Selective Hepatic Artery Ligation for Hepatic Haemangioendothelioma: Case Report and Review of the Literature

R. Bachmann; B. Genin; P. Bugmann; D. Belli; S. Hanquinet; P. Liniger; C. Le Coultre

doi:10.1055/s-2003-42245

European Journal of Pediatric Surgery, Inhaltsverzeichnis

Eur J Pediatr Surg 2003; 13(4): 280-284
DOI: 10.1055/s-2003-42245

Case Report

Georg Thieme Verlag Stuttart, New York · Masson Editeur Paris

Selective Hepatic Artery Ligation for Hepatic Haemangioendothelioma: Case Report and Review of the Literature

R. Bachmann¹ , B. Genin² , P. Bugmann² , D. Belli¹ , S. Hanquinet³ , P. Liniger² , C. Le Coultre²

¹Paediatric Department, University Children's Hospital, Geneva, Switzerland
²Paediatric Surgery Department, University Children's Hospital, Geneva, Switzerland
³Paediatric Radiology Department, University Children's Hospital, Geneva, Switzerland

Abstract

Although benign, hepatic haemangioendotheliomas (HHE) are rare vascular tumours of the infant which have a high mortality rate secondary to high output congestive heart failure. The management of these tumours is still controversial and none of the different medical or surgical options has been unanimously accepted. We report the case of a neonate with congestive heart failure (CHF) due to a localized HHE, treated successfully by selective ligation of the left hepatic artery branch irrigating the tumour, under perioperative ultrasound Doppler control. A review of the literature showed 35 cases of HHE treated by hepatic artery ligation (HAL) with a survival rate of 80 %.

Résumé

Malgré leur nature bénigne, les hémangio-endothéliomes hépatiques (HEH) sont de rares tumeurs vasculaires du nourrisson induisant un taux de mortalité élevé en raison de l'insuffisance cardiaque à haut débit fréquemment associée. La prise en charge de ces tumeurs est encore controversée et aucun traitement médical ou chirurgical ne fait l'unanimité. Nous rapportons le cas d'un nouveau-né avec insuffisance cardiaque secondaire à un HEH localisé, traité avec succès par une ligature sélective d'une branche de l'artère hépatique gauche irrigant la tumeur, sous contrôle d'un ultrason doppler per-opératoire. Une revue de la littérature nous permet de dénombrer 35 cas d'HEH traités par ligature de l'artère hépatique avec un taux de survie global de 80 %.

Resumen

Aunque el hemangioendotelioma hepático (HHE) es un tumor vascular benigno raro del lactante muy joven puede producir una alta mortalidad por insuficiencia cardiaca congestiva hoperdinámica. El tratamiento de estos tumores es controvertido y ninguna de las opciones médicas y quirúrgicas es unánimemente aceptada. Presentamos el caso de un recién nacido con insuficiencia cardiaca congestiva debida a un HHE localizado que se trató con ligadura selectiva bajo control ultrasónico doppler preoperatorio de la arteria hepática izquierda que irrigaba al tumor. Una revisión de la literatura muestra 35 casos de HHE tratados con ligadura de la arteria hepática con una sobrevivencia global de 80 %.

Zusammenfassung

Hepatische Hämangioendotheliome (HHE) sind seltene vaskuläre Tumoren der Leber des Säuglings, die, obwohl benign, für eine sehr hohe Mortalität verantwortlich sind durch die sekundäre kongestive Herzinsuffizienz. Die Behandlungsmöglichkeiten sind sehr zahlreich, aber keine ist erfolgreich genug, um generell angenommen zu sein. Wir berichten von einem Neugeborenen mit einem lokalisierten HHE und sekundärer Herzinsuffizienz, das durch eine selektive Bindung des Zweiges der Arteria hepatica, die den Tumor vaskularisierte, unter peroperativer Doppler-Ultraschall-Kontrolle, geheilt wurde. In einer extensiven Literaturanalyse zählten wir 35 Fälle von HHE, die durch eine Bindung der Arteria hepatica behandelt wurden und von denen 80 % überlebt haben.

Key words

Infantile hepatic haemangioendothelioma - congestive heart failure - hepatic artery ligation

Mots-clés

Hémangioendothéliome hépatique du nourrisson - insuffisance cardiaque congestive - ligature artère hépatique

Palabras clave

Hemangioendotelioma hepático del lactante - insuficiencia cardiaca congestiva - ligadura de la arteria hepática

Schlüsselwörter

Hämangioendotheliome der Leber im Säuglingsalter - kongestive Herzinsuffizienz - Bindung der Arteria hepatica

Volltext

Referenzen

References

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Dr. Bernard Genin

Clinique de Chirurgie Pédiatrique, Hôpital des Enfants

6, rue Willy-Donzé

1211 Genève 14

Switzerland

eMail: bernard.genin@hcuge.ch