Endoscopic evaluation of the porta hepatis in patients with biliary atresia

Endoscopic inspection of the porta hepatis was undertaken through external enterostomy in nine postoperative patients with biliary atresia. The endoscopic appearance of bile flow in the porta hepatis was classified into three types. 1) Ductal type (D-type), which revealed good bile flow from distinct bile duct orifices (4 cases); 2) Oozing type (O-type), which showed adequate bile flow, but no definite bile duct (3 cases); and 3) Covered type (C-type), in which the porta hepatis was covered with bile "clots" and fibrous tissue (2 cases). The clinical course of the D-type was excellent with immediate disappearance of jaundice and relatively good liver function. In the case of the O-type, jaundice disappeared in 2 but persisted in one. Two patients with C-type died of hepatic failure or sepsis in the early postoperative period. In the D-type intrahepatic bile ducts were well visualized by cholangiography; however, those of the O-type were not so clear. Endoscopic inspection of the porta hepatis is very useful for evaluation of the postoperative state of bilioenteric fistulae in patients with biliary atresia.     

Histopathological studies on intrahepatic bile ducts in the vicinity of porta hepatis in biliary atresia.

Intrahepatic bile ducts in the vicinity of the porta hepatis were histologically investigated in 9 cases of biliary atresia and the pattern of bile duct was divided into two groups. In the first group, there were a few large bile ducts which had continuity to the extrahepatic bile duct. In the second group, many small bile ducts were histologically observed, but there was no large bile duct at the porta hepatis. This type of abnormal bile ducts was seen in the cases of obliteration of bile ducts at porta hepatis.     

Ischemic cholangitis in hepatic allografts.

In this report, our objectives are to introduce the term "ischemic cholangitis" as an etiologic designation and to describe its manifestations. Herein we use the label "ischemic cholangitis" as a collective term for ischemic bile duct necrosis, cholangitis caused by ischemia but without necrosis, and biliary fibrosis as a manifestation of ischemic damage. The condition was observed in 12 allografts, either at the time of retransplantation (9 cases) or at autopsy (3 cases). Ischemic cholangitis involved primarily perihilar extrahepatic and intrahepatic bile ducts. The findings included duct necroses (eight cases), strictures (four cases), and cholangiectases (four cases); some of these features coexisted. In addition, complicating ascending cholangitis and cholangitic abscesses were noted in three cases. Ischemic cholangitis was caused by hepatic artery thrombosis (in nine patients) or stenosis (in one) or by occlusion of parabiliary arteries by fibrointimal proliferations, probably attributable to old thromboses (in two, in conjunction with associated foam cell arteriopathy in one). Biopsy specimens before retransplantation or autopsy were obtained in 11 patients, only 1 of whom had an infarct as direct evidence of ischemia. Nine patients had evidence of biliary obstruction or bile flow impairment; in two cases, specimens were normal or nondiagnostic relative to cholangitis. Features of cellular rejection associated with the manifestations of bile flow impairment and ischemia were noted in five cases. Thus, biopsy features that suggest biliary obstruction, with or without cellular rejection, may be a manifestation of ischemic cholangitis. We conclude that ischemic cholangitis is an important cause of cholestatic graft failure but that this type of cholangitis is difficult to diagnose because of its misleading biopsy manifestations.     

Ursodeoxycholic acid therapy in the treatment of biliary atresia

The prognosis of operated biliary atresia in the cases with bile excretion chiefly depends upon the prevention of ascending cholangitis. An antibiotic is therefore intravenously administered during the early postoperative phase, but cannot be used over a long period. In the cases showing satisfactory bile excretion after operation, ascending cholangitis is rare because of rapid disappearance of jaundice. Regarding this, the authors prescribed ursodeoxycholic acid (UDCA) at 10-15 mg/kg/day to 6 infants with biliary atresia for several weeks after operation, and then determined the effects of UDCA in improving jaundice and bile excretion. As a result, serum bilirubin and serum total bile acid (STBA) levels were decreased in 4 of the 6 infants. In the remaining 2 infants, their STBA levels showed no decrease, but were rather increased; these infants subsequently died of hepatic failure. These results suggested that UDCA is useful in the treatment of cholestasis associated with biliary atresia in the cases attaining postoperative bile excretion. It was also suggested that the treatment with UDCA should be stopped when the STBA levels increased after the beginning of the treatment. Therefore, it was thought that STBA levels measured during UDCA therapy could serve as a good indicator of the choleretic effect of UDCA.     

Spectrum of biliary disease in childhood

Obstructive biliary disease in childhood is not common, but should be considered in the differential diagnosis of a child with jaundice, abdominal pain, or an abdominal mass. We have reviewed the experience at Vanderbilt Children's Hospital from 1970 to 1985, during which 87 children 18 years of age or younger with biliary tract disease were seen. Twelve patients (14%) had congenital disorders including choledochal stenosis, Caroli's disease, choledochal cyst, teratoma of the common hepatic duct and common bile duct, congenital septate biliary tree, and isolated atresia of the distal common bile duct. Thirty patients had neonatal cholestatic syndromes, and 37 had calculous disease of the gallbladder or extrahepatic bile ducts. Three patients had obstruction of the common bile duct caused by fibrosing pancreatitis. Two had sclerosing cholangitis. Obstruction of the common bile duct was caused by metastatic neoplasm in three patients. We discuss principles of diagnosis and management.     

Cholangitis‐related biliary pneumatosis cysts in the dawning era of routine direct endoscopic visualization of biliary diseases

Biliary pneumatosis has recently been reported in intrahepatic amd extrahepatic bile ducts complicating bacterial cholangitis, as characterized by cholangioscopy. This clinical report adds another case of small‐scale pneumatosis cysts in the common bile duct to the literature. Similary to previously reported cases, the recent patient likewise had a history of acute cholangitis. It might be tempting to speculate that this finding might prove more common than expected in the dawning era of routine use of cholangioscopic characterization of biliary tract diseases.     

Clinical value of MRC in the follow-up of liver transplant patients with a choledochojejunostomy

BACKGROUND: We investigated the clinical value of magnetic resonance cholangiography (MRC) in liver transplant patients receiving choledochojejunostomy (CDJ). METHODS: Twenty-five MRCs were performed in 23 initially asymptomatic patients 19 months (mean) after liver transplantation with biliary reconstruction via CDJ. The images were evaluated by consensus (two investigators) for bile duct strictures and dilatations. As a standard of reference, clinical follow-up (including laboratory analysis) was used in 20 cases and direct cholangiography or surgery in three cases. RESULTS: Fourteen pathologic findings were observed in 11 patients (anastomotic strictures in four, left or right bile duct strictures in three, and peripheral segmental dilatations with or without strictures in seven). Patients with pathologic MRC findings had significantly higher levels of alkaline phosphatase (p < 0.05) and more frequently had histories of cholangitis than did patients with normal MRC. Four of six patients with stenoses of the central bile ducts subsequently developed biliary complications requiring treatment (three confirmed by direct cholangiography). In patients with unremarkable bile ducts or only peripherally located changes on MRC, no bile duct complications or relevant changes in the cholestasis parameters occurred during follow-up (mean = 30 months). CONCLUSION: MRC can noninvasively detect pathologic biliary tract changes in liver transplant patients in the asymptomatic stage and provide information for planning invasive therapeutic procedures.     

ERCP后CT及MPR诊断肝外梗阻性黄疸病因(附24例分析)

目的:探讨ERCP后加做肝胆胰CT对肝外胆道梗阻病因的诊断价值。材料和方法:24例肝外胆道梗阻病人,经ERCP造影后立即进行CT扫描,应用MPR技术充分显示胆道及其周围情况。结果:24例中,胆管癌5例,胰头癌7例,壶腹癌1例,胆总管下端结石9例,胆总管炎2例,均经手术证实,无假阳性及假阴性。结论:ERCP后加做肝胆胰CT及MPR是诊断肝外梗阻性黄疸的一种可靠手段。     

多层螺旋CT诊断胆道梗阻性疾病68例分析

目的探讨多层螺旋CT在胆道梗阻性疾病的临床应用价值。方法 68例行CT常规平扫及薄层扫描的胆道梗阻患者,其中30例进行增强CT三期扫描。结果 68例中胆管结石41例,胰头癌15例,胆管癌3例,胆管炎7例,壶腹癌2例。结论多层螺旋CT扫描在胆道梗阻性疾病的诊断中准确性高,是胆道梗阻性疾病有效的检查方法。     

Neonatal NK cells target the mouse duct epithelium via Nkg2d and drive tissue-specific injury in experimental biliary atresia

Biliary atresia is a neonatal obstructive cholangiopathy that progresses to end-stage liver disease. Although the etiology is unknown, a neonatal adaptive immune signature has been mechanistically linked to obstruction of the extrahepatic bile ducts. Here, we investigated the role of the innate immune response in the pathogenesis of biliary atresia. Analysis of livers of infants at diagnosis revealed that NK cells populate the vicinity of intrahepatic bile ducts and overexpress several genes involved in cytotoxicity. Using a model of rotavirus-induced biliary atresia in newborn mice, we found that activated NK cells also populated murine livers and were the most abundant cells in extrahepatic bile ducts at the time of obstruction. Rotavirus-primed hepatic NK cells lysed cholangiocytes in a contact- and Nkg2d-dependent fashion. Depletion of NK cells and blockade of Nkg2d each prevented injury of the duct epithelium after rotavirus infection, maintained continuity of duct lumen between the liver and duodenum, and enabled bile flow, despite the presence of virus in the tissue and the overexpression of proinflammatory cytokines. These findings identify NK cells as key initiators of cholangiocyte injury via Nkg2d and demonstrate that injury to the duct epithelium drives the phenotype of experimental biliary atresia.     

Laparoscopic internal intestinal drainage of bile lakes in a patient with recurrence of jaundice after laparoscopic revision of Kasai portoenterostomy for biliary atresia: A case report

Bile lake, of the postoperative complications after Kasai portoenterostomy (PE) for biliary atresia, causes cholangitis that may induce progressive fibrosis of the liver. Standard treatment for bile lakes has not yet been established, but there are reports that surgical internal intestinal drainage for bile lakes effectively prevents cholangitis and maintains jaundice-free status. In this case, insertion of the percutaneous transhepatic biliary drainage into the bile lake allowed continuous drainage of large volumes of bile juice. However, reoperation following laparotomy increases the surgical risk of subsequent liver transplantation due to postoperative adhesion. Laparoscopic surgery was selected for the patient who was likely to require liver transplantation in the future. In this case, laparoscopic internal intestinal drainage of bile lakes was performed safely by a Cavitron ultrasonic surgical aspirator for the recurrence of jaundice after laparoscopic revision of PE. Cholangitis and jaundice were rapidly resolved after this surgical procedure.     

Caroli病的64排螺旋CT诊断

目的 探讨Caroli病的64排螺旋CT表现及诊断价值.方法 对经手术病理证实的15例Caroli病的64排螺旋CT影像资料进行回顾性分析.结果 所有病例CT均表现为肝内多发囊状或条状低密度影,与肝内胆管相通,增强扫描无强化,其中4例可见＂中心圆点征＂.Caroli病I型12例,其中2例合并肝内多发血管瘤及肝内多发小囊肿,4例在扩张的胆管内见结石并3例见有胆管积气,1例合并胆管炎改变,其余5例仅发现肝内胆管扩张.Caroli病II型3例,其中2例伴有肝硬化;1例合并肝内小胆管炎及门静脉周围纤维化.结论 64排螺旋CT三维重建的应用,可以多方位、多角度全面观察肝内多发囊性病变的分布及与肝内胆管分支的关系,对Caroli病的诊断、鉴别诊断及明确病变范围有重要价值.     

Value of ERCP in the diagnosis and management of pre-and postoperative biliary complications in hydatid disease of the liver

Twenty-nine patients with symptomatic hydatid disease of the liver were evaluated preoperatively by endoscopic retrograde cholangiopancreatography (ERCP) in the years 1982–1987. In the same period, 6 patients who previously underwent surgery for the parasitic disease also underwent ERCP because of postoperative symptoms of biliary obstruction.Findings at ERCP excluded biliary tract involvement in 11 cases. Positive findings were shown in 24 patients, including compression of the bile ducts in 4, small cysto-biliary communications in 5, intrabiliary rupture in 9, residual hydatid material in the common bile duct, and sclerosing cholangitis in 3.Endoscopic removal of migrated hydatid debris was achieved in 8 patients, avoiding reoperation in 4.     

磁共振胆管成像和动态十二指肠液检查鉴别诊断婴儿肝炎与胆道闭锁

目的探讨磁共振胆管成像(MRC)和十二指肠液引流对婴儿期胆道闭锁(EHBA)与婴儿肝炎综合征(IHS)的鉴别诊断价值。方法应用磁共振和婴儿十二指肠引流管对52例婴儿期持续性阻塞性黄疸行MRC和十二指肠液检查,以胆总管、肝总管显影和十二指肠液检查有胆汁(胆汁酸阳性)并经随访黄疸消退诊断为婴儿肝炎综合征;以胆总管、肝总管未显示,十二指肠液检查无胆汁(胆汁酸阴性)且经外科手术探查诊断为胆道闭锁;并将胆道闭锁MRC及十二指肠检查结果与手术、病理结果对比分析。结果52例患儿中最后有34例确诊为婴儿肝炎综合征,18例确诊为胆道闭锁。34例婴儿肝炎综合征中30例MRC能清楚显示胆总管、肝总管结构,2例显示较小胆囊,2例胆总管、肝总管未显示。首次十二指肠液检查有胆汁31例,3例无胆汁。18例胆道闭锁中13例胆囊、胆总管和肝总管未显示,4例仅显示较小胆囊,1例仅显示较大胆囊,十二指肠液检查均无胆汁。18例确诊为胆道闭锁的患儿中,17例与MRC及十二指肠引流诊断相符。结论十二指肠引流液检查能直接观察有无胆汁,诊断胆道闭锁的敏感性为100%,特异性为91.1%;MRC能显示肝外胆道结构,诊断胆道闭锁的敏感性为94.4%,特异性88.24%;十二指肠与MRC联合诊断胆道闭锁的敏感性94.4%,特异性97.06%,因此二者结合应用能较准确鉴别诊断婴儿肝炎与胆道闭锁。     

Endoscopic management of persistent biliary leakage resulting from complete transection of the bile duct at cholecystectomy

BACKGROUND AND STUDY AIMS: Injuries to the bile duct are not uncommon during cholecystectomy. While minor injuries are amenable to endoscopic therapy, major ones, such as complete transection of the duct, require surgical intervention. We report on the endoscopic management of such injuries. PATIENTS AND METHODS: We included in the study ten patients who had persistent postoperative bile drainage (either through a surgically placed catheter or through a biliocutaneous fistula) after their cholecystectomy procedure had been complicated by complete transection of the bile duct. Plastic biliary endoprostheses were placed in the bilioma through the papilla of Vater. In one patient, both the right and the left hepatic ducts were opacified by injecting contrast material through the drainage catheter and it was possible to place stents in both the ducts. RESULTS: All the patients improved clinically after the procedure. In one patient the stent became dislodged and an elective Roux-en-Y hepaticojejunostomy was performed, but it was possible to remove the stents from all the other patients. Two patients were referred for surgery but in both cases the bile flow through the bile duct was shown to be so good on nuclear imaging that they were not operated on. All the non-operated patients are well after a mean +/- SD follow-up of 22.3 +/- 5.5 months. CONCLUSIONS: Placement of biliary stents in the bilioma is a useful adjunct to percutaneous drainage in patients with complete transection of the bile duct. After placement of a biliary stent in the bilioma the percutaneous drainage catheter may be removed. In one of our study patients it was also possible to place stents in the intrahepatic ducts and the bile duct was reconstructed. Long-term follow-up of these patients and further studies are required to assess the role of endoscopic management as an alternative to surgery in patients with this condition.     

多腔T型管在腹腔镜胆总管切开及胆道镜下液电碎石术中的应用

目的探讨＂多腔T型管＂在腹腔镜胆总管切开及胆道镜下液电碎石术中的应用效果。方法选择2012年6月至2013年12月收治的60例肝内外胆管结石患者。60例患者按随机数字表法分为治疗组（30例）和对照组（30例）,均采用腹腔镜胆总管切开、胆道镜下液电碎石术,治疗组术中放置多腔T型管;对照组术中放置普通T型管。记录两组患者手术时间、术中出血量、术后首次肛门排气时间、首次肛门排便时间、术后胆汁丢失量及平均术后住院日和住院总费用;记录并发症发生情况（包括切口感染、电解质紊乱、反流性胆管炎、胆漏等）及术后2个月结石残留率、术后6个月结石复发情况。结果 （1）治疗组手术时间、术中出血量与对照组比较差异无统计学意义（P均〉0.05）。治疗组术后首次肛门排气时间、首次肛门排便时间、术后胆汁丢失总量、术后平均住院天数、住院总费用均少于对照组,差异均有统计学意义（P〈0.05或P〈0.01）。（2）治疗组术后出现并发症6例（20.00%）,包括切口感染2例、电解质紊乱2例、反流性胆管炎1例、胆漏1例;对照组出现术后并发症16例（53.33%）,包括切口感染3例、电解质紊乱7例、反流性胆管炎4例、胆漏2例。两组并发症发生率比较差异有统计学意义（P〈0.01）。两组并发症均经保守治疗治愈。（3）治疗组结石残留率（3.33%）稍低于对照组（20.00%）,两组比较差异无统计学意义（P〉0.05）。术后随访6个月,两组均未发现结石复发。结论在腹腔镜胆总管切开、胆道镜下液电碎石术中放置多腔T型管,具有术后恢复较快、并发症较少、术后结石残留率低等优势。     

Multiple comet tail artifacts in the liver: a case of congenital hepatic fibrosis showing unusual biliary appearance

Congenital hepatic fibrosis (CHF) is a form of autosomal recessive polycystic kidney disease. Because of the common underlying pathophysiology of ductal plate malformation, CHF can be accompanied by an abnormal biliary appearance, which is characterized by a saccular or fusiform dilatation of the bile ducts. We encountered the case of a 35-year-old man suffering from CHF concomitant with esophageal varices, which were treated by endoscopic sclerotherapy. The patient had elevated serum concentrations of alkaline phosphatase and γ-glutamyl transpeptidase without apparent biliary disease, including hepatolithiasis or a history of cholangitis. Magnetic resonance cholangiography showed an abnormal biliary appearance, which was not saccular or fusiform but had multiple stenosis with unknown causes. B-mode sonogram showed multiple comet tail artifacts in the liver parenchyma, probably corresponding to the compact fibrosis bands and bile in the bile duct as well as peripheral bile duct dilatation, which was proven pathologically. We propose that multiple comet tail artifacts in the liver may suggest the presence of a bile duct abnormality in patients with CHF, suggesting the potential risk for developing biliary complications.     

Secondary sclerosing cholangitis from percutaneous transhepatic biliary drainage in a patient with gallbladder cancer after surgery: A case report

Secondary sclerosing cholangitis (SSC) is a chronic cholestatic liver disease characterized by fibrosis and stricture of the bile ducts. SSC in association with multiple factors such as spontaneous choledochoduodenal fistula and metastatic gallbladder cancer has rarely been reported. However, to the best of our knowledge, reports of SSC after percutaneous transhepatic biliary drainage (PTBD), especially in cases with diffuse calcification of the bile duct walls, have not been reported. We report a case of SSC from PTBD in a patient with gallbladder cancer after surgery. The patient underwent external percutaneous biliary drainage for malignant bile duct obstruction after cholecystectomy. Repeated exchanges were performed at the first and the sixth month after PTBD using an internal and external drainage catheter. Two months after the third catheter exchange, findings of laboratory and imaging examinations were suggestive of SSC. The liver function tests of the patient were suggestive of cholestasis. Multidetector computed tomography showed diffuse calcification of the bile duct walls. Cholangiography showed intrahepatic biliary stenosis or dilatation.     

Obstruction of extrahepatic bile ducts by lymphocytes is regulated by IFN-gamma in experimental biliary atresia

The etiology and pathogenesis of bile duct obstruction in children with biliary atresia are largely unknown. We have previously reported that, despite phenotypic heterogeneity, genomic signatures of livers from patients display a proinflammatory phenotype. Here, we address the hypothesis that production of IFN-gamma is a key pathogenic mechanism of disease using a mouse model of rotavirus-induced biliary atresia. We found that rotavirus infection of neonatal mice has a unique tropism to bile duct cells, and it triggers a hepatobiliary inflammation by IFN-gamma-producing CD4(+) and CD8(+) lymphocytes. The inflammation is tissue specific, resulting in progressive jaundice, growth failure, and greater than 90% mortality due to obstruction of extrahepatic bile ducts. In this model, the genetic loss of IFN-gamma did not alter the onset of jaundice, but it remarkably suppressed the tissue-specific targeting of T lymphocytes and completely prevented the inflammatory and fibrosing obstruction of extrahepatic bile ducts. As a consequence, jaundice resolved, and long-term survival improved to greater than 80%. Notably, administration of recombinant IFN-gamma led to recurrence of bile duct obstruction following rotavirus infection of IFN-gamma-deficient mice. Thus, IFN-gamma-driven obstruction of bile ducts is a key pathogenic mechanism of disease and may constitute a therapeutic target to block disease progression in patients with biliary atresia.     

64排螺旋CT结合3D血管重建技术对儿童肝移植术后的评估价值

目的:探讨64排螺旋CT结合3D血管重建技术在儿童肝移植术后的评估价值。方法:回顾性分析2018年1月-2019年4月在我院接受肝移植术后的131例儿童患者,其中先天性胆道闭锁83例、胆道闭塞33例、先天性肝硬化合并门脉高压10例、肝衰竭5例。全部患者均行64排螺旋CT肝脏平扫、四期增强及CTA+CTV扫描,采用MPR、VR、CPR及MIP对肝脏及血管进行重建。由2位副主任诊断医师对图像进行分析和诊断,内容包括移植后肝实质及毗邻器官,术后血管吻合口、肝胆管血管的通畅情况和走向、胆道情况及腹腔积液等。结果:发现肝脏灌注不良50例,肝脏、脾脏增大54例,肝内胆管扩张43例,腹腔积水32例,术后血管吻合口狭窄30例。结论:64排螺旋CT结合3D血管重建及多种后处理技术,能有效地反映出儿童肝移植术后的肝实质、血管及胆道情况,为临床早日发现肝移植术后并发症及对术后评估提供可靠依据。