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Outcome of intrahepatic portosystemic shunt diagnosed prenatally

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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.

Conclusion: 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.

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Abbreviations

CPSS:

congenital porto-systemic shunt

IUGR:

intrauterine growth retardation

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Author information

Authors and Affiliations

  1. Service de réanimation néonatale du CHU de Nice, Nice, France

    Bérengère Francois & Stéphanie De Smet

  2. Service d’hépatologie gastro-entérologie et nutrition pédiatrique du CHRU de Lille, Lille, France

    Fréderic Gottrand

  3. Service d’hépatologie gastro-entérologie et nutrition pédiatrique du CHU de Lyon, Bron Cedex, France

    Alain Lachaux

  4. Service de radiologie pédiatrique des Hôpital Lenval, CHU de Nice, Nice, France

    Corinne Boyer

  5. Service maternité du Centre Hospitalier Princesse Grace, Monaco-Ville, Monaco

    Bernard Benoit

Authors
  1. Bérengère Francois
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  2. Fréderic Gottrand
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  3. Alain Lachaux
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  4. Corinne Boyer
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  5. Bernard Benoit
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  6. Stéphanie De Smet
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Contributions

Conception and design: Bérengère Francois, Stéphanie De Smet

Provision of study materials and patients: Bérengère Francois, Stéphanie De Smet, Frédéric Gottrand, Alain Lachaux, Bernard Benoit, Corinne Boyer

Data interpretation: Bérengère Francois, Stéphanie De Smet, Corinne Boyer, Bernard Benoit

Manuscript writing: Bérengère Francois, Stéphanie De Smet, Corinne Boyer, Bernard Benoit

Corresponding author

Correspondence to Bérengère Francois.

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The authors declare that they have no conflict of interest.

Research involving human participants

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Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Additional information

Communicated by Peter de Winter

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Francois, B., Gottrand, F., Lachaux, A. et al. Outcome of intrahepatic portosystemic shunt diagnosed prenatally. Eur J Pediatr 176, 1613–1618 (2017). https://doi.org/10.1007/s00431-017-3013-x

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  • DOI: https://doi.org/10.1007/s00431-017-3013-x

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