploration, and 1 had external pancreatic fistula that closed spontaneously. Follow-up information was available for 28 (80%) patients. Mean duration of follow up was 25 months (median, 12 months; range, 6 months to 9 years). Three patients required re-operation, for anastomotic stricture (n = 2) and hepatolithiasis and recurrent cholangitis (n = 1) during follow up.

Conclusions

Excision of the extrahepatic part of the cyst and drainage of the intrahepatic part by a wide hilar or subhilar anastomosis gave satisfactory results in the majority of patients with type IV-A choledochal cysts. Close long-term follow up of these patients is essential, because they are likely to present with complications related to the residual intrahepatic part of the disease.     

Diagnosis and management of choledochal cyst: 20 years of single center experience

We report the first case series from Africa and the Middle East on choledochal cyst,a disease which shows significant geographical distribution with high incidence in the Asian population.In this study,the epidemiological data of the patients are presented and analyzed.Attention was paid to diagnostic imaging and its accuracy in the diagnosis and classification of choledochal cyst.Most cases of choledochal cyst disease have type?ⅠandⅣ-A cysts according to the Todani classification system,which support the etiological theories of choledochal cyst,especially Babbitt’s theory of the anomalous pancreaticobiliary duct junction,which are clearly stated.The difficulties and hazards of surgical management and methods used to avoid operative complications are clarified.Early and late postoperative complications are also included.This study should be followed by multicenter studies throughout Egypt to help assess the incidence of choledochal cysts in one of the largest populations in Africa and the Middle East.     

Course of choledochal cysts according to the type of treatment

Abstract

Objective. To compare natural course and treatment results of various types of choledochal cysts. Material and methods. Complication rate in treated (T) and natural course in not treated (NT) patients with choledochal cysts were compared. The level of bilioenteric anastomosis after cyst resection was categorized and their post-procedure courses were analyzed. Results. A total of 204 patients (T, 174; NT, 30) were included. Patients with initial malignancies were excluded from the outcome analysis (n = 13). Of the remaining 191 patients, the mean follow-up time was 35.9 months (range 6?310.7 months). Biliary stricture was the most troublesome post-operative complication, and occurred in 10 patients (6.1%). It occurred significantly more frequently in the above hilar anastomosis group than in at or below (p-trend = 0.017), and also with older age (p-trend = 0.019). Common bile duct stone was the most frequent adverse event among NT (42.9%). Overall, there were fewer complications among treated patients (T, 17.2%; NT, 67.9%; p < 0.001). Anomalous pancreaticobiliary union (APBU) was present in 69 patients (37.5%), which was associated with higher prevalence of carcinoma (APBU-present, 13%; APBU-absent, 3.5%; p = 0.019). No malignancy developed in both T and NT groups during follow-up. Conclusions. This study suggests, that even asymptomatic choledochal cysts should be treated, not only for prevention of malignancies, but also to lower the future adverse events. Bilioenteric anastomosis at larger caliber duct is recommended for prevention of post-operative biliary strictures.     

Surgical treatment of choledochal cysts in children

Choledochal cyst is often associated with pancreatobiliary malunion, and pancreatic juice usually refluxes into the bile duct via the malunion. Various pathological conditions develop in the biliary tract, pancreas, and liver: cholangitis, biliary dilatation, biliary perforation, biliary cancer, acute pancreatitis, and/or biliary cirrhosis. The performance of cystenterostomy has recently been abandoned because of high morbidity after surgery. Accurate delineation of the biliary tree and the pancreatobiliary junction obtained by endoscopic retrograde cholangiopancreatography or operative cholangiography is necessary. Cyst excision should be performed to prevent ascending cholangitis and biliary cancer. Many types of procedures are employed in biliary reconstruction, but free drainage of bile is imperative for preventing cholangitis and stone formation. This can be achieved only by a wide anastomosis performed at the hilum after ductoplasty.     

Surgical treatment of type IV choledochal cysts

The benefit of total resection of the dilated bile duct has remained unclear. We describe here our surgical management of 13 patients with type IV choledochal cysts. All six younger patients (25–35 years old) underwent resection of the extrahepatic bile duct (EHBD) and hepaticojejunostomy (HJ), whereas three of the seven older patients (50–68 years old) underwent resection of the EHBD resection and HJ, with the remaining four older patients undergoing total resection of the dilated bile duct and removal of a pancreatobiliary maljunction (PBMJ) in the form of a S4a+S5 hepatectomy (so-called Taj Mahal) and/or pancreas head resection with second portion pancreaticoduodenectomy. No malignancies were detected in the dilated bile duct after resection in the younger patients, but cancer of the gallbladder and/or the dilated bile duct was found in two (27.5%) of the older patients. No cancers were detected during the long-term follow up (1974–2008) in those patients who underwent EHBD resection plus partial hepatectomy, but cancer developed in the remnant duct in one of the older patients who underwent EHBD resection alone. Based on our findings, we recommend that type IV choledochal cysts should be treated by total excision of the dilated bile duct, including the PBMJ, due to its frequent association with malignancy, and to prevent the development of cancer in the remnant duct and improve the long-term survival rate.     

Secondary excision of choledochal cysts after previous cyst-enterostomies

BACKGROUND/AIMS: Patients with a choledochal cyst previously treated by internal drainage should undergo secondary cyst excision. The results of such secondary excisions have not yet been reported. METHODOLOGY: Over a 27-year period, 121 patients underwent excision of a choledochal cyst at our hospitals. Of these, 14 patients underwent secondary cyst excision following internal drainage. These patients were compared retrospectively with the remaining 107 patients who underwent primary cyst excision. RESULTS: Blood loss and operative time were greater, and early and late post-operative complications were significantly more frequent in the secondary excision group. Wound infection (early complication), pancreatic stones (late complication), and hepatolithiasis (late complication) were significantly more common in the secondary excision group. Histologically, inflammation and biliary glands were more frequently seen in the resected bile ducts in the secondary excision group. CONCLUSIONS: The long-term results of secondary excision of choledochal cysts are worse than after primary excision. The frequent late complications may be related to the development of biliary glands as a consequence of cyst-enterostomy.     

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.     

Diagnosis and management of giant cell arteritis: an Asia‐Pacific perspective

Giant cell arteritis is the commonest primary vasculitis of the elderly. However, the prevalence does vary widely between populations with highest incidence amongst Northern Europeans and lowest amongst East Asians. Preliminary studies suggest that clinical manifestations may differ between different populations. Newer diagnostic approaches including ultrasound, MR angiography and PET imaging are under review. While there have been recent advances in the diagnosis of GCA particularly with regard to imaging, there is an urgent need for improvements in methods of diagnosis, treatment and requirement for screening. Glucocorticoid treatment remain the backbone of therapy. However, glucocorticoid therapy is associated with significant adverse effects. Conventional and novel immunosuppressive agents have only demonstrated modest effects in a subgroup of steroid refractory GCA due to the different arms of the immune system at play. However, recently a study of IL‐6 blockade demonstrated benefit in GCA. Newer approaches such as fast‐track pathways can also result in improvements in consequences of GCA including blindness.     

Diagnosis and treatment of congenital choledochal cyst: 20 years' experience in China

AIM To summarize the experience of diagnosis andtreatment of congenital choledochal cyst in the past 20years(1980-2000).METHODS The clinical data of 108 patients admitted from1980 to 2000 were analyzed retrospectively.RESULTS Abdominal pain,jaundice and abdominal masswere presented in most child cases.Clinical symptoms inadult cases were non-specific,resulting in delayeddiagnosis frequently.Fifty-seven patients(52.7%)hadcoexistent pancreatiobiliary disease.Carcinoma of thebillary duct occurred in 18 patients(16.6%).Ultrasonicexamination was undertaken in 94 cases,ERCP performedin 46 cases and CT in 71 cases.All of the cases werecorrectly diagnosed before operation.Abnormalpancreatobiliary duct junction was found in 39 patients.Before 1985 the diagnosis and classification of congenitalcholedochal cyst were established by ultrasonographypreoperatively and confirmed during operation,the mainprocedures were internal drainage by cyst enterostomy.After 1985,the diagnosis was established by ERCP andCT,and cystectomy with Roux-en-Y hepaticojejunostomywas the conventional procedures.In 1994,we reported anew and simplified operative procedure in order to reducethe risk of choledochal cyst malignancy.Postoperativecomplication was mainly retrograde infection of biliarytract,which could be controlled by the administration ofantibiotics,there was no perioperative mortality.CONCLUSION The concept in diagnosis and treatment ofcongenital choledochal cyst has obviously been changedgreatly.CT and ERCP were of great help in theclassification of the disease.Currently,cystectomy withRoux-en-Y hepaticojejunostomy is strongly recommendedas the choice for patients with type Ⅰ and type Ⅳ cysts.Piggyback orthotopic liver transplantation is indicated intype Ⅴ cysts(Caroli's disease)with frequently recurrentcholangitis.     

Diagnosis and treatment of hepatic cysts

Hepatic cysts are at the present time of widespread use of abdominal ultrasonography a frequent finding. The authors summarize contemporary diagnostic possibilities of liver cysts and submit variants of the therapeutic procedure in case of symptomatic liver cysts. They present their own experience with the treatment of hepatic cysts by the evaluation method combined with administration of 96% ethanol into the cyst. They compare this therapeutic procedure with the method of simple evaluation of the contents of the cyst. The method of evaluation of hepatic cysts under ultrasonographic control with subsequent administration of 96% alcohol appears to be more successful from the aspect of the long-term effect, manifested by the disappearance of clinical symptoms and non-refilling of the cyst.     

Efficacy of ERCP via the accessory papilla in children with choledochal cysts

BACKGROUND: ERCP via the major duodenal papilla in children with choledochal cyst may not clearly visualize the entire pancreatic duct and the junction of the pancreatic and biliary ducts. This may be caused by obstruction of the pancreatic duct by a dilated common bile duct. METHODS: Patients with choledochal cysts who underwent ERCP with injection of contrast medium at the major duodenal papilla were classified as either belonging to a MP group, in which the entire pancreatic duct and junction of the pancreatic and biliary ducts were visualized, or to a M/AP group, in which these structures were either partially visualized or not visualized. ERCP via the accessory papilla subsequently was performed in the M/AP group by using 0.6- or 0.8-mm metal-tip catheters. The efficacy of ERCP via the accessory papilla in children with choledochal cysts was evaluated. OBSERVATIONS: ERCP was performed in 13 patients. Seven were assigned to the MP group and 6 to the M/AP group. ERCP via the accessory papilla in the M/AP group was successful in 5 of the 6 patients; pancreas divisum was demonstrated in one, a protein plug at the main pancreatic duct in another, and the entire pancreatic duct and junction of the pancreatic and biliary ducts in the remaining 3 patients. No patient developed pancreatitis as a result of ERCP, including ERCP via the accessory papilla. CONCLUSIONS: In children with choledochal cyst, ERCP via the accessory papilla is an effective method for visualization of the detailed structure of the entire pancreatic ductal system and junction of the pancreatic and biliary ducts when ERCP via the major duodenal papilla is unsuccessful.