Significance of the anomalous arrangement of the pancreaticobiliary duct in the etiology of biliary atresia.

BACKGROUND/PURPOSE: The anomalous arrangement of the pancreaticobiliary duct (AAPBD) is one theory used to explain the etiology of biliary atresia. We investigated whether AAPBD could be involved and evaluated its significance for the etiology of biliary atresia. MATERIALS AND METHODS: Of 43 patients with biliary atresia, the area between the common bile duct and the duodenum could be visualized by operative cholangiogram in 5 patients with an uncorrectable type of biliary atresia. Three of the 5 showed an anomalous arrangement of the pancreaticobiliary duct. In these 3 patients, the type of anomalous arrangement of the pancreaticobiliary duct and the length of the common channel were studied by operative cholangiogram. Histological findings of the gallbladder and the common bile duct were examined in addition to the measurement of the serum amylase levels. RESULTS: All 3 patients showed AAPBD with the P-C type of pancreaticobiliary junction. The length of the common channel ranged from 7 mm to 12 mm. Two of the 3 cases did not show an elevated serum amylase level. Epithelial hyperplasia of the gallbladder was observed in one patient, while the other two showed no hyperplasia. Inflammatory changes in the mucosa of the gallbladder and the common bile duct were not remarkable in these 3 patients. CONCLUSIONS: From these results it seems that AAPBD in biliary atresia might not be an etiological factor for atresia of the extrahepatic bile duct, but might be an associated anomaly in biliary atresia. Other factors should be examined to clarify the etiological factor leading to lumenal obstruction of the extrahepatic bile duct.     

Type I biliary atresia without extrahepatic biliary cyst

Currently, magnetic resonance cholangiography (MRC) is used for the differentiation of biliary atresia (BA) from other causes of infantile cholestasis. The authors present a case of type I BA without an extrahepatic biliary cyst in a 2-month-old girl. MRC clearly visualized the patency of the gallbladder, cystic duct, and hepatic ducts with disappearance of the common bile duct. Intraoperative cholangiography demonstrated a cloudy appearance of the intrahepatic bile ducts, confirming the diagnosis of type I BA. We believe that this is the first reported case of type I BA without an extrahepatic biliary cyst diagnosed by MRC.     

Xanthogranulomatous cholecystitis in a 10-year-old boy

A 10-year-old boy presented with a 5-month history of recurrent abdominal pain, fever, and jaundice. Ultrasound examination revealed a thick-walled gallbladder and dilatation of the biliary tree. Endoscopic retrograde cholangiography showed irregular common bile duct dilatation. After cholecystectomy for acalculous cholecystitis, histological examination of the operative specimen revealed the features of xanthogranulomatous cholecystitis. The abnormalities observed in the bile duct were secondary. This unusual destructive inflammatory process has previously only been described in adults.     

Factors of biliary carcinogenesis in choledochal cysts.

The long-term complications of choledochal cysts include malignant degeneration of the epithelial lining of the biliary tract. Possible factors leading to malignant degeneration were examined by measuring amylase levels in the biliary tract, intraoperative biliary manometry and a histopathological study. 27 patients, 1 to 13 years of age, with choledochal cysts, were studied over a 13-year period. Amylase levels in the bile of choledochal cysts and the gallbladder were measured. The pressure profile of the biliary tract was recorded as the probe was withdrawn from the sphincter of Oddi (SO) to the distal end of the common bile duct. Hyperplasia of the gallbladder mucosa and malignancy of the biliary tract were investigated. Patients with high levels of biliary amylase had higher pressure differences between the SO and the duodenum than those with low levels of biliary amylase. The incidence of mucosal hyperplasia of the gallbladder mucosa was significantly higher in the fusiform type than in the cystic type. Adenocarcinoma in a cystic choledochal dilatation was found in a 12-year-old girl with high amylase levels. The cause of regurgitation of pancreatic juice into the biliary system might be due to a high pressure difference between the SO and the duodenum in addition to the lack of sphincter function at the abnormal junction of the pancreaticobiliary ductal system. Early diagnosis and surgical treatment to prevent the regurgitation leading to hyperplasia and malignancy of the biliary tract are important for children with choledochal cysts.     

HEPATIC HETEROTOPIAS IN THE JEJUNUM: A Case Study over Time Showing Progressive Degenerative Changes

Multiple foci of heterotopic liver in the jejunum were sequentially discovered in an infant boy at the ages of 1 day, 2 months, and 4 months. This is the second reported case of jejunal heterotopic liver, a rare entity in any site. Progressive histological changes indicative of biliary duct obstruction were observed in the hepatic heterotopias, which demonstrated no connections to the main body of the liver or biliary tree.     

Cholestatic form of viral hepatitis and thickening of the walls of the intrahepatic bile ducts

A biphasic acute hepatitis A is reported in a child. The cholestatic form of the second episode was associated with an ultrasonographic thickening of the gallbladder and intrahepatic bile duct walls. A possible relationship between the abnormal US appearance of intrahepatic bile ducts and cholestasis is discussed.     

Porcelain gallbladder with extrahepatic bile duct obstruction in a child

An extrahepatic bile duct obstruction was diagnosed in a 13-year-old boy presenting with pruritus, abdominal pain and jaundice. Several weeks after sphincterotomy and biliary stenting via endoscopic retrograde cholangiopancreaticography which relieved the obstruction, the patient was operated on. Severe fibrosis encased the extrahepatic biliary tract, so only cholecystectomy was performed because planned hepaticojejunoanastomosis could jeopardize the vascular supply to the liver. Histopathology showed calcification of the gallbladder wall and chronic fibroproliferative changes in the surrounding tissue. The stricture of extrahepatic biliary duct resolved after 3 years of repeated replacement of stents. The stenting was thereafter terminated. In the following 3 years no dilation of intrahepatic bile ducts and no laboratory signs of cholestasis recurred and the now 19-year-old boy is doing well. Neither a case of porcelain gallbladder with extrahepatic bile duct obstruction in a child nor a successful treatment of the obstruction by long-term stenting has been described in the literature yet.     

Granular cell tumor of the biliary system

Granular cell tumors (GCT) are rare benign neoplasms of Schwann cell origin which have been found in virtually every location in the body. Their location in the biliary system is unusual, and typically occurs in black females during the fourth decade of life. Forty-eight cases have been reported since 1952. We present two patients treated for obstructive jaundice caused by GCT of the extrahepatic biliary tree. The literature on biliary GCT is reviewed and their management is outlined. Local excision with Roux-y-hepaticojejunostomy was performed in one patient with GCT obstructing the common bile duct at the level of the cystic duct. Pancreaticoduodenectomy was performed on one patient for GCT of the common bile duct involving the ampulla and adjacent pancreas. GCT of the extrahepatic biliary system are rare but should be considered in black females who present with obstructive jaundice in the fourth decade of life. Local excision with cholecystectomy and reconstruction of the biliary remnant is indicated. Primary biliary anastomosis is desired but choledochojejunostomy or hepaticojejunostomy may be necessary. Rarely, pancreaticoduodenectomy may be indicated in GCT of the distal common bile duct. © 1994 Wiley-Liss, Inc.     

Pre-operative ultrasonographic diagnosis of biliary atresia—with reference to the presence or absence of the extrahepatic bile duct

Our aim was to evaluate the efficacy of ultrasonographic (US) examination in the pre-operative diagnosis of biliary atresia (BA) with special reference to the presence or absence of extrahepatic bile duct. Thirty consecutive neonates and infants aged 8 to 169 days (mean: 62 days) suspected of having biliary atresia were examined pre-operatively in real time B-mode ultrasonography. We used a 5 or 7.5 MHz probe of micro convex type. Patients were fasted and sedatives administered. When the common bile duct was absent, we considered it a positive finding for BA diagnosis; if not, it was considered a negative finding. A definitive diagnosis of BA was confirmed at surgery by gross morphology or intra-operative cholangiography. US findings had a sensitivity of 83% (19 of 23 BA patients), a specificity of 71% (5% of 7 non BA patients) and an accuracy rate of 80%. The positive predictive value was 90% (19 of 21), while the negative predictive value was 56% (5 of 9). There were four false-negative cases. Two were BA cases with patent distal common bile duct, one was BA in which the hepatic artery was determined to be the common bile duct, and the other was a subtype of extrahepatic bile duct dilatation (the so-called, "correctable type"). We employed US criteria for visualization of the extrahepatic bile duct for pre-operative diagnosis of BA. US examination referring to the presence or absence of the extrahepatic bile duct is an effective and useful method for clinical survey.     

Hepaticoantrostomy

A new method of biliary drainage after resection of a choledochal cyst ist described. The steps leading to the biliary reconstruction are: (1) cholangiography through the gallbladder; (2) selective proximal gastric vagotomy; (3) excision of the gallbladder and abnormal common bile duct; (4) transection of the stomach between antrum and body; (5) inverting sutures of the lesser curvature of the antrum; (6) end-to-end anastomosis between antrum and hepatic ducts; (7) inverting sutures at the lesser curvature of the proximal stomach; and (8) end-to-side anastomosis between stomach and descending duodenum. Correspondence to: H. Sauer     

A case of biliary stricture caused by cavernous transformation of the portal vein

A case is reported of extrahepatic obstruction of the portal vein with the unusual complication of obstructive jaundice that resulted from compression of the common bile duct caused by cavernous transformation of the varicose network. It is surgically impossible to repair choledochal stenosis resulting from enlarged varices in the vicinity of the common bile duct. In this case, internal drainage from the biliary duct to the duodenum using a silastic tube and a reservoir implanted in the subcutaneous space successfully eliminated the jaundice.     

Neonatal sepsis presenting as a choledochal cyst

We report a case of a neonate who presented with Klebsiella sepsis and a fusiform dilation of the common bile duct on ultrasound suspected to be a choledochal cyst type Ia. In addition, a biliary sludge was noted in the gallbladder. All findings resolved with resolution of the sepsis. We discuss the case and review the literature regarding the association of sepsis with cholestasis and biliary sludge. CONCLUSIONS: we suggest that in any case of sepsis presenting as a choledochal cyst, it is essential to repeat the ultrasound after complete recovery before a surgery is planned.     

胆道发育不良患儿的临床病理特点回顾

目的 探讨胆道发育不良患儿的临床表现、病理特点、诊断、治疗及预后,提高对该病的认识.方法 回顾性分析天津市儿童医院外科2010年6月至2016年11月收治的5例胆道发育不良患儿的发病情况、治疗过程,并通过复习国内外文献对胆道发育不良的疾病特点进行总结.结果 5例胆道发育不良患儿均因生后不久出现皮肤、巩膜黄染,内科治疗病情无改善入外科治疗;肝功能检查见胆红素水平升高,以直接胆红素升高为主,伴肝酶不同程度增高;超声检查示胆囊干瘪未充盈或胆囊腔狭小,胆总管显示不清.5例患儿行手术探查,术中造影显示肝内、外胆道通畅,管腔纤细;病理检查见部分汇管区中小叶间胆管缺失,小叶间胆管/汇管区＜0.5,诊断为胆道发育不良,予留置胆囊引流管,术后抗炎、补液、保肝及对症治疗.经2个月～6年门诊随访,5例患儿均存活至今,其中3例黄疸清除,1例皮肤仍黄染并伴有瘙痒,1例未退黄.结论 术前检查提示梗阻性黄疸患儿,术中造影提示胆道纤细,结合病理检查证实小叶间胆管/汇管区比例＜0.5,可以明确胆道发育不良的诊断.     

Unusual gallbladder findings in two brothers with metachromatic leukodystrophy

Characteristic biliary tree abnormalities in metachromatic leukodystrophy (MLD) include gallbladder polyposis and haemobilia. We report two brothers with MLD, who presented with uncommon biliary complications. One presented with gastric outlet obstruction secondary to gallbladder enlargement, which was treated by percutaneous aspiration. He later developed gallbladder carcinoma with liver metastases. His brother demonstrated US findings consistent with gallstones. Received: 20 August 1997 Accepted: 23 February 1998     

Successful late Kasai portoenterostomy.

This article describes an infant with extrahepatic biliary atresia who underwent a successful hepatoportoenterostomy at age 132 days in spite of a delay in diagnosis. Contributing to the delay in diagnosis of this case were the lack of appreciation of alcoholic stools, the presence of a gallbladder and distal common bile duct on ultrasound examination, and insufficient biopsy material for interpretation. This case emphasizes the variability in presentation and potential difficulties in diagnosis and the need for intraoperative cholangiogram and exploratory laparotomy in such cases. An approach to the patient with extrahepatic biliary atresia who is diagnosed late is discussed. A successful outcome can be achieved for such patients.     

Applications of nuclear magnetic resonance in paediatric surgery: magnetic resonance cholangiopancreatography and new sequences

The aim of the study was to evaluate fast magnetic resonance cholangio-pancreatography (MRCP) sequences as an alternative and safe investigation method for neonatal and children's pancreaticobiliary diseases. Between January 2000 and December 2000, five children (age: 1 month 14 years; mean: 7 years) affected by pancreaticobiliary diseases or already operated for biliary pathologies were studied. Patients were evaluated by 1.5 T magnet single shot T2-weighted sequences (1 image per s, TR = infinite, TE = 150-180 ms). T1-weighted conventional sequences were obtained to study parenchymal tissue. No patient needed general anaesthetic. Only in one case was sedation necessary. Fast MRCP sequences provided very precise information on biliary tract anatomy. They revealed the intra and extrahepatic bile ducts, the gallbladder, the common bile duct and the bilio-pancreatic junction in all cases investigated. MRCP allowed us to evaluate Roux-en-Y type bilio-enteric anastomosis as accurately as percutaneous transhepatic cholangiography (PTC). In addition MRCP was the only reliable study in evaluating Roux-en-Y type anastomosis where ultrasonography (US) and endoscopic retrograde cholangiography (ERCP) could not be used. In conclusion MRCP is an accurate and non-invasive method with which to investigate the anatomy of the pancreaticobiliary tract in children. It could become the investigation of choice after US in the case of biliary and pancreatic diseases.     

Hitching the gallbladder in laparoscopic-assisted cholecysto-cholangiography: a simple technique

Neonatal cholestatic disorder in the late neonatal period requires often cholangiography to differentiate between biliary atresia and other causes of prolonged neonatal jaundice. A simplified method of a laparoscopic-assisted cholecysto-cholangiography is presented. Retrospective chart review was conducted of all patients who from May 2002 to April 2012 underwent a laparoscopic-assisted cholecysto-cholangiography with routine fixation of the fundus of the gallbladder to the lateral aspect of the abdominal wall. A total of 18 infants (8 boys) aged 41–104 (median 64) days underwent laparoscopic-assisted cholecysto-cholangiography for prolonged jaundice. The technique identified ten cases of a patent bile duct system and eight biliary atresias. (Thirty-two cases of suspected biliary atresia were confirmed by laparoscopy alone.) Two cases required suturing of a bile leak at the puncture site. Hitching the gallbladder to the lateral abdominal wall is a simple method allowing an optimal radiographic assessment of the extra- and intra-hepatic bile duct anatomy.     

Hepatic duct stone associated with chlamydia sepsis: a rare condition in childhood

Stone formation in the biliary system is a rare condition in infants. A few cases of bile stones in the biliary tree have been reported with underlying predisposing factors, such as sepsis and antibiotic usage. This article describes a surgically treated 16-week-old infant with recurrent cholangitis who had a bile stone in the hepatic duct after chlamydia sepsis. Accepted: 4 February 1997     

Imaging findings in 75 pediatric patients with pancreaticobiliary maljunction: a retrospective case study

Background

Pancreaticobiliary maljunction (PBM) is often associated with congenital choledochal cyst, protein plugs and pancreatitis. Early diagnosis and timely treatment largely depend on imaging. We assessed a series of PBM in children, comparing imaging procedure with histological and pathological findings with regard to diagnosis.

Methods

A retrospective analysis was conducted in 75 pediatric patients with PBM. PBM was defined as common channel at >5 mm. Two radiologists assess the shape of the bile duct and gallbladder, pancreatitis, surgical pathology, symptom profiles, operative notes and pathological records were compared with the imaging findings.

Results

Dilatation of the bile duct was detected in 45 subjects out of the 46 subjects who underwent computed tomography (CT) and nine was diagnosis as PBM. Forty out of 41 subjects were revealed bile duct dilatation in ultrasonography (US). Bile duct dilatation was seen in 59 out of 60 subjects receiving magnetic resonance cholangiopancreatography (MRCP) and 39 were diagnosed as PBM. Seventy-four out of 75 subjects successfully underwent intraoperative cholangiography (IOC); a diagnosis of PBM was established in 60 cases based on IOC alone. The diagnosis rate of pediatric PBM varied significantly among the four groups (P?<?0.0001). Pair-wise comparison showed a significant difference between the groups of MRCP and CT (P?<?0.0001), MRCP and US (P?<?0.0001), IOC and CT (P?<?0.0001), IOC and US (P?<?0.0001), CT and US (P?=?0.0027), and there is no significant difference between the groups of IOC and MRCP (P?=?0.0502).

Conclusion

US, IOC, CT and MRCP are valuable in showing dilatation of the bile duct and complications in pediatric PBM. MRCP is non-invasive, gives clear views of the pancreaticobiliary junction and should be the first choice for the diagnosis of PBM in children.     

Clinical and pathological characteristics of cystic lesions of extrahepatic bile duct in neonates

Aim: To investigate the differences in clinical and pathological manifestations between biliary atresia with extrahepatic biliary cyst and choledochal cyst in neonates. Methods: Symptoms and clinical signs in 5 neonates with biliary atresia with extrahepatic biliary cyst (4 of type I and 1 of type III) and 17 neonates with choledochal cyst were recorded. The levels of serum alkaline phosphatase, bilirubin, direct bilirubin, transaminase, gamma-glutamyl transpeptidase were analysed. Width and length of gallbladder and choledochal cyst were measured on B-mode ultrasound before surgery. Intrahepatic or extrahepatic biliary ducts were visualized with intraoperative cholangiography. The pathologic features in specimens of the liver were studied with light- and electron transmission microscopy. Results: All malformations occurred more commonly in girls, and obstructive jaundice was the main manifestation in both groups. Laboratory tests showed similar results for all patients in this study. With regard to pathological features, no significant difference was seen in either light microscopy or transmission electron microscopy, but it was shown with ultrasound that the length and width of the cysts and the gallbladder in neonates with biliary atresia were all shorter than the measurements in patients with choledochal cyst. The intrahepatic bile ducts could not be visualized on intraoperative cholangiography in type III biliary atresia. Deformation of the biliary ducts within the liver and stricture of the portal bile duct were the predominant features in type I biliary atresia, while the bile duct within the liver was normal or dilated in neonates with choledochal cyst.

Conclusions: Cystic lesions of the extrahepatic bile duct might be a common manifestation of biliary atresia and choledochal cyst. Intraoperative cholangiography is a precise and effective technique in the differential diagnosis of those lesions and helps decide on the most rational method of treatment.