Abstract：
We analyzed the characteristics of the population with congenital portosystemic shunt diagnosed during the antenatal period and the organization of their perinatal care. This multicentric retrospective study included all the patients with a prenatal diagnosis of congenital portosystemic shunt. Between 1999 and 2015, 12 patients were included. Prenatal diagnosis was done at a median 26.5 weeks of gestation (21-34). All the patients presented intrahepatic CPSS, three of them had associated congenital cardiopathy, and one a Bannayan-Zonana syndrome. Ten patients had simple outcome on conservative treatment, eight of them having a spontaneous closure of their portosystemic shunt within the first 2 years of life. One patient had surgical treatment which failed and he developed a focal nodular hyperplasia. Another patient had radiological interventional closure of his shunt which was complicated by a venal portal thrombosis.
CONCLUSIONS: Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases. What is known: • Multiples studies exist on congenital porto systemic shunt but when the diagnosis is done after birth. • The evolution, management, and complication are well known. What is new: • There is very few studies with only patients diagnosed in antenatal and it is a large series of cases. • Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases.
CONCLUSIONS: Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases. What is known: • Multiples studies exist on congenital porto systemic shunt but when the diagnosis is done after birth. • The evolution, management, and complication are well known. What is new: • There is very few studies with only patients diagnosed in antenatal and it is a large series of cases. • Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases.
摘要：
我们分析了产前诊断为先天性门体分流的人群的特征及其围产期护理的组织。这项多中心回顾性研究包括所有产前诊断为先天性门体分流术的患者。在1999年至2015年期间，纳入了12名患者。产前诊断是在中位妊娠26.5周（21-34）进行的。所有患者均出现肝内CPSS，其中三人患有先天性心脏病，还有一个是Bannayan-Zonana综合征.10例患者的保守治疗结果简单，其中8人在生命的前2年内自发关闭了门体分流。一名患者的手术治疗失败，他发展为局灶性结节增生。另一名患者的分流管放射学介入闭合，并发静脉门静脉血栓形成。
结论：产前诊断的肝内门体分流术的结果在大多数病例中是良好的。已知的内容:•存在关于先天性门体分流术的多次研究,但是当诊断在出生后完成时。•Theevolution,管理,并发症是众所周知的。新的内容：•很少有研究仅在产前诊断的患者，这是一个大系列的案例。•在大多数情况下，产前诊断的肝内门体分流术的结果良好。
结论：产前诊断的肝内门体分流术的结果在大多数病例中是良好的。已知的内容:•存在关于先天性门体分流术的多次研究,但是当诊断在出生后完成时。•Theevolution,管理,并发症是众所周知的。新的内容：•很少有研究仅在产前诊断的患者，这是一个大系列的案例。•在大多数情况下，产前诊断的肝内门体分流术的结果良好。
