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Intrahepatic portosystemic shunts, from prenatal diagnosis to postnatal outcome: a retrospective study
Or Steg Saban1,2, Tal Weissbach2,3, Reuven Achiron2,3
1Pediatrics B Department, Sheba Medical Center, Ramat-Gan, Israel.
Insights
Congenital intrahepatic portosystemic shunts (IHPSS) diagnosed prenatally typically resolve spontaneously. While most children with IHPSS survive, some may experience failure to thrive or developmental delays, especially those with multiple shunts.
Area of Science:
- Pediatric Gastroenterology
- Fetal Medicine
- Vascular Malformations
Background:
- Congenital intrahepatic portosystemic shunts (IHPSS) are rare vascular anomalies where blood bypasses the liver.
- Prenatal diagnosis of IHPSS is uncommon, and outcomes for these cases are not well-documented.
- Existing research often focuses on symptomatic, postnatally diagnosed patients.
Purpose of the Study:
- To investigate the natural course and outcomes of IHPSS diagnosed prenatally.
- To compare postnatal outcomes between patients with single versus multiple intrahepatic shunts.
Main Methods:
- Retrospective analysis of fetal IHPSS cases diagnosed via ultrasound (2006-2019) at a single tertiary center.
- Prospective follow-up of diagnosed cases at a pediatric gastroenterology unit.
- Comparison of outcomes based on the number of intrahepatic shunts (single vs. multiple).
Main Results:
- Twenty-six children were included; 77% experienced growth restriction.
- Spontaneous shunt closure occurred in 96% of patients by a median age of 7.5 months.
- Failure to thrive (FTT) was more common in those with multiple shunts (62.5% vs. 16.7%).
- All patients survived with minimal sequelae; mild developmental delay was noted in 26.9%.
Conclusions:
- Prenatally diagnosed IHPSS generally close spontaneously within two years.
- Children with prenatally detected IHPSS may face risks of FTT and mild developmental delay.
- Regular pediatric gastroenterology surveillance is recommended for these patients.
Objective:
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.
Methods And Design:
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.
Results:
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.
Conclusions:
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.
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