新生儿先天性肝动静脉瘘:病例报告及文献复习  

Congenital hepatic arteriovenous fistula in neonates:A report of two cases and literature review

作　　者：关瑞莲 孙川川 林土莲 张磊 唐建平 李易娟[4] Guan Ruilian;Sun Chuanchuan;Lin Tulian(Department of Neonatology,Women and Children's Medical Center,Guangzhou 510000,Guangdong Province,China)

机构地区：[1]广州市妇女儿童医疗中心新生儿科,510000 [2]广州市妇女儿童医疗中心增城院区新生儿科,510000 [3]广州市妇女儿童医疗中心新生儿外科,510000 [4]中山大学附属第一医院儿科

出　　处：《实用肝脏病杂志》2023年第5期757-760,共4页Journal of Practical Hepatology

基　　金：广东省卫生健康委员会医学科研基金资助项目(编号:A2019376)。

摘　　要：本文报道了2例不同类型的新生儿先天性肝动静脉瘘(HAVF)罕见病例。1例为血管型HAVF,产前未能发现胎儿腹部血管异常。在出生后28 h因突发心源性休克和肺动脉高压危象而被诊断,另1例为肿瘤型HAVF,产前早期超声检查发现胎儿腹部血管瘤,并予以连续跟踪,在出生后2 d出现心力衰竭和肺动脉高压症状。2例患儿均使用腹部血管彩色多普勒超声和腹部强化CT检查诊断,均给予强心、利尿、口服普萘洛尔对症治疗,1 w后肺动脉压逐渐下降,2~3 w后出院。随访10 m,影像学检查显示2例患儿HAVF逐渐消失,血管瘤体缩小,生长发育正常,未见不良事件发生。本文结合相关文献复习,对先天性HAVF的病理生理、临床特点、诊断方法和治疗选择进行了分析和总结,以期提高新生儿科医师对该病的诊治水平。This article reported two rare cases of different types of neonatal congenital hepatic arteriovenous fistula(HAVF).One case was vascular HAVF,and abdominal vascular abnormalities of the fetus could not be found before birth,and cardiogenic shock and pulmonary hypertension crisis occurred 28 hours after birth.After continuous follow-up,he developed symptoms of heart failure and pulmonary hypertension 2 days after birth.Another fetus had tumorous HAVF.Both children were diagnosed by abdominal vascular color Doppler ultrasonography and abdominal enhanced CT scan,and were given inotropic diuresis and oral propranolol for symptomatic treatment.After 1 week,the pulmonary arterial pressure gradually decreased,and they were discharged after 2 to 3 week medical cares.During the follow-up of more than 10 months,the imaging examination showed that the HAVF of the two children gradually disappeared,the hemangioma shrank,the growth and development were normal,and no adverse events occurred.At the discussion of this article,the pathophysiology,clinical features,diagnostic methods and treatment options in fetus with congenital HAVF were given at base of literature review,and we hope it might improve neonatologists'awareness of the disease.

关 键 词：先天性动静脉瘘 肝脏 诊治 新生儿 

分 类 号：R722.1[医药卫生—儿科] R445.1[医药卫生—临床医学]