Outcomes of liver transplantation for Alagille syndrome after Kasai portoenterostomy: Alagille Syndrome with agenesis of extrahepatic bile ducts at porta hepatis

BackgroundAlagille syndrome (ALGS) is an autosomal dominant disorder, characterized by a paucity of intrahepatic bile ducts, resulting in significant cholestasis, and peculiar extrahepatic features. Some ALGS patients show a considerable overlap with biliary atresia (BA), and they can undergo Kasai procedure. The purpose of this study is to show the manifestations of BA overlapped ALGS cases in our institution, and to compare the outcomes of ALGS patients following liver transplantation (LT) between those who previously underwent Kasai surgery (ALGS-Kasai group) and those who did not (ALGS-non-Kasai group).MethodsMedical records of ALGS patients who underwent LT in Kyoto University Hospital, Japan from January 1992 to March 2018 were analyzed. ALGS diagnosis was determined according to physical, radiologic, and histopathological findings.ResultsThirty-one patients were ascertained (ALGS-Kasai: 4 males and 5 females vs. ALGS-non-Kasai: 14 males and 8 females, p = 0.43). Of 31 ALGS patients, 96.8% of children had pulmonary artery stenosis, 54.8% showed facial features, 29% revealed skeletal anomalies and 9.7% demonstrated ocular anomalies. The age at LT was significantly younger in ALGS-Kasai than ALGS-non-Kasai group (1.47 [interquartile range (IQR), 0.75–1.92] vs. 5.1 [IQR, 1.4–9.29] years; p = 0.038). Overall patient survival did not significantly differ between ALGS-Kasai (88.9%) and ALGS-non-Kasai patients (86.4%) (p = 0.84). Furthermore, the 1-year, 5-year, and 10-year patient survival rates for ALGS-Kasai group were 100%, 88.9%, and 88.9%, respectively, whereas those for ALGS-non-Kasai group were 90.9%, 90.9%, and 86.4%, respectively, with p-values of 0.36, 0.90, and 0.84, respectively.ConclusionsBA overlapped ALGS cases had neonatal progressive cholestasis which prompted Kasai procedure, and early liver dysfunction after Kasai led to performing LT. The ALGS-Kasai patients undergo LT at earlier ages than the ALGS-non-Kasai patients, however, overall patients' survival rates are similar between groups. Overall ALGS patients' survival rate after LT is considered high.Levels of EvidenceLevel III; Case–control study or Retrospective comparative study.     

New approach in diagnosis of bile duct disease with ultrasound and magnetic resonance imaging

Abstract Advances in technology have made possible the clinical application of magnetic resonance cholangiography, endoscopic, intra-operative and laparoscopic ultrasound in the study of the biliary tree, in addition to conventional magnetic resonance imaging and transabdominal ultrasound. The role of the conventional, as well as the newer techniques, in the diagnosis of bile duct disease and the limitations and pros and cons of each technique, will be the subject of the present discussion.     

Newer diagnostic procedures in pancreatobiliary surgery

Newer diagnostic procedures useful in establishing accurate indication for surgical intervention in pancreatobiliary system have been reviewed during a five year period from 1968 to 1972.     

Accuracy of computed tomographic intravenous cholangiography (CT-IVC) with iotroxate in the detection of choledocholithiasis

To determine the accuracy of computed tomographic intravenous cholangiography (CT-IVC) for detection of choledocholithiasis. Sixty-five patients undergoing endoscopic retrograde cholangiography (ERC) underwent CT-IVC prior to ERC, using a single detector helical CT following intravenous infusion of 100 ml iotroxate. Patients with bilirubin levels >3 times normal were excluded. ERC was indeterminate in three patients (4.7%) and CT-IVC in four (6.3%). Twenty-three patients had ductal calculi at ERC, and CT-IVC was positive in 22, with two false positives and one false negative: sensitivity 95.6%, specificity 94.3%. Stones were multiple in nine and solitary in 14. Of the 14 solitary stones, ten were 5 mm and eight were 4 mm. The bilirubin level in positive cases was within normal in 20. Maximum intensity projection (MIP) reformats showed stones in only 27% of cases and surface rendered (SR) reformats in none. CT-IVC is highly accurate for detection of ductal calculi, including single small calculi, with a normal or near normal serum bilirubin. Axial images should be used for interpretation rather than MIP or SR reformats.     

Laparoscopic common bile duct exploration

Background: Herein I describe my >12-year experience with laparoscopic common bile duct exploration (LCBDE). Methods: From 21 September 1989 through 31 December 2001, 3,580 patients presented with symptomatic biliary tract disease. Laparoscopic cholecystecomy (LC) was attempted in 3,544 of them (99.1%) and completed in 3,527 (99.5%). Laparoscopic cholangiograms (IOC) were performed in 3,417 patients (96.4%); in 344 cases (9.7%), the IOC was abnormal. Forty-nine patients (1.4%) underwent preoperative endoscopic retrograde cholangiopancreatography (ERCP), and 33 patients (0.9%) underwent postoperative ERCP. LCBDE was attempted in 326 cases and completed in 321 (98.5%). It was successful in clearing the duct in 317 of the 344 patients with abnormal cholangiograms (92.2%). Results: The mean operating time for all patients undergoing LC with or without cholangiograms or LCBDE or other additional surgery was 56.9 min. Mean length of stay was 22.1 h. The mean operating time for LC only patients (n = 2530)—that is, those not undergoing LCBDE or any other additional procedure—was 47.6 min; their mean postoperative length of stay was 17.2 h. Ductal exploration was performed via the cystic duct in 269 patients, (82.5%) and through a choledochotomy in 57 patients (17.5%). T-tubes were used in patients in whom there was concern for possible retained debris or stones, distal spasm, pancreatitis, or general poor tissue quality secondary to malnutrition or infection. In cases where choledochotomy was used, a T-tube was placed in 38 patients (67%), and primary closure without a T-tube was done in 19 (33%). There were no complications in the group of patients who underwent choledochotomy and primary ductal closure without T-tube placement or in the group in whom T-tubes were placed. Conclusions: Common bile duct (CBD) stones still occur in 10% of patients. These stones are identified by IOC. IOC can be performed in >96.4% of cases of LC. LCBDE was successful in clearing these stones in 97.2% of patients in whom it was attempted and in 92.2% of all patients with normal IOCs. Most LCBDEs in this series were performed via the cystic duct because of the stone characteristics and ductal anatomy. Selective laparoscopic placement of T-tubes in patients requiring choledochotomy (67%) appears to be a safe and effective alternative to routine T-tube drainage of the ductal system. ERCP, which was required for 5.8% of patients with abnormal cholangiograms, and open CBDE, which was used in 2.0%, still play an important role in the management of common bile duct pathology. The role of ERCP, with or without sphincterotomy, has returned to its status in the prelaparoscopic era. LCBDE may be employed successfully in the vast majority of patients harboring CBD stones.     

PERCUTANEOUS LAPAROSCOPIC CHOLECYSTECTOMY: INDICATIONS,CONTRAINDICATIONS AND COMPLICATIONS

The indications, contraindications and complications of percutaneous laparoscopic cholecystectomy (PLC) were established from a group of 308 patients referred for cholecystectomy. Of the 308 patients 86% underwent PLC, 5% were commenced laparoscopically, but converted to open cholecystectomy and 9% were performed as open cholecystectomy from the outset. Complications included two bile leaks from the gall-bladder bed, one cystic duct stump leak and three retained stones. Pre-operative rather than intra-operative duct imaging was used so that common duct stones could be removed before operation. PLC is a safe procedure that has now become the standard technique for cholecystectomy.     

Extended right hepatectomy for hilar cholangiocarcinoma with resection of the left hepatic duct prior to hepatic resection

Extended hepatectomy for hilar cholangiocarcinoma results in high operative or in-hospital mortality rates despite of the recent progress in perioperative management. As a new procedure to prevent postoperative hepatic failure in hilar cholangiocarcinoma infiltrating predominantly the right hepatic duct, we devised a combination of extended right lobectomy plus caudal lobectomy with resection of the left hepatic duct prior to hepatic resection by utilizing intraoperative cholangiography, and applied the procedure to a 70-year-old patient. He had a favorable postoperative course and remains recurrence-free at 4 years after operation. This is a procedure for confining the extent of hepatectomy to the minimum necessary, aiming at curative resection of hilar cholangiocarcinoma.     

磁共振胰胆管成像的临床应用与进展

本文综述了磁共振胰胆管成像 (MRCP)的原理、技术及临床应用 ,讨论其与直接胆道造影比较的优、缺点 ,认为MRCP在很多情况下已可取代传统的诊断性内窥镜逆行胰胆管造影术 (ERCP)或经皮肝胆管造影术(PTC)的作用 ,并推测未来MRCP还将应用于胰胆管病变的介入治疗。     

16层计算机断层摄影结合多平面重建技术在胆管阻塞病人中的应用

[目的]评价16层计算机断层摄影结合多平面重建技术在胆管阻塞病人中的应用。[方法]使用16层计算机断层摄影结合多平面重建技术(MPR)对60例被认为患有胆管阻塞的病人进行诊断。诊断中没有使用任何胆管造影剂。其中24例病人接受了磁共振胰胆管造影成像(MRCP)检查,16例病人进行了逆行胰胆管造影(ERCP)检查,28例病人进行了活组织切片检查或外科手术。我们将16层计算机断层摄影检查结果与磁共振胰胆管造影成像、逆行胰胆管造影、活组织切片检查及外科手术的结果进行了对比。以手术病理结果为标准。[结果]检查结果如下:胆总管结石(34例),恶性狭窄(14例),良性狭窄(2例),胆石症(12例)。1例病人的一枚小胆总管结石16层计算机断层摄影未能检出。2例16层计算机断层摄影初诊为胆管结石的病人在接受其他相关检查时诊断为恶性胆管狭窄。16层计算机断层摄影诊断胆管结石的准确率为91.1%。16层计算机断层摄影诊断胆管狭窄的准确率为87.5%。[结论]16层计算机断层摄影及多平面重建技术是一种快速和无创技术,在诊断胆管阻塞原因方面具有相当高的准确性。     

术中胆道造影在胆总管可疑结石中的应用

目的探讨经胆囊管造影在胆总管可疑结石中的应用价值。方法在胆囊切除后,对手术前怀疑有胆总管结石的78例病人,经胆囊管进行造影摄片。结果78例病人中发现有胆总管结石的4例,左肝内胆管结石1例,无并发症发生。结论术中胆道造影可以减少胆囊切除后残余结石的发生,也减少了很多不必要的胆总管阴性探查给病人带来的痛苦,是一种操作简单、准确性高、安全有效的好方法,有较好的临床应用价值。