Pediatric Radiology, Hopital Bicêtre, 78 Rue du Général Leclerc, Le Kremlin Bicêtre 94275, France.
Pediatr Radiol. 2012 Mar;42(3):298-307. doi: 10.1007/s00247-011-2214-0. Epub 2011 Sep 18.
The clinical presentation of foetal hepatic haemangioma (HH) is highly variable, from asymptomatic to life-threatening.
The aim of this study was to describe foetal hepatic haemangioma and identify prognostic factors.
Antenatal and postnatal imaging studies, clinical and biological records of infants with antenatally diagnosed HH (2001-2009) were reviewed.
Sixteen foetuses had one focal lesion, with a mean volume of 75 ml (5-240 ml). One had multifocal HH. Most presented as a focal well-delimited heterogeneous vascular mass. Four had associated cardiomegaly, five had cardiac failure. Eight of the nine foetuses with cardiac disorders were symptomatic at birth: cardiac failure with pulmonary hypertension (9), consumptive coagulopathy (8), compartmental syndrome (2). All received supportive medical treatment, four embolisation. Five of these died. The remaining eight had a normal cardiac status. Two became symptomatic after birth: one with a large porto-hepatic shunt and one with significant mass effect. Prenatal cardiac abnormality (univariate, P = 0.031), enlargement of more than one hepatic vein (P = 0.0351) and large volume (P = 0.0372) were associated with symptomatic disease.
Hepatic haemangioma associated with prenatal cardiac disorders, large volume and more than one enlarged hepatic vein have poorer outcome and require specific perinatal multidisciplinary management.
胎儿肝脏血管瘤(HH)的临床表现差异很大,从无症状到危及生命。
本研究旨在描述胎儿肝脏血管瘤并确定其预后因素。
回顾了 2001 年至 2009 年间经产前诊断为 HH 的婴儿的产前和产后影像学研究、临床和生物学记录。
16 例胎儿有 1 个局灶性病变,平均体积为 75ml(5-240ml)。1 例为多灶性 HH。大多数表现为局灶性界限清楚的混杂性血管肿块。4 例伴有心脏增大,5 例有心力衰竭。在 9 例有心脏疾病的胎儿中,8 例在出生时出现症状:肺动脉高压性心力衰竭(9 例)、消耗性凝血病(8 例)、间隔综合征(2 例)。所有患儿均接受支持性药物治疗,4 例行栓塞治疗。其中 5 例死亡。其余 8 例心脏功能正常。2 例在出生后出现症状:1 例存在大的门体分流,1 例存在明显的肿块效应。产前心脏异常(单因素,P=0.031)、1 条以上肝静脉扩张(P=0.0351)和大体积(P=0.0372)与症状性疾病相关。
与产前心脏疾病、大体积和 1 条以上扩张的肝静脉相关的肝脏血管瘤预后较差,需要进行特定的围产期多学科管理。