囊肿切除肝总管空肠Roux-en-Y吻合术治疗先天性胆管囊肿

目的 观察囊肿切除肝总管空肠Roux-en-Y吻合术治疗小儿先天性胆管囊肿的疗效。方法 采用囊肿切除肝总管空肠Roux-en-Y吻合术治疗小儿先天性胆管囊肿56例。结果 56例患儿手术全部成功。术后患儿胆管囊肿症状消失，肝胆系统结构和功能均恢复正常。术后并发胆道感染1例，腹膜炎1例，肺炎3例，无胆瘘和伤口感染者，无死亡病例。结论 囊肿切除肝总管空肠Roux-en-Y吻合术是治疗小儿先天性胆管囊肿的有效术式。     

Choledochal cysts in adults and their complications

BackgroundDespite refinements in the management of choledochal cysts in children, an increasing number of patients present with ongoing symptoms in adult life. The aim of this study was to review the management of adult patients with choledochal cysts in a tertiary referral centre.MethodA retrospective review was carried out of all adult patients presenting with choledochal cysts to this department between 1992 and 2000. Patient records were reviewed and detailed analyses were made of the clinical presentation, radiological and biochemical findings, anatomical anomalies, management, complications and outcomes.ResultsOf 16 patients (12 women and 4 men; median age 23 years), 8 had undergone previous upper gastrointestinal operations before referral, including 5 who had had previous cyst drainage procedures. All patients underwent elective complete cyst excision with Roux-en-Y hepaticojejunostomy. There were no operative deaths and there was a low early postoperative morbidity rate (25%). There was no evidence of biliary malignancy in any cyst. During a median postoperative follow-up of 44 months, five patients (31%) continued to experience cholangitis and two of these required additional revisional procedures, but are now symptom-free.ConclusionPatients with choledochal cysts should be referred to specialised tertiary surgical units. Total choledochal cyst excision with Roux-en-Y hepaticojejunostomy is the treatment of choice. Patients with previous inadequate cyst excisional procedures should undergo revisional surgery, to reduce recurrent symptoms and the risk of developing cholangiocarcinoma.     

先天性胆总管扩张症38例分析

目的 研究胆总管扩张症的正确诊断与治疗方法。方法 回顾性分析1985年1月至2004年1月我科手术治疗的38例先天性胆总管扩张症患者的资料，术后平均随访50个月。结果 行胆总管十二指肠吻合术2例，胆总管空肠Roux—Y吻合术9例，行胆总管扩张部切除加肝总管空肠Roux—Y吻合术27例；术后胆瘘3例，胰瘘并腹腔出血各1例，无手术死亡。结论 胆总管扩张部切除加胆肠Roux—Y吻合是本病的主要治疗方法，疗效好，局限性肝内胆管扩张可行部分肝叶切除术。     

Management of a ruptured bile duct cyst

A case of a ruptured bile duct cyst in a 25-year-old male patient is presented. The initial management of the clinical presentation of acute abdomen consisted of an exploratory laparotomy and a T-tube cystostomy of a choledochal cyst. Two months later, he was admitted to our surgical department. Preoperative evaluation showed a type IV-A choledochal cyst. The patient underwent excision of the choledochal cyst, cholecystectomy, and the construction of a Roux-en-Y end-to-side hepaticojejunostomy.     

Endoscopic biliary manometry in patients with suspected sphincter of Oddi dysfunction and in patients with cystic dilatation of the bile ducts

We studied the motility of the sphincter of Oddi in 12 patients with suspected sphincter of Oddi dysfunction, in four patients with cystic dilatation of the bile ducts (two Caroli's cases and two fusiform choledochal cyst cases), and in 33 patients with retained common duct stones. In these last 33 patients, the motor activity of the sphincter of Oddi was similar to that recorded in nine control subjects without pancreatic or biliary diseases. In the suspected Oddi dysfunction cases, both the basal sphincteric pressure and the frequency of the phasic contractions were significantly elevated (P<0.001). Patients with biliary cystic dilatation showed an increased basal pressure, but the frequency of the contractions was elevated in only those with choledochal cysts and the amplitude in only one of the two patients with Caroli's disease. Motor disorders of the sphincter of Oddi provide a basis for an alternative etiopathogenesis of cystic disease of the biliary system and a possible explanation for pain and dilatation of the bile duct in patients with suspected sphincter of Oddi dysfunction.     

Extrahepatic cyst excision and partial hepatectomy for Todani type IV-A cysts

Background

Extrahepatic cyst excision and Roux-en-Y hepaticojejunostomy is not satisfactory in many patients with complex Todani type IV-A choledochal cysts.

Aims

To report the results of combined extrahepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy for type IV-A choledochal cysts.

Methods

The records of patients who received extrahepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy for type IV-A choledochal cysts from January 2002 to December 2011 were retrospectively reviewed, and surgical outcomes analysed.

Results

59 patients (30.5% males; mean age, 43.2 ± 18.4 years) were included. Radical excision of cystically dilated bile ducts was achieved in 53 patients (89.8%). Bile leakage, delayed wound healing, and abdominal infection occurred in 5 (8.47%), 7 (11.86%), and 3 (5.08%) patients, respectively. Forty-nine patients (83.1%) were followed for an average of 42.6 ± 15.3 months. During the follow-up, 6 patients (12.2%) experienced recurrent cholangitis. Long-term biliary function was excellent in 33 (67.4%), good in 9 (18.4%), fair in 5 (10.2%), and poor in 2 (4.1%) patients.

Conclusion

Combined extra-hepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy is effective for the treatment of complex Todani type IV-A choledochal cysts with substantial intrahepatic bile duct involvement and hilar bile duct stenosis.     

Congenital choledochal cyst: Occurrence of primary intrahepatic stones and prevention of carcinogenesis

Intrahepatic biliary stones and carcinogenesis are two major complications seen after corrective surgery for congenital choledochal cyst. The incidence of these two complications was reviewed in patients seen at out-patient clinics at a mean follow-up time of 13 years and 6 months. Intrahepatic biliary stones developed in 5 of 56 patients who had undergone a standard excisional operation; in 4 of the 5 patients, the stones were found in the preexisting intrahepatic biliary dilatation (with or without a downstream stenosis), 7–15 years postoperatively. Biliary stricture with dilatation is a putative cause of intrahepatic bile duct stones, and this hypothesis was supported here, since stones developed in all 3 of the patients who had the peculiar configuration of intrahepatic biliary dilatation and stenosis. Biliary carcinoma was not observed in any of the 56 patients who had undergone standard excision of a choledochal cyst followed by hepatico-jejunostomy, but developed in 1 of 12 patients in whom complete excisional resection of the dilated extrahepatic bile duct had not been performed. The occurrence of carcinoma in choledochal cysts is reviewed.     

Case Report: Giant choledochal cyst

We report an adult female with a rare giant choledochal cyst. The patient presented following a normal pregnancy with the classical triad of an abdominal mass associated with jaundice and right upper quadrant abdominal pain. The cyst was excised using an intramural technique and biliary reconstruction achieved with a Roux-en-Y hepaticojejunostomy. Our patient has remained well with no evidence of malignancy over a 12 year review period. The aetiology and current management of this condition are discussed.     

Computed tomography virtual endoscopy with angiographic imaging for the treatment of type IV-A choledochal cyst

Type IV-A choledochal cysts (CCs) are a congenital biliary anomaly which involve dilatation of the extrahepatic and intrahepatic bile ducts. We present the case of a 30-year-old woman with type IV-A CC, on whom three-dimensional computed tomography (3D CT) and virtual endoscopy were performed. 3D CT revealed partial dilatation in the posterior branch of the intrahepatic bile duct and a relative stricture between it and the extrahepatic bile duct. Virtual endoscopy showed that this stricture was membrane-like and separated from the surrounding blood vessels. Based on these image findings, complete cyst resection, bile duct plasty for the stricture, and hepaticojejunostomy were safely performed. To the best of our knowledge, there are no reports of imaging by virtual endoscopy of the biliary tract which show the surrounding blood vessels running along the bile duct.     

Choledochal cysts in pregnancy： Case management and literature review

AIM: To evaluate the diagnosis, management principles and long-term results of congenital choledochal cysts in pregnancy. METHODS: Three adult patients were diagnosed as choledochal cysts in pregnancy from 1986 to 1989 and their long-term results were evaluated. RESULTS: The first patient had a Roux-en-Y cysto-jejunostomy with T-tube external drainage and died of septic shock and multi-organ failure 25 d after operation. In the second patient, 4 wk after percutaneous trans-choledochal cyst was drained externally with a catheter under US guidance, four weeks later the patient delivered vaginally, and had a cysto-jejunostomy 3 mo after delivery, and lived well without any complications for 15 years after operation. The third patient received Roux-en-Y cysto-jejunostomy after a vertex delivery by induced labor at 28 wk gestation, and demonstrated repetitively intermittent retrograde cholangitis within 10 years, and then died of well-differentiated congenital cholangioadenocarcinoma one month after re-operation with exploratory biopsy at the age of 36. CONCLUSION: More conservative approaches such as external drainage of choledochal cyst should be considered for pregnant patients with high risk, complete excision of choledochal cyst during hepaticojejunostomy or modified hepaticojejunostomy is highly recommended at the optimal time.     

Bile duct cancer developed after cyst excision for choledochal cyst

Oncogenesis after cyst excision for choledochal cyst and suitable surgical procedures for this operation are discussed. The clinical data of 23 patients with cancer of the biliary tree after excision of choledochal cyst reported in the English-language and Japanese literature were reviewed, and data for 1353 Japanese patients with choledochal cyst and/or pancreaticobiliary malunion were analyzed. In the 23 patients reported in the literature, age at cyst excision ranged from 1 to 55 years (average, 23.0 ± 13.7 years), and cancers were detected at age 18—60 years (average, 32.1 ± 12.2 years), with intervals between cyst excision and cancer detection of 1—19 years (average, 9.0 ± 5.5 years). Sites of cancer development were: intrahepatic, six; anastomotic, eight; hepatic side residual cyst, three; and the intrapancreatic duct, six. In the Japanese patients with choledochal cyst and/or pancreaticobiliary malunion, the incidence of cancer associated with primary choledochal cyst and/or pancreaticobiliary malunion was 16.2% (219/1353). The incidence of cancer development after cyst excision in this population, of whom 1291/1353 underwent surgery, was assumed to be 0.7%. Nearly half of the 23 patients in the literature had undergone inadequate cyst excision. Oncogenesis of cancers after cyst excision is possibly different from that of choledochal cysts.     

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.     

Choledochal cysts: analysis of disease pattern and optimal treatment in adult and paediatric patients

Background. Choledochal cysts are dilations of the biliary tree. Although commonly reported in Asian populations, the incidence outside of Asia is as low as 1:150 000. The largest series of patients with choledochal cyst disease outside of Asia is this one, studying 70 patients treated in Vancouver between 1971 and 2003. Patients and methods. This was a retrospective chart review. Results. In all, 19 paediatric and 51 adult patients were evaluated; 21% of paediatric and 25% of adult patients were Asian. All paediatric patients had type I or IV cysts, whereas adult patients represented the different subtypes. Abdominal pain was the presenting symptom in 79% of children and 88% of adults, vomiting was present in 42% of children and 63% of adults and jaundice was seen in 31.5% of children and 39% of adults. Ultrasound was used in 94.7% of children, and ERCP in 80% of adults. In all, 84% of paediatric patients, 100% of adult patients with type I cysts and 85.7% of adult patients with type IV cysts received complete cyst excision and Roux-en-Y hepaticojejunostomy. Complications in both groups were low. Conclusions. Although Vancouver does have a large Asian population, this does not explain how common choledochal cysts are in this city. Alhough some authors argue that paediatric and adult disease are caused by different aetiologies, presentation patterns in our study between the two groups were very similar. We recommend complete cyst excision and Roux-en-Y hepaticojejunostomy as the surgery of choice, and advocate early surgery after diagnosis to promote ease of surgery and prevention of future complications.     

CASE OF A CLOSED CHOLEDOCHOCELE WITH BILIARY AND PANCREATIC DRAINAGE VIA THE ACCESSORY DUCT CAUSING ACUTE PANCREATITIS

Type III choledochal cysts, also called choledochoceles, are rare cystic dilatations of the terminal biliary tree. We report the case of a ‘closed choledochocele’, in which the bile and ventral pancreatic ducts emptied into the cyst, but because there was no outlet into the duodenum, drainage was retrograde via the accessory duct. This type of choledochocele variant has never previously been described in the medical literature.     

Assessment of a postoperative anastomotic stricture following correction surgery of a type IVa choledochal cyst using Gd-EOB-DTPA-enhanced magnetic resonance cholangiography

Choledochal cyst is a relatively uncommon disease which is characterized by congenital dilatation of the intra and/or extrahepatic part of the biliary tree. Type IVa choledochal cysts are managed surgically through total excision of the entire extrahepatic part of the abnormal bile ducts and a simultaneous hepaticoenterostomy. Postoperative anastomotic stricture after excision of choledochal cysts and hepaticojejunostomy is a well-known late complication. We report a case of a 17-year-old female in whom gadoxetic acid-enhanced magnetic resonance cholangiography assisted in the evaluation of a biliary stricture following bile duct procedures after choledochal cyst correction surgery.     

Familial occurrence of congenital bile duct cysts

Congenital bile duct cysts are now a well-documented anomaly of the biliary tree, and have become more common in Japan. Familial occurrence of congenital bile duct cysts, however, is extremely rare, with only six reported cases in the literature. We report a familial pattern of congenital bile duct cysts in a mother and her daughter. A 33-year-old female was admitted for evaluation of right upper quadrant abdominal pain and fever 6 days after an uneventful delivery of her second child. A com- puted tomography (CT) and ultrasound scan (US) revealed an obstructed biliary tract. Percutaneous transhepatic biliary drainage was then performed, and a cholangiogram revealed a Scholtz type B choledochocele without an anomalous connection of the pancreaticobiliary ducts. Endoscopic US demonstrated that the choledochocele was associated with a stone in the cyst. A pylorus-preserving pancreatoduodenal resection was performed, and a histological study revealed that the choledochocele was lined by biliary mucosa without evidence of malignancy. The newborn infant had an abdominal tumour. An US and CT revealed a congenital bile duct cyst. An operation was performed and the intraoperative cholangiogram showed an Alonso-Lej type I congenital bile duct cyst with an anomalous connection of the pancreaticobiliary ducts. Whether congenital bile duct cysts are hereditary remains to be elucidated.     

Endoscopic biliary drainage for children with persistent or exacerbated symptoms of choledochal cysts

Background

Symptoms of choledochal cysts sometimes persist or become exacerbated. As preoperative management for patients with these cysts, we prospectively employed endoscopic drainage, based on the theory that protein plugs cause symptoms by obstructing the pancreatobiliary ducts.

Methods

Children with choledochal cysts underwent endoscopic retrograde cholangiopancreatography (ERCP). When ERCP showed compaction with filling defects in patients with persistent or worsening symptoms (study patients), the placement of a short biliary stent tube was attempted for drainage. The clinical and ERCP findings of the study patients were compared with those of patients who were asymptomatic at ERCP (asymptomatic patients).

Results

There were 13 study patients (median age 2.9 years) and 41 asymptomatic patients (4.7 years) enrolled in the study between August 2005 and February 2011. Study patients more frequently had jaundice and elevated transaminase levels. ERCP showed that all study patients had obstruction or compacted filling defects in the common channel or the narrow segment distal to the cyst. Insertion of a stent tube was successful in 11 patients. Symptoms were relieved soon after biliary drainage. Surgery revealed that the obstructing materials were protein plugs, except in one case, which involved fatty acid calcium stones.

Conclusions

These results support the protein plug theory. Endoscopic short-tube stenting is adequate and effective as preoperative management.     

Embryonal rhabdomyosarcoma of the common bile duct mimicking choledochal cyst

Embryonal rhabdomyosarcoma of the common bile duct is a very rare malignancy of childhood. The radiological appearance of the lesion is similar to that of congenital choledochal cyst if there is no local invasion to the adjacent tissues. The authors present a case of embryonal rhabdomyosarcoma of the common bile duct which was considered to be a congenital choledochal cyst preoperatively, and they discuss this very rare childhood tumor, with a brief survey of the literature. In conclusion, it is important to know that this rare tumor can simulate congenital choledochal cyst; it should be considered in the differential diagnosis of obstructive jaundice in children.     

Endoscopic diagnosis of pancreaticobiliary maljunction

Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a junction of the pancreatic and bile ducts located outside the duodenal wall, usually forming a markedly long common channel. As the action of the sphincter of Oddi does not functionally affect the junction in PBM patients, continuous pancreatobiliary reflux occurs, resulting in a high incidence of biliary cancer. PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst) and PBM without biliary dilatation (maximal diameter of the bile duct ≤ 10 mm). The treatment of choice for PBM is prophylactic surgery before malignant changes can take place. Endoscopic retrograde cholangiopancreatography (ERC P) is the most effective examination method for close obs ervation of the pattern of the junction site. When the communication between the pancreatic and bile ducts is maintained, despite contraction of the sphi ncter on ERCP, PBM is diagnosed. In these pat ients, levels of pancreatic enzymes in the bile are gene rally elevated, due to continuous pancreatobiliary reflux via a long common channel. Magnetic resonance cholangiopancreatography and 3D-computed tomography can diagnose PBM, based on findings of an anomalous union between the common bile duct and the pancreatic duct, in addition to a long common channel. Endoscopic ultrasonography and intraductal ultra sonography can demonstrate the junction outside the duodenal wall, and are useful for the diagnosis of asso ciated biliary cancer. Gallbladder wall thickness on ultra so nography can be a screening test for PBM.     

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.