非肝硬化性门静脉高压(NCPH)是指除肝硬化外多种疾病导致的门静脉高压症。NCPH常见的原因有门静脉血栓形成、先天性肝纤维化和特发性门静脉高压等。这组疾病的主要特点是门静脉高压相关的表现突出,而肝功能储备相对较好,鉴别该类疾病需要临床,影像学和病理学的深入检查。通过适当的内外科治疗,多数患者预后较好。 Non-cirrhotic portal hypertension (NCPH) refers to a variety of diseases in addition to cirrhosis of the portal hypertension. Common causes of NCPH are portal vein thrombosis, congenital liver fibrosis and idiopathic portal hypertension. The main features of this group of diseases are the prominent manifestations associated with portal hypertension and the relatively good reserve of liver function. The identification of such diseases requires in-depth clinical, radiological and pathological examination. With proper surgical treatment, most patients have a better prognosis.