Department of Transplant Surgery, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke City, Tochigi 329-0498, Japan.
J Hepatobiliary Pancreat Sci. 2018 Aug;25(8):359-369. doi: 10.1002/jhbp.572.
Congenital portosystemic shunt (CPS) is classified into type I (congenital absence of the portal vein) and type II, depending on the presence or absence of the intrahepatic portal vein. Reports still exist on cases in which psychiatric diseases or mental retardation was diagnosed or cases in which diagnosis and treatment are not performed until severe pulmonary complications occur, indicating the need to recognize CPS. Herein, we reviewed epidemiology, etiology, classification, symptom, diagnosis, and treatment based on clinical view points of CPS. For clinical view points, classification according to (1) the presence or absence of the intrahepatic portal venous system (IPVS) and (2) the extrahepatic or intrahepatic of shunt sites, facilitates the understanding of pathophysiological conditions and is useful in selecting a treatment for symptomatic CPS. Radiological and pathological examinations are important in IPVS evaluations, and IPVS evaluations are currently essential to make diagnoses by portography with balloon occlusion and liver biopsy. Symptomatic CPS (hepatic nodular lesions, portosystemic encephalopathy, and pulmonary complications, etc.) is an indication of treatment by shunt closure, but an indication of treatment for asymptomatic CPS is the challenge going forward.
先天性门体分流 (CPS) 分为 I 型（门静脉先天性缺失）和 II 型，取决于是否存在肝内门静脉。仍有报告称诊断出精神疾病或智力迟钝，或直到出现严重的肺部并发症才进行诊断和治疗，表明需要认识 CPS。在此，我们根据 CPS 的临床观点综述了其流行病学、病因、分类、症状、诊断和治疗。从临床观点来看，根据 (1) 肝内门静脉系统 (IPVS) 的存在与否和 (2) 分流部位的肝外或肝内进行分类，有助于了解病理生理状况，并有助于选择有症状 CPS 的治疗方法。影像学和病理学检查对于 IPVS 评估很重要，目前，通过球囊阻塞门静脉造影和肝活检进行诊断，IPVS 评估至关重要。有症状 CPS（肝结节病变、门体系统脑病和肺部并发症等）是分流关闭治疗的指征，但无症状 CPS 的治疗指征是未来的挑战。
