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[多发性肝血管瘤:新生儿心脏停搏的罕见原因。1例病例及文献复习]

[Multinodular hepatic angioma: a rare cause of neonatal asystole. A case and review of the literature].

作者信息

Lefèvre M, Bouhour J B, Petitier H, Leborgne J, De Berranger P, Alix D

出版信息

Arch Mal Coeur Vaiss. 1977 Nov;70(11):1197-202.

PMID:414677
Abstract

The clinical findings in a case with a diffuse multinodular type of angioma of the liver are reported. The clinical picture was typical: the appearance after the first week of a severe congestive failure mimicking a congenital heart disorder, of the left to right shunt type, with gross hepatomegaly of the "vascular" type with a systolo-diastolic murmur, but with no cutaneous evidence of angioma. The very severe prognosis during the first months of life of patients with this malformation, as illustrated by the 58 deaths out of the 78 cases reported in the literature, indicated ligature of the hepatic artery before the age of one month. The satisfactory outcome (with a nine months follow-up) for this case, as for the three other operated cases in the literature, leads us to conclude that this operation is indicated after a detailed arteriographic examination has been made, and provided that the other anatomical conditions are favourable.

摘要

报告了一例弥漫性多结节型肝血管瘤的临床病例。临床表现典型:在第一周后出现严重的充血性心力衰竭,类似先天性心脏病,为左向右分流型,伴有“血管性”肝肿大,可闻及收缩期-舒张期杂音,但无皮肤血管瘤表现。文献报道的78例患者中,有58例死亡,这表明这种畸形的患者在出生后第一个月内预后极差,提示应在1个月龄前结扎肝动脉。该病例以及文献中其他3例手术病例的良好预后(随访9个月)使我们得出结论,在进行详细的血管造影检查后,且其他解剖条件有利时,应进行该手术。

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