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Archives de pédiatrie
Volume 18, n° 2
pages 149-152 (février 2011)
Doi : 10.1016/j.arcped.2010.11.007
Received : 2 August 2009 ;  accepted : 24 November 2010
Les fentes labio-palatines médianes : un diagnostic qui en cache un autre
Medial cleft lip: One diagnosis masking another
 

N. Mansouri Hattab , S. Lahmiti, A. Bouaichi, A. Hiroual, M. El Bouihi, T. Fikry
Unité de chirurgie maxillo-faciale et esthétique, service de traumatologie A, CHU Mohammed VI, université Cadi Ayyad, Marrakech, Maroc 

Auteur correspondant. Service de chirurgie maxillo-faciale et esthétique, hôpital Ibn-Tofail, quartier Guéliz, 40000, Marrakech, Maroc.
Résumé

Les fentes labio-palatines medianes représentent l’une des malformations faciales entrant dans le cadre des holoprosencéphalies (HPE) et imposent un bilan neuro-morphologique et malformatif complet. Nous rapportons 2 cas de fentes labio-palatines médianes et discutons les particularités de l’HPE. La première observation rapporte le cas d’un nouveau-né vu à la naissance et présentant une fente labio-alvéolaire médiane associée à une dysmorphie crânio-faciale complexe. Le bilan malformatif révélait l’absence de structures cérébrales médianes ainsi qu’une trisomie 13. Le bébé décédait à 48h de vie. Le second cas était celui d’un nouveau-né de 28 j présentant une fente labio-palatine médiane complète associée à une dysmorphie crânio-faciale. Le bilan malformatif était négatif. L’échographie transfontanellaire ne révélait pas d’anomalie de la ligne médiane. L’HPE est une malformation crânio-faciale rare et grave. Elle peut être isolée ou entrant dans le cadre de syndrome malformatif. Le bilan malformatif doit être complet. Le traitement chirurgical est réservé aux formes viables. Le conseil génétique pour les formes héréditaires reste la seule prévention possible.

The full text of this article is available in PDF format.
Summary

Median cleft lip and palate are one of the facial manifestations of holoprosencephaly, a brain malformation resulting from a defect in the development of the prosencephalon, the embryonic precursor of the forebrain. It can occur in isolation or as part of a broader pattern of malformation in many different syndromes. The diagnosis of holoprosencephaly requires a complete malformative assessment.

Case report

First case: a newborn baby seen at birth for a median cleft lip associated with a complex craniofacial malformation. The search for malformations revealed the absence of the median cerebral structure and trisomy 13. The baby died at 48h.

Second case

A 28-day-old newborn baby consulting for a median cleft lip and palate. The brain ultrasounds showed no anomalies of the median cerebral structure. The malformative assessment revealed no other malformation.

Discussion

Holoprosencephaly is a complex developmental brain malformation. From the advances in neuroimaging and genetics, our understanding of the etiology and pathogenesis of this condition has advanced dramatically. This advance should lead to improved management of common medical complications and more optimal family counseling. Surgical treatment is the preferred therapy for viable malformations.

The full text of this article is available in PDF format.


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