Bile duct stricture due to caused by portal biliopathy: Treatment with one-stage portal-systemic shunt and biliary bypass

Portal biliopathy is a rare complication of extrahepatic portal vein obstruction. Jaundice occurs in symptomatic patients with fibrotic strictures. Short-term improvement in such patients can be achieved with endoscopic retrograde cholangio-pancreatography with balloon dilatation and stent placement. Surgery in these patients is traditionally two staged. We report the results of a one-stage procedure combining non-selective portal-systemic shunt surgery with biliary bypass, performed successfully on a 24-year-old man with a tight biliary stricture resulting from portal biliopathy. At 18-month follow up, the patient shows he is doing well, with normal liver function tests.     

Biliary stricture caused by portal biliopathy: case report and literature review

Portal biliopathy is a rare condition that is usually not diagnosed and only in few cases causes symptoms. Those symptoms are caused by vascular obstruction of the biliary tree in patients with portal hypertension. We report a case of a 29 years man who presented with history of intermittent jaundice, persistent elevation of hepatic function test and hematemesis as a manifestation of portal hypertension without liver damage. We present the clinical, radiological and pathological characteristics and literature review of the cases that had been reported, their diagnoses, treatment and clinical implication.     

Cholangiojejunal fistula caused by bile duct stricture after intraoperative injury to the common hepatic artery

A 68‐year‐old man, admitted for the treatment of recurrent cholangitis after a pancreatoduodenectomy (PD) performed 3 years previously was diagnosed as having multiple hepaticolithiasis. On laparotomy, the hepatic artery was not recognized. The anastomosed common hepatic duct was obstructed, and a fistula had been formed between the right hepatic duct and the Roux limb of the jejunum. Lithotripsy was performed from this fistula and it was reanastomosed. Angiography was performed postoperatively and it revealed common hepatic artery injury, most likely to have occurred during the previous PD. The patient's postoperative course was uneventful and he has been asymptomatic for 8 months after the operation, indicating that reanastomosis of the fistula can be an effective method. The stricture of the anastomosis was suspected to be mainly due to cholangial ischemia, because no episode of anastomotic leak or retrograde biliary infection had occurred during the PD perioperative period. There are several reports of late stricture of anastomosis 5 or more years after cholangiojejunostomy. This patient, therefore, requires further long‐term follow up.     

Therapeutic approaches for portal biliopathy: A systematic review

Portal biliopathy(PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/nonneoplastic extrahepatic portal vein obstruction(EHPVO) and portal cavernoma(PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC(77%-100%), only a part of these(5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic(Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical(bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension.     

门脉高压性胆病诊治进展

门脉高压性胆病(PHB)为继发于门静脉高压特别是肝外门静脉阻塞患者的肝内、外胆管和/或胆囊壁、胆囊管的异常改变.PHB的发病机制尚不完全清楚,目前认为与门静脉海绵状血管瘤曲张压迫胆管和胆管壁缺血性损害有关.约20% PHB患者存在胆道系统症状,且与年龄、疾病持续时间、胆石症的发生频率以及肝功能异常相关.磁共振胆管造影术和门静脉造影术是PHB患者的首选检查方法,有症状者可予内镜或外科手术治疗,治疗目的为降低门脉高压和解除梗阻.     

Bile duct disorders

Drug-induced bile duct injury related prolonged or chronic cholestasis is recognized as a common side effect of treatment with several drugs. The severity and duration of the clinical symptoms suggest that this increase in number of reports is not only related to clinician and pathologists being increasingly aware of the condition, but also may represent a true increase in incidence likely related to a time-related growing experience with newer drugs. This clinical presentation encompasses a wide variety of features that may be the source of diagnostic difficulties, especially in the cases where cholestasis occurs days or weeks after the completion of therapy. Even more puzzling is the initial picture of hepatocholangitis, which may be silent and ensuing bile duct paucity with chronic anicteric cholestasis may be another source of diagnostic difficulties in the long-term. These diagnostic difficulties suggest that some of the cases of the so-called "idiopathic adulthood ductopenia" may originate from overlooked drug induced vanishing bile duct syndrome. The pathogenesis of the syndrome remains largely unknown and the determinants of prognosis and outcome. From reproducible data obtained in different studies investigating HLA-dependent predisposition, one may assume that genetics plays a major role even if other unknown additive factors are also likely involved. Severity of initial hepatocholangitis is likely to represent another important determinant of severity and prognosis, however to be assessed in larger longitudinal studies. Therapy of large bile duct injury mimics that of primary sclerosing cholangitis. Treatment of small bile duct injury remains disappointing. Corticosteroids are invariably ineffective. Ursodeoxycholic acid as been shown to induce improvement of clinical and biochemical cholestasis in some selected cases, its efficacy being however unpredictable. Preliminary data about the natural history of the vanishing bile duct syndrome suggest that therapy might be more effective when initiated early.