摘要目的:探讨新生儿肝血管瘤伴动静脉瘘（hepatic hemangioma with arteriovenous fistula，HH-AVF）的临床特征及诊疗情况。方法:选择2020年1月至2024年1月郑州大学附属儿童医院新生儿科收治的HH-AVF患儿临床资料进行回顾性分析。结果:共纳入HH-AVF患儿4例，男3例，女1例，出生体重（3 430±221）g，起病日龄（16.8±13.1）d，均有肝脏占位病变。2例患儿呼吸困难需呼吸机支持，其中1例心影增大，并发心力衰竭、肺动脉高压，另1例并发肺动脉高压；1例患儿并发全身皮肤多发血管瘤；1例患儿并发颈血管瘤并反复出血。4例均接受介入治疗，介入治疗时日龄（34.0±22.5）d，同时口服普萘洛尔，随访4~16个月，复查CT均显示病灶较确诊时显著减小，未再出现呼吸困难、心力衰竭、肺动脉高压等表现，监测肝功能、凝血功能等未发现异常。结论:新生儿期起病的HH-AVF多为先天性，临床表现不一，发生心力衰竭、肺动脉高压等并发症时极为凶险，介入治疗效果显著，可有效降低病死率。

abstractsObjective:To study the clinical characteristics and management of neonatal hepatic hemangioma with arteriovenous fistula (HH-AVF).Methods:This study retrospectively analyzed four cases of neonatal HH-AVF admitted to Children's Hospital affiliated with Zhengzhou University from January 2020 to January 2024, focusing on their clinical manifestations, laboratory findings, treatment approaches, and long-term follow-up outcomes.Results:Among the four cases, there were three male and one female newborns, with a birth weight of (3 430±221) g. All cases presented with space-occupying lesions in the liver. Two newborns experienced dyspnea requiring ventilator support; one of them was complicated with heart failure, pulmonary hypertension, and an enlarged cardiac shadow, while the other had pulmonary hypertension. One newborn developed multiple skin hemangiomas, and another presented with cervical hemangiomas with recurrent bleeding. All four newborns with HH-AVF received interventional therapy at an average age of (34.0±22.5) d, along with oral propranolol. During the follow-up period (4-16 months), the reexamination CT showed significant reduction compared to the initial diagnosis. Reoccurrence of symptoms such as dyspnea, heart failure, or pulmonary hypertension was not observed. Monitoring of liver function, coagulation function, and overall growth and development were normal compared to peers of the same gender and age.Conclusions:HH-AVF in the neonatal period is predominantly congenital and presents with variable clinical manifestations. Complications such as heart failure and pulmonary hypertension are particularly dangerous. The application of interventional therapy has demonstrated remarkable efficacy and a substantial reduction in fatality rates.