CONGENITAL CUTIS APLASIA OF THE SCALP - A REVIEW OF 17 CASES AND PROPOSED SURGICAL APPROACHES

Aplasia cutis congenita of the scalp is a cons genital anomaly, charac terised by a lack of covering tissue on the vertex of the skull. Seven teen patients were treated in Lille CHR from 1964 to 1992. Three patie nts were of the same family (the father and his two sons). The frequen cy has probably been underestimated, due to the high under-diagnosis o f minor forms (localized alopecia), The defect was purely cutaneous in 10 patients, scalp and bone in 7 patients, among whom 6 had exposure of the sagittal sinus. The size varied from 49 mm(2) to 192 cm(2). The main area of involvement was medial with parietal extension. Seven ch ildren had associated malformations: trisomy 13 with cardiopathy (2), cleft lip and palate (3), limb malformation (2), epilepsy (1), and abn ormal ears (2). Three patients had been treated at the time of cicatri cial alopecia by excision and suture or tissue expansion. Thirteen pat ients were treated in the neonatal period. The various methods used we re spontaneous healing (I), skin graft (1), excision and suture (4), l ocal T or W plasties (2), scalp rotation flap (5), and 1 patient died before any treatment. Four patients died in neonatal period: 2 trisomi es 13, 1 meningitis with septicaemia, and 1 sagittal sinus rupture. On e child required a secondary procedure at age 7; this was a cranioplas ty and latissimus dorsi muscle free flap. The prognosis is highly cond itioned by the associated pathology, e.g. trisomy 13, the size of the bone defect with, in addition, sagittal sinus exposure, and the rapidi ty of the treatment.