摘要
目的:探讨脐-门-体静脉分流(umbilical-portal-systemic venous shunts,UPSVS)的临床特征和预后。方法:本研究为回顾性队列研究。研究对象为2019年5月至2024年3月在广州医科大学附属妇女儿童医疗中心产前检查并出生的38例UPSVS患儿。分析这些患儿的一般情况、分型与自然病程、临床表现与干预,以及合并症等情况。采用独立样本t检验、Mann-Whitney U检验或χ2检验(或Fisher精确概率法)进行统计学分析。结果:(1)一般情况:38例患儿出生胎龄38(37~38)周,出生体重(2751±90)g,60.5%(23/38)为男婴。26.3%(10/38)合并胎儿生长受限,21.1%(8/38)伴静脉导管缺如,86.8%(33/38)接受了产前基因检测,其中4例存在基因异常(分别为BRAF、DNAH9、GATA4和PPP2R5D)。(2)分型与自然病程:肝内型和肝外型分别占81.6%(31/38)和2.6%(1/38),15.8%(6/38)出生后分流血管已闭合,无法分型。92.1%(35/38)患儿分流血管在1岁内自然闭合,闭合时间3(3~6)个月。(3)临床表现与干预:氧疗组的3月龄分流率[22.0%(0.0%~76.4%)与0.0%(0.0%~4.1%),Z=-2.29]、血氨[(78.4±19.2)与(59.8±18.5)μmol/L,t=-2.50]及住院时间[10(5~18)与5(3~7)d,Z=-2.00]均高于无氧疗组(P值均<0.05)。2例(5.3%)需介入治疗(肝外型和肝内型各1例,分流率分别为87.9%和50.0%),术后均恢复良好。(4)合并症:结构畸形发生率47.4%(18/38),其中6例需手术干预;保守治疗的病例中,以肝血管瘤和皮肤血管瘤(均为13.1%,5/38)为主。结论:UPSVS患儿以肝内型多见,常伴有多种结构畸形,但总体预后较好,大部分患儿在1岁内分流可自行闭合,但也有部分可能需要手术干预。
Objective To investigate the clinical features and prognosis of umbilical-portal-systemic venous shunts(UPSVS).Methods This retrospective cohort study involved 38 neonates with prenatally diagnosed UPSVS born at Guangzhou Women and Children's Medical Center from May 2019 to March 2024.Demographic data,types and the natural course of UPSVS,clinical manifestations,interventions,and complications were evaluated.Statistical analyses included independent t-test,Mann-Whitney U test,and Chi-square test/Fisher's exact test.Results(1)The cohort comprised 60.5%(23/38)males with a median gestational age of 38 weeks(37-38 weeks)and a mean birth weight of(2751±90)g.The main complications were fetal growth restriction(26.3%,10/38),and absence of the ductus venosus(21.1%,8/38).Prenatal genetic testing was performed on 86.8%(33/38)of the infants.Among them,genetic abnormalities were identified in four cases,involving the BRAF,DNAH9,GATA4,and PPP2R5Dgene,respectively.(2)Intrahepatic and extrahepatic UPSVS accounted for 81.6%(31/38)and 2.6%(1/38),respectively;spontaneous postnatal closure was observed in 15.8%(6/38)of the cases,making classification impossible.Spontaneous shunt closure occurred in 92.1%(35/38)of the cases within the first year of life,with a median closure time of 3 months(IQR:3-6 months).(3)Comparative analysis showed infants in the oxygen therapy group had significantly higher shunt rate[median:22.0%(0.0%-76.4%)vs.0.0%(0.0%-4.1%),Z=-2.29],elevated blood ammonia concentration[(78.4±19.2)vs.(59.8±18.5)μmol/L,t=-2.50],and prolonged hospitalization[10(5-18)vs.5(3-7)d,Z=-2.00]compared to those in the non-oxygen therapy group(all P<0.05).Two infants(5.3%),one extrahepatic type with a shunt rate of 87.9%and one intrahepatic type with a shunt rate of 50.0%,underwent successful interventional closure and recovered well.(4)Structural anomalies were observed in 47.4%(18/38)of the infants,with six requiring surgical correction.Conservative management is predominantly indicated for hepatic and cutaneous hemangiomas(13.1%and 5/38).ConclusionsUPSVS predominantly presents as intrahepatic shunts in pediatric patients and is frequently complicated by multiple structural abnormalities.Although some patients require surgical intervention,the overall prognosis is favorable as spontaneous shunt closure occurs in most cases within the first year of life.
作者
程映
吕俊健
王海玉
何秋明
钟微
Cheng Ying;Lyu Junjian;Wang Haiyu;He Qiuming;Zhong Wei(Department of Neonatal Surgery Intensive Care Unit,Guangzhou Women and Children's Medical Center,Guangzhou Medical University,Guangzhou 510623,China;Department of Ultrasound,Guangzhou Women and Children's Medical Center,Guangzhou Medical University,Guangzhou 510623,China)
出处
《中华围产医学杂志》
2026年第1期47-51,共5页
Chinese Journal of Perinatal Medicine
基金
广州市重大疑难(罕见)疾病项目(2024MDRD17)。
关键词
脐静脉
门体分流术
胎儿
婴儿
新生
预后
Umbilical veins
Portasystemic shunt,surgical
Fetus
Infant,newborn
Prognosis
