Abstract：
Congenital intrahepatic portosystemic shunts (IHPSS) are rare vascular malformations resulting in blood bypassing the liver to the systemic circulation. Previous studies included symptomatic patients diagnosed postnatally, but the outcome of IHPSS diagnosed prenatally is rarely reported. We present a cohort of children prenatally diagnosed with IHPSS and report their natural course and outcome.
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.
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.
摘要：
目的：先天性肝内门体分流术（IHPSS）是罕见的血管畸形，导致血液绕过肝脏进入体循环。以前的研究包括产后诊断的有症状的患者，但产前诊断的IHPSS的结果很少报道。我们介绍了一组产前诊断为IHPSS的儿童，并报告了他们的自然病程和结局。
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。
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。
