This was a retrospective study of all fetal cases diagnosed by ultrasound with IHPSS between 2006 and 2019 at a single tertiary centre which were prospectively followed up at the paediatric gastroenterology unit. The postnatal outcome was compared between patients with a single versus multiple intrahepatic shunts.
Twenty-six patients (70.3% boys) were included in the study, of them, eight (30.8%) patients had multiple intrahepatic shunts. The median gestational age at diagnosis was 29.5 weeks. Growth restriction affected 77% of the cohort. Postnatally, spontaneous shunt closure occurred in 96% of patients at a median age of 7.5 months (IQR 2.2-20 months). Failure to thrive (FTT) and mild developmental delay were observed in eight (30.8%) and seven (26.9%) patients, respectively. FTT was significantly more prevalent in patients with multiple shunts compared with patients with a single shunt (62.5% vs 16.7%, p=0.02); however, the rate of shunt closure and age at time of closure were similar between these groups. All patients survived with limited to no sequelae.
IHPSS usually close spontaneously by 2 years of age. Children with prenatally detected IHPSS may develop FTT and mild developmental delay. Close surveillance at a paediatric gastroenterology unit may be beneficial.
方法:这是一项回顾性研究,对2006年至2019年在一个三级中心通过IHPSS超声诊断的所有胎儿病例进行了回顾性研究,并在儿科胃肠病科进行了前瞻性随访。比较了单个肝内分流和多个肝内分流的患者的产后结局。
结果:研究中纳入了26例患者(70.3%为男孩),其中,8例(30.8%)患者有多个肝内分流。诊断时的中位胎龄为29.5周。生长限制影响了77%的队列。出生后,96%的患者发生自发性分流闭合,中位年龄为7.5个月(IQR2.2-20个月).在8例(30.8%)和7例(26.9%)患者中观察到茁壮成长失败(FTT)和轻度发育迟缓。分别。与单分流患者相比,多分流患者的FTT明显更普遍(62.5%vs16.7%,p=0.02);然而,这些组的分流闭合率和闭合时的年龄相似.所有患者均存活,无后遗症。
结论:IHPSS通常在2岁时自发关闭。产前检测到IHPSS的儿童可能会出现FTT和轻度发育迟缓。儿科胃肠病科的密切监测可能是有益的。