【摘要】 目的 结合文献探讨儿童先天性肝内门体分流(congenital intrahepatic portosystemic venous shunts, CIPSVS)的临床表现及治疗策略。方法 回顾性分析湖南省儿童医院收治的1例以肺动脉高压为首发症状的先天性肝内门体分流V型静脉导管未闭(patent ductus venosus, PDV)患儿临床资料及术后随访情况，并进行相关文献复习。结果 本例患儿表现为反复呼吸道感染及肺动脉高压，行静脉导管结扎术，术后随访半年未发生呼吸道感染，且肺动脉高压较前降低。同时对国内外相关文献进行分析，共纳入41例患儿，行介入手术4例，开腹手术17例，腔镜手术3例，术后临床症状均缓解，但有17例未报道治疗方案。结论 CIPSVS临床罕见，且临床表现高度多样化，当患儿出现肝门静脉血灌注不足导致肝功能受损、代谢异常以及心肺系统异常灌注时，应考虑此病的可能，治疗需要个体化。更多还原

【Abstract】 Objective To explore the clinical manifestations and treatment strategies of congenital intrahepatic portosystemic venous shunt(CIPSVS) in children with a review of literature. Methods The clinical and postoperative follow-up data was retrospectively analyzed for a child with type V CIPSVS,namely, patent ductus venosus(PDV) with pulmonary hypertension as the first symptom, and the relevant literature was reviewed and analyzed. Results The patient had repeated respiratory tract infection and pulmonary hypertension, and underwent venous catheter ligation.No respiratory tract infection occurred in the 6-month postoperative follow-up, with pulmonary hypertension lower than before.Relevant literature reports were analyzed simultaneously and 41 cases were included.There were 4 cases treated with interventional surgery, 17 cases with open surgery, and 3 cases with endoscopic surgery, all seeing clinical symptoms relieved after surgery.But there were 17 cases that did not report the treatment plan. Conclusion CIPSVS is a clinically rare disease with its clinical manifestations highly diverse.In the cases of liver dysfunction and abnormal metabolism due to insufficient hepatic portal blood perfusion, and abnormal perfusion of cardiopulmonary system, CIPSVS may be the possible cause.Its treatment require individualization.更多还原