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Les cholestases néonatales

Neonatal cholestasis

Revue de médecine périnatale volume 2, pages 188–196 (2010)Cite this article

Résumé

Tout ictère néonatal persistant au-delà de deux semaines de vie impose un diagnostic étiologique urgent, avec un premier bilan comportant au minimum un examen de la couleur des selles, et un dosage de la bilirubine totale et conjuguée. En cas de cholestase néonatale (CN) confirmée, l’hypovitaminose K doit être systématiquement prévenue par l’injection de vitamine K1, et l’enfant doit être adressé rapidement dans un centre spécialisé. L’atrésie des voies biliaires (AVB) est la première cause de CN. Son diagnostic est urgent, car les chances de succès du traitement conservateur du foie natif (intervention de Kasai ou ses variantes) décroissent rapidement avec l’âge de l’enfant. Une échographie hépatique normale n’exclut pas une AVB. Le diagnostic peut souvent être suspecté avant l’intervention et sera confirmé par les constatations opératoires complétées si nécessaire par une cholangiographie. En cas d’échec de rétablissement du flux biliaire après l’intervention de Kasai, la cirrhose biliaire continue de progresser et conduit à la transplantation hépatique, souvent dans les premières années de vie. Les autres causes de cholestases obstructives sont la lithiase de la voie biliaire principale (VBP), le kyste du cholédoque et la perforation spontanée des voies biliaires. Là encore, la levée de l’obstacle biliaire (par chirurgie ou radiologie interventionnelle) est urgente, afin d’éviter l’évolution vers la fibrose hépatique et la cirrhose, très rapide dans les premiers mois de vie. Les principales étiologies médicales de CN sont: le syndrome d’Alagille, le déficit en alpha-1-antitrypsine, les cholestases familiales fibrogènes et la mucoviscidose. Les autres causes médicales de CN sont multiples, certaines pouvant nécessiter un traitement spécifique: infectieuses, endocriniennes, nutritionnelles, métaboliques ou maladies de surcharge.

Abstract

Neonatal jaundice lasting more than two weeks needs urgent investigations, starting with examination of stool colour and blood tests with total and conjugated serum bilirubin. If neonatal cholestasis (NC) is confirmed, vitamin K should be immediately injected, and the child should be referred to a specialised centre for investigations and treatment. Biliary atresia (BA) is the first cause of NC. Its diagnosis is urgent, since the chance of success of the conservative surgical treatment (Kasai operation or variants) decreases rapidly as the age at surgery increases. Normal ultrasound scans cannot rule out BA. The diagnosis can often be suspected before surgery, and is confirmed by operative findings with or without cholangiogram. In case of failure to restore the biliary drainage, biliary cirrhosis progresses and leads to liver transplantation, generally in the first years of life. The other causes of neonatal obstructive jaundice are stones in the main bile duct, choledocal cyst and spontaneous perforation of bile ducts. Correction of biliary obstruction (by surgery or interventional radiology) is also urgent to prevent progression of liver fibrosis, which is very rapid in the first months of life. The most common medical aetiologies of NC are: Alagille syndrome, 1-antitrypsin deficiency, progressive familial intrahepatic cholestasis and cystic fibrosis. Other medical causes of NC are numerous, including infectious, hormonal, nutritional, metabolic or storage diseases. Some of the conditions may require a specific therapy.

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Authors and Affiliations

  1. Groupe médicochirurgical d’hépatologie pédiatrique, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, F-75015, Paris, France

    D. Debray & C. Chardot

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  1. D. Debray
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  2. C. Chardot
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Correspondence to D. Debray or C. Chardot.

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Debray, D., Chardot, C. Les cholestases néonatales. Rev. med. perinat. 2, 188–196 (2010). https://doi.org/10.1007/s12611-010-0081-9

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  • DOI: https://doi.org/10.1007/s12611-010-0081-9

Mots clés

  • Cholestase néonatale
  • Diagnostic
  • Traitement
  • Chirurgie
  • Pronostic

Keywords

  • Neonatal cholestasis
  • Diagnosis
  • Treatment
  • Surgery
  • Prognosis