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介绍临床以肝脾肿大、门脉高压、上消道出血为主要症状的一种少见的遗传病一先天性肝纤维化的概念、诊断与治疗。１９９３年以来收治先天性肝纤维化３例，均经临床及病理证实，行脾切除、贲门胃底断流术。术后随访１～３年，患儿发育、营养佳，无再出血，肝功能正常，近期疗效满意。复习文献结合本组病例诊治认为，临床发现肝脾肿大、门脉高压、肝内胆管多发性扩张、肝功能基本正常者应想到该病。肝脏组织学特点为汇管区纤维增生明显，肝小叶结构正常。为预防术后并发症应早期诊断，及时治疗。
Introduction to clinical hepatosplenomegaly, portal hypertension, upper gastrointestinal bleeding as the main symptoms of a rare genetic disease congenital liver fibrosis concept, diagnosis and treatment. Since 1993, 3 cases of congenital liver fibrosis were confirmed by clinical and pathological confirmed splenectomy, cardia gastric fundus. Follow-up 1 to 3 years after surgery, children with development, good nutrition, no further bleeding, normal liver function, the recent satisfactory results. Review the literature combined with the diagnosis and treatment of patients in this group that the clinical findings of hepatosplenomegaly, portal hypertension, multiple intrahepatic bile ducts, liver function should be thought of the disease should be normal. Histological characteristics of the liver for the portal area fibrous hyperplasia, hepatic lobule structure is normal. To prevent postoperative complications should be early diagnosis and timely treatment.