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Original research
Intrahepatic portosystemic shunts, from prenatal diagnosis to postnatal outcome: a retrospective study
  1. Or Steg Saban1,2,
  2. Tal Weissbach2,3,
  3. Reuven Achiron2,3,
  4. Marina Pekar Zlotin2,4,
  5. Yael Haberman2,5,
  6. Adi Anis Heusler6,7,
  7. Eran Kassif2,3,
  8. Batia Weiss2,5
  1. 1 Pediatrics B Department, Sheba Medical Center, Ramat-Gan, Israel
  2. 2 Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
  3. 3 Obstetrics and Gynecology, Sheba Medical Center, Tel Hashomer, Israel
  4. 4 Department of Obstetrics and Gynecology, Yitzhak Shamir Medical Center Assaf Harofeh, Tzrifin, Israel
  5. 5 Pediatric Gastroenterology and Nutrition Unit, Sheba Medical Center, Tel Hashomer, Israel
  6. 6 Department of OBGYN, Laniado Hospital, Netanya, Israel
  7. 7 The Adelson School of Medicine, Ariel University, Ariel, Israel
  1. Correspondence to Dr Batia Weiss, Pediatric Gastroenterology and Nutrition, Sheba Medical Center, Tel Hashomer, 5262000, Israel; batya.vais{at}sheba.health.gov.il

Abstract

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.

  • Gastroenterology
  • Neonatology
  • Paediatrics

Data availability statement

All data relevant to the study are included in the article or uploaded as supplemental information.

https://doi.org/10.1136/archdischild-2023-325424

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    Data availability statement

    All data relevant to the study are included in the article or uploaded as supplemental information.

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    Footnotes

    • OSS and TW contributed equally.

    • Contributors OSS was involved in the conception of work, created data collection plan, interpreted the data and wrote the original draft. TW was involved in the conception of work, created data collection plan, interpreted the data, performed formal analysis and wrote the original draft. RA supervised and guided the data collection, and gave his final approval for the manuscript. MPZ and AAH were part of the data collection team. YH reviewed the manuscript and gave her final approval of the manuscript. BW was involved in the conception of the work and creating the data collection plan. She supervised and revised the manuscript. OS is guarantor.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.