Service de chirurgie pédiatrique, Hôpital de La Timone, 13385 Marseille, France.
J Pediatr Surg. 2012 Aug;47(8):1490-5. doi: 10.1016/j.jpedsurg.2012.02.022.
The objective of this study is to describe the evolution of 8 cases of congenital malformations of the umbilical-portal-hepatic venous system diagnosed before the first month of life.
All cases of congenital malformation of the portal and hepatic venous system diagnosed prenatally or during the first month of life in our institution were systematically reviewed since November 2000. Clinical features, imaging, and anatomical findings were reviewed, focusing primarily on clinical and radiologic evolution.
Eight cases of congenital malformation of the umbilical-portal-hepatic venous system were studied. Fifty percent of these malformations were diagnosed prenatally. We report 4 portosystemic shunts. Three involuted spontaneously, and the fourth one required surgical treatment. We report a variation of the usual anatomy of portal and hepatic veins that remained asymptomatic, an aneurysmal dilatation of a vitelline vein causing portal vein thrombosis that needed prompt surgical treatment with good result, a complex portal and hepatic venous malformation treated operatively, and a persistent right umbilical vein that remained asymptomatic.
Prenatal diagnosis of malformations of the umbilical-portal-hepatic venous network is uncommon. Little is known about the postnatal prognosis. Clinical, biologic, and radiologic follow-up by ultrasonography is essential to distinguish pathologic situations from normal anatomical variants.
本研究旨在描述 8 例在生命第一个月前诊断出的先天性脐门静脉肝静脉系统畸形的演变过程。
自 2000 年 11 月以来，我们对我院所有产前或生命第一个月内诊断出的先天性门静脉和肝静脉系统畸形的病例进行了系统回顾。回顾了临床特征、影像学和解剖学发现，主要关注临床和影像学的演变。
研究了 8 例先天性脐门静脉肝静脉系统畸形。这些畸形中有 50%是产前诊断的。我们报告了 4 例门体分流。其中 3 例自发消退，第 4 例需要手术治疗。我们报告了一种门静脉和肝静脉通常解剖结构的变异，这种变异一直无症状，脐静脉的动脉瘤样扩张导致门静脉血栓形成，需要及时手术治疗，结果良好，还报告了一种复杂的门静脉和肝静脉畸形，需要手术治疗，以及一条持续存在的无症状的右脐静脉。
脐门静脉肝静脉网络畸形的产前诊断并不常见。对其出生后的预后知之甚少。通过超声进行临床、生物学和影像学的随访是必要的，以区分病理性情况和正常解剖变异。
