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.     

Biliary atresia type I cyst and choledochal cust: can we differentiate or not?

Background/purpose

It is difficult to discriminate between choledochal cust with obstructive jaundice and biliary atresia with a cyst at the porta hepatis in neonates or young infants. This review evaluates whether it is possible to differentiate between these two diseases. We here also provide an overview of our experience with type I cyst biliary atresia patients.

Methods

Among all the biliary atresia infants who we treated, the infants who were diagnosed with type I cyst biliary atresia were identified and reviewed for their management and outcome. The clinical course and management in different reports were reviewed and compared to the cases presented to our institution.

Results

Among the 220 biliary atresia cases, 11 (5 %; male/female: 4/7) were diagnosed to be type I cyst biliary atresia. Two received hepaticoenterostomy and nine received hepatic portoenteros. Three patients had severe late complications; overall, nine (81.8 %) were alive with their native liver and without jaundice.

Conclusions

Patient with choledochal cust are likely to represent larger cysts and inversely, smaller, static, anechoic cysts are more likely to represent cystic biliary atresia. However, exceptional cases were yet presented, and a definitive diagnosis may not be reached. Thus a complete differentiation between choledochal cust from type I cyst biliary atresia is yet hard to reach.     

Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst

Background/purpose

Cholangitis and intrahepatic stones occur long after total cyst excision in patients with congenital choledochal cyst (CCC). Our study aimed to characterize morphological features of intrahepatic biliary dilatation and stenosis before and after total cyst excision, based on long-term follow-up data.

Methods

Pre- and postoperative morphological features of intrahepatic biliary dilatation were determined in 63 patients with CCC.

Results

Postoperatively, hepatic ductal dilatation persisted in 50 patients and hepatic ductal dilatation with stenosis in 35 patients. Hepatic duct stenosis was seen in 35 patients: unilateral hepatic duct stenosis in 21, and bilateral stenosis in 14. Stenosis at the confluence of the right and left hepatic ducts occurred more often in the cystic type of dilatation than in the cylindrical type and was seen more often on the left side than the right. Cases with postoperative cholangitis or intrahepatic stones featured stenosis at the confluence of left and both hepatic ducts (n?=?2); and alternating dilatation and stenosis of left hepatic ducts and branches (n?=?3). However, no statistical associations were observed between the hepatic ductal stenosis and cholangitis or stone formation (P?=?0.153).

Conclusions

Cystic-type biliary dilatations persist postoperatively, frequently accompanied by ductal stenosis. Alternating dilatation and stenosis is a common morphological feature for postoperative cholangitis and stones.     

Outcomes of choledochal cysts with or without intrahepatic involvement in children after extrahepatic cyst excision and Roux-en-Y hepaticojejunostomy

Background. Type I and type IV-A choledochal cysts (CC) in Todani’s classification are the most frequent types of CC. Unlike type I cyst, in which the dilatation is confined to the extrahepatic bile duct, type IV-A affects both extra and intrahepatic ducts.Aim. To review our experience of complete cyst excision with Roux-en-Y hepaticojejunostomy for the treatment of type I and type IV-A CC in childhood, in order to better characterize these entities.Material and methods. Data was collected retrospectively from a cohort of children who underwent cyst resection for CC from 1989 to 2011 in our institution.Results. Twelve patients were submitted to surgical excision of extrahepatic cyst and hepaticojejunostomy for treatment of type I (n = 6) and type IV-A (n = 6) cysts, with a complication rate of 25% (n = 3) and no mortality. Long term follow-up was available in 92% of patients, with a median of 10 years (2-22 years). Morbidities consisted of bile leak (2 patients) and late-onset cholestasis (1 patient); two of these required anastomotic revision. The results did not reveal any significant differences between the groups regarding postoperative outcomes (P > 0.05). Preoperative intrahepatic dilatation was found to permanently vanish in 83% of patients diagnosed with type IV-A cyst after operative repair.Conclusions. Intrahepatic dilatation of type IV-A cyst in children did not adversely affect the postoperative outcome after conventional surgical repair. This operative approach was effective in the management of type I and type IV-A cysts.     

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.     

Choledochal cyst,pancreatobiliary malunion,and cancer

A review of the clinical features of biliary cancer with choledochal cyst and pancreatobiliary malunion is presented, together with a recent case report. Biliary cancer develops in about 25% of patients with choledochal cyst and pancreatobiliary malunion, and usually occurs in younger patients (usually those in their 40s) than does biliary carcinoma in the general population. The risk of malignancy in the retained cyst with internal drainage is higher than that in the primary cyst. Early excision of the retained cyst should be performed as quickly as possible, even if the patient is symptomless. Some bile acid fractions and refluxed pancreatic enzymes in bile are possibly responsible for carcinogenesis. Carcinoma generally develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic bile duct. In cystic dilatation, cancer usually occurs in the common bile duct, while in diffuse or non-dilated type, it develops in the gallbladder. Multicentric carcinomas develop in the bile duct either synchronously or metachronously. The prognosis of biliary cancer is usually dismal. However, aggressive procedures are now gaining better results than conventional approaches. The procedure of choice for choledochal cyst or malunion is to prevent the development of cancer by performing an early excision. Removal of the entire extrahepatic bile duct is necessary, even in patients with malunion and no biliary dilatation. Carcinoma rarely arises in the intrahepatic bile duct after excisional surgery, probably due to the long-standing stricture of the bile duct. Capacious anastomosis and/or ductoplasty is essential. Carcinoma may also develop in the remnant bile duct. Excision of the distal duct extending into the pancreas is also necessary.     

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.     

The risk of carcinogenesis in congenital choledochal cyst patients: an analysis of 214 cases

Background. Choledochal cysts are most common in Asian populations. In addition, the incidence of biliary tract malignancies from choledochal cysts is increasing, but the risk of carcinogenesis is still unclear.Material and methods. Clinical data from 214 congenital choledochal cyst cases from 1968 to 2013 were retrospectively analyzed.Results. Todani type I was more common (139, 65.0%) than type IVa (53, 24.8%) or type V (17, 7.9%) in these choledochal cyst patients. Biliary tract malignant tumors occurred in the gallbladder (2, 13.3%), common bile duct (10, 66.7%), and intrahepatic bile duct (3, 20%) in 15 patients (7.0%), including one patient in whom malignant transformation occurred in the intrahepatic bile duct in a type IVa patient 15 years after extrahepatic cyst resection. An age at symptom onset ≥ 60 years was a risk factor (p < 0.001), while an initial complete surgery was a protective factor for carcinogenesis (p = 0.001).Conclusions. Choledochal cysts should be removed once diagnosed because of an increased risk of malignant transformation with increasing age. Complete cyst removal is necessary for the first surgical treatment. Additional hepatectomy should be considered for type IVa choledochal cysts because cholangiocarcinoma can arise from the intrahepatic bile duct years after the extrahepatic cyst excision.     

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.     

Endoscopic Ultrasound-Guided Antegrade Treatments for Biliary Disorders in Patients with Surgically Altered Anatomy

Introduction

Endoscopic retrograde cholangiopancreatography in patients with surgically altered anatomy is challenging. Several endoscopic ultrasound (EUS)-guided biliary access techniques have been reported as effective alternatives. EUS-guided antegrade treatments (AG) have been developed more recently but have not yet been studied well.

Aims

To evaluate the feasibility and safety of EUS-AG for biliary disorders in patients with surgically altered anatomies.

Methods

We retrospectively identified all the patients who underwent EUS-AG. The left intrahepatic bile duct (IHBD) was initially punctured from the intestine followed by cholangiography, antegrade guidewire manipulation, and bougie dilation of the fistula. Either antegrade biliary stenting (ABS) or antegrade balloon dilation (ABD) was performed depending on the biliary disorders. In stone cases, the stones were antegradely pushed out using a balloon. After ABD, a nasobiliary drainage tube was placed to prevent possible bile leak and to keep an access route for any possible repeat procedures.

Results

EUS-AG was attempted in seven patients including choledocholithiasis in five, malignant biliary obstruction in one, and bilioenteric anastomosis stricture in one. EUS-AG was not performed in one patient because EUS-cholangiography did not indicate the presence of stones. In the remaining six patients, the IHBD was successfully punctured, followed by cholangiography, guidewire insertion, and bougie dilation. ABS and ABD were successfully performed in one and five patients, respectively. Antegrade procedures with ABD were repeated twice in one patient. Mild complications were observed in two patients.

Conclusions

EUS-AG for biliary disorders in patients with surgically altered anatomy is feasible. Further studies are warranted.     

Endoscopic treatment of biliary complications after right-lobe living-donor liver transplantation with duct-to-duct biliary anastomosis

Background/Purpose

The aims of this study were to characterize the features of the biliary complications that occur after right-lobe living-donor liver transplantation (RL-LDLT) with duct-to-duct biliary anastomosis, and to evaluate the efficacy of treating biliary complications endoscopically.

Methods

The records of 273 consecutive patients who underwent RL-LDLT with duct-to-duct biliary anastomosis from July 1999 through July 2005 at Kyoto University Hospital were reviewed to determine the overall incidence of postoperative biliary complications and the outcome of endoscopic repair of those complications.

Results

Biliary complications occurred in 93 (34.1%) of the patients. These complications were: 80 biliary strictures (75 anastomotic and 5 nonanastomotic) and 16 biliary leakages (5 patients with biliary leakage also had a biliary stricture); most (72%) of the anastomotic strictures were complex (i.e., fork-shaped or trident-shaped). The strictures and leakages were repaired by the endoscopic placement of multiple inside stents above the sphincter of Oddi, and by nasobiliary drainage, respectively. The procedure was successful in repairing 51 (68.0%) of the anastomotic strictures and 8 (50.0%) of the biliary leakages.

Conclusions

Endoscopic stenting of the bile ducts is efficacious in treating biliary complications related to RL-LDLT with duct-to-duct biliary anastomosis and the stenting should be attempted before surgical revision of strictures and leakages.     

Small bile duct involvement in IgG4-related sclerosing cholangitis: liver biopsy and cholangiography correlation

Background

IgG4-related sclerosing cholangitis (IgG4-SC) needs to be differentiated from primary sclerosing cholangitis (PSC). In this study, we performed a retrospective study to reveal cases in which liver needle biopsy was useful for differential diagnosis.

Methods

Nineteen patients with IgG4-SC and 22 patients with PSC were studied. All patients underwent endoscopic retrograde cholangiography and liver needle biopsy. We defined small bile duct involvement of IgG4-SC histologically as damage to the small bile duct associated with infiltration of ??10 IgG4+ plasma cells per high power field (HPF). Clinicopathological characteristics were compared between IgG4-SC patients with and without small bile duct involvement.

Results

Small bile duct involvement was observed in 5 (26%) of the patients with IgG4-SC. Patients with small bile duct involvement showed a higher incidence of intrahepatic biliary strictures on cholangiography (80 vs. 21%, p?=?0.038). Conversely, 4 of 7 (57%) patients with intrahepatic biliary strictures on cholangiography had histologically evident small duct involvement. The number of IgG4+ plasma cells was significantly correlated with the site of the most proximal stricture on cholangiograms (p?=?0.021). The number of IgG4+ plasma cells per HPF was significantly higher in IgG4-SC patients with intrahepatic biliary strictures than in those with PSC (13.4 vs. 0.4?cells/HPF, p?<?0.001).

Conclusions

Involvement of small bile ducts is more frequent in patients with intrahepatic biliary strictures on cholangiography, and liver needle biopsy is especially useful for these patients.     

Place de la fenestration dans la prise en charge des kystes biliaires simples

Introduction

Simple hepatic cysts must be differenciated from cystadenoma and from cystadenocarcinoma. The treatment is different and surgical indications are restricted. We want to enhance the superiority of fenestration in the management of simple hepatic cysts through clinical cases and review of the literature.

Observation

We report two cases of simple hepatic cyst the clinical presentation of which were pain and superinfection of the cyst. Initial diagnosis was made after ultrasonography and computed tomography scan. The first case was a recurrent cyst of segments IV-V, 3 years after a surgical drainage and was treated with aspiration, surgical drainage and alcohol sclerotherapy. Subcutaneous and intracystic abscesses occurred four months later treated with multiple percutaneous radiological drainage then with fenestration of the cyst and partial pericystectomy. The long-term outcomes was good. The second case was a huge cyst of the right lobe of the liver treated with fenestration with a good outcome.

Conclusion

The diagnosis of simple hepatic cysts is easily done at radiology. Surgery is only indicated when the cyst is symptomatic or complicated. In these two cases, fenestration of the cyst was curative and it remains the recommended technique.     

Value of prophylactic abdominal drainage in 1269 consecutive cases of elective liver resection

Background

The value of prophylactic abdominal drainage in patients undergoing hepatectomy is controversial. We carried out a retrospective study to clarify the value of this procedure.

Methods

The study subjects were 1269 consecutive patients who underwent elective hepatectomy with drain insertion for malignant tumors without associated gastrointestinal procedures or bilio-enteric anastomosis. Symptomatic abdominal fluid collections were treated by the drain salvage method, percutaneous puncture, and/or re-operation, in that order of preference.

Results

One patient died (mortality rate, 0.07%) and 7 patients had postoperative bleeding (0.6%). Bile leakage, found in 111 (8.7%) patients, subsided with retention of the drain in 78 (70%) and use of the drain salvage technique in 8 (7.2%), whereas percutaneous puncture and re-operation were required in 11 (9.9%) and 14 (12.6%). Symptomatic fluid collection, observed in 65 (5.1%) patients, was treated by the drain salvage technique in 20 (31%) patients, while puncture and re-operation were required in 25 (38%) and 20 (31%).

Conclusions

Placement of drains was effective in a considerable proportion of patients undergoing hepatectomy, with regard to reducing the frequency of development of subphrenic fluid collections and biliary fistula/biloma formation.     

A case of intrahepatic cholangiocarcinoma associated with Type IV choledochal cyst

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.     

Diagnosis and management of giant choledochal cysts: complexities compared to smaller cysts

Background

This study presents our 11-year surgical experience with choledochal cysts (CDC), focusing on the complexity in the diagnosis and management of large-size CDCs.

Methods

Medical records of 70 patients who underwent surgery for CDC between 2000 and 2011 were retrospectively reviewed. Of them, ten patients were found with cyst size >10 cm (group A), and we compared the differences in the clinical presentation, radiological diagnosis and surgical management with those with cysts ≤10 cm in size (group B).

Results

Abdominal pain and lump were noted in all group A patients, but only 67 % and 14 % in group B patients, respectively. Sixty percent of group A, but only 14 % of group B patients, had a triad of abdominal pain, mass and jaundice. Cholangitis was more common in group A patients. Most of group B patients were correctly diagnosed with ultrasound alone, but misdiagnosis occurred more frequently in group A. Magnetic resonance cholangiopancreatography correctly achieved the diagnosis in all. Operative time (5 vs. 3 h) and blood loss (300–500 vs. 50–100 mL) were more in group A in comparison to group B.

Conclusions

The clinical presentation and surgical management of giant choledochal cysts differ from their smaller counterparts. Diagnostic dilemma was common with giant CDC especially with ultrasound and CT.

     

Congenital choledochal dilatation: Classification, clinical features, and long-term results

Choledochal cyst is generally accompanied by pancreatobiliary malunion. Pancreatic juice usually refluxes into the bile duct via the malunion, and bile juice occasionally regurgitates into the pancreatic duct. As a result, various pathological conditions occur in the biliary tract, pancreas, and liver. We have revised our classification of choledochal cyst on the basis of malunion. Abdominal mass, pain, and jaundice are regarded as the triad of choledochal cysts, but symptoms are quite non-specific. In neonates and infants less than 12 months of age, choledochal cyst is usually huge and no cylindrical dilatation is observed. Vomiting and jaundice with acholic stool are often observed. No hyperamylasemia is seen, despite the presence of a malunion. Whereas children over 13 months of age tend to show diffuse dilatation, and an abdominal mass is rarely palpable. Epigastralgia with hyperamylasemia, fever, vomiting, and slight jaundice are often observed. Cyst excision is the treatment of choice to prevent the development of ascending cholangitis and cancer in the cyst. Late complications of excisional surgery include recurrent cholangitis, pancreatitis, and rarely, biliary malignancy. These complications usually develop 5 years or more after initial surgery and become the object of reoperation. Complete removal of the extrahepatic bile duct from the hepatic hilum to the intrapancreatic duct and a hilar hepaticoenterostomy with wide anastomosis after ductoplasty, including the coexisting primary stricture, are essential at the primary surgery. Provided a large anastomosis, favorable results can be obtained with either duodenostomy or Roux-Y jejunostomy. Surgical enlargement of the hilar ducts and careful lifelong follow-up should be routine in all patients who have undergone cyst excision. When ductal stricture with intrahepatic gallstones is confirmed, reoperation at an early stage is indicated to obtain a good quality of life with short hospitalization.     

Biliary reconstruction with wide-interval interrupted suture to prevent biliary complications in pediatric living-donor liver transplantation

Background/Purpose

Liver transplantation is an established therapy for children with end-stage chronic liver disease or acute liver failure. However, despite refinements of surgical techniques for liver transplantation, the incidence of biliary tract complications has remained high in recent years. Therefore, we suggest our anastomotic technique with wide-interval interrupted suture to prevent biliary complications in pediatric living-donor liver transplantation (LDLT).

Methods

Forty-nine LDLTs were performed on 49 pediatric recipients with end-stage liver disease. Biliary reconstruction was performed using a 2.5× magnifying surgical loupe, via end bile duct to side Roux-en-Y hepaticojejunostomy (n?=?47) and duct-to-duct choledochocholedochostomy (n?=?2) with an external stent. A stay suture with 6-0 absorbable materials was placed at each end of the anastomotic orifice. Two interrupted sutures of the posterior row were performed. After completion of the suture of the posterior row, an external transanastomotic stent tube was inserted into the intrahepatic bile duct and was fixed with posterior row material. Finally, two interrupted sutures of the anterior wall were performed, totaling six stitches. The transanastomotic stent tube emerging out of the blind end of the Roux-en-Y limb was covered with a round ligament and was usually left in place for 1?month after the operation.

Results

The median follow-up period was 58.0?months (range 8?C135?months). In 33 recipients, the bile duct was used to perform the reconstruction with a single lumen. In 5 cases, there were 2 bile ducts that were formed to enable a single anastomosis. In 10 cases, there were 2 separated ducts and each duct was anastomosed with the recipient jejunum. In one case, there were 3 ducts that were formed to enable two anastomoses. Twenty-two percent of the living-donor grafts required 2 biliary anastomoses. Forty-four patients (89.8%) are alive (ranging from 8?months to 11?years), and 5 patients have died. Two patients had biliary complications, an anastomotic stricture in one (2.0%) and bile leakage in one. There were no complications due to anastomotic tubes.

Conclusions

Biliary reconstruction with wide-interval interrupted suture prevents anastomotic strictures and bile leakage in pediatric LDLT.     

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.     

Partial Internal Biliary Diversion in Progressive Familial Intrahepatic Cholestasis: Introduction of a New Approach

Introduction:

Facilitation of biliary salts secretion represents the mainstay of treatment for progressive familial intrahepatic cholestasis (PFIC). The purpose of this study was to introduce a new approach for the treatment of progressive familial intrahepatic cholestasis (PFIC) to avoid ostoma.

Case Presentation:

An 11-year-old girl with the diagnosis of PFIC underwent cholecystoappendicostomy with myotomy operation. Because of anastomosis stricture, she was reoperated with cholecystojejunocolic anastomosis and intussuscepted valve surgery. She was followed for 9 months. Despite disappointing outcomes of internal drainage with cholecystoappendicostomy, results of cholecystojejunocolic anastomosis with intussuscepted valve surgery were promising.

Discussion:

The cholecystojejunocolic anastomosis with intussuscepted valve surgery could be considered as a forthcoming approach in the treatment of intrahepatic cholestasis.