Anterior basal encephalocele in the median cleft face syndrome. Comments on nosology and treatment.
Two cases of median cleft syndrome with associated basal encephalocele are presented. The median cleft face syndrome consists of several craniofacial defects and occurs, as far as we know, sporadically. Extracranial defects should be looked for carefully since they may indicate the presence of a specific syndrome with its own prognosis and mode of inheritance. Basal encephaloceles are sometimes part of the syndrome and may cause serious, or even life-threatening, symptoms. With regard to the treatment of basal encephaloceles there still exists a lot of controversion, but most authors agree that it is better to postpone operation until after the age of three because mortality is lower then.
Grubben
C
C
Centre for Human Genetics, University Hospital Leuven, Belgium.
Fryns
J P
JP
De Zegher
F
F
Van Den Berghe
H
H
D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D002675:Child, Preschool; D002971:Cleft Lip / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D002972:Cleft Palate / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D004677:Encephalocele / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D005147:Facial Bones / Q000002:abnormalities*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D012886:Skull / Q000002:abnormalities*; D013577:Syndrome