MALFORMATIONS CONGÉNITALES

Toute anomalie constitutionnelle qui apparaît dès la naissance d’un individu constitue ce qu’on appelle une malformation congénitale.

Ces anomalies peuvent être extrêmement variables. Par exemple, il peut s’agir d’une simple incurvation du petit doigt ; mais une absence de cerveau peut survenir qui entraîne la mort immédiate.

On distingue donc traditionnellement, d’une part, les malformations majeures – qui sont incompatibles avec la vie ou qui handicapent plus ou moins lourdement l’individu –, et, d’autre part, les malformations mineures, qui se révèlent sans conséquence sérieuse.

Il est clair que la limite entre ces deux groupes d’anomalies est souvent très difficile à établir. C’est pourtant ce que l’on a tenté de faire dans le présent article, en limitant la question aux seules malformations majeures.

L’incidence sociale qu’exercent les malformations congénitales est déjà considérable par le nombre des cas, leur gravité, et du fait qu’il convient de réaliser une prophylaxie de ces anomalies faisant appel à la protection contre les radiations ionisantes (radioactivité et rayons X) et contre les substances toxiques souvent utilisées à des fins médicales, mais risquant de léser l’enfant avant sa naissance.

Fréquence

Chercher à évaluer la fréquence des malformations congénitales, c’est se heurter à un grand nombre de difficultés d’ordre diagnostique et d’ordre pratique (valeur des statistiques). Il semble néanmoins raisonnable d’admettre qu’au moins 3 p. 100 des naissances sont grevées d’une malformation majeure qui peut, dans certains cas, ne se manifester que plus tard au cours de la vie. Cette fréquence paraît constante quelle que soit la race, le milieu social, etc. Le seul facteur de variabilité paraît être le sexe : le garçon semble plus vulnérable que la fille. La consanguinité des parents augmente les risques, en particulier celui de malformations complexes.

La fréquence des décès par malformations congénitales par rapport à la mortalité infantile globale varie selon les pays. Elle semble néanmoins inversement proportionnelle à la mortalité par causes exogènes (carence, infections). En France, elle représente environ 10 p. 100 de la mortalité infantile. Le tiers de la mortalité par malformations se situe dans la période néo-natale, près de la moitié au cours du premier mois et approximativement 80 p. 100 dans la première année.

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