Congenital « healed » cleft lip (CHCL) is an unusual anomaly including a paramedian « scar » of the upper lip, which appears as if a typical cleft lip has been corrected in utero.
The CHCL is frequently associated with an ipsilateral notch in the vermilion, and « collapsed » nostril.
Twenty-five CHCL cases are presented, eighteen of which were an isolated malformation found among the 3,950,715 births examined in two similar birth defect registries : ECEMC in Spain and ECLAMC in Latin America.
Like open cleft lip, of which it seems to be a variant, CHCL is most frequently seen among males (14/18 isolated cases), it preferentially affects the left side (10/18 cases), and it segregated together with cleft lip in one family.
The five CHCL cases with other congenital anomalies included : two cases with hydrocephalus, two VACTERL associations, and one atypical oblique facial cleft infant with single umbilical artery.
CHCL may result from a defective fusion of the frontonasal and maxillary processes (before week 7 of embryonic life), or from a spontaneously repaired open cleft lip, later on.
In either way, these cases heal with a visible scar, and the pre-occurrence of CHCL in two families suggests a familial predisposition to this phenomenon.
Mots-clés Pascal : Fissure congénitale, Lèvre, Palais, Cicatrice, Congénital, Nourrisson, Homme, Epidémiologie, Espagne, Europe, Amérique Latine, Amérique, Stomatologie, Cavité buccale pathologie, Maladie congénitale, Malformation
Mots-clés Pascal anglais : Cleft, Lip, Palate, Scar, Congenital, Infant, Human, Epidemiology, Spain, Europe, Latin America, America, Stomatology, Oral cavity disease, Congenital disease, Malformation
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 95-0503704
Code Inist : 002B10C02. Création : 01/03/1996.