ISSN:
1435-0130
Keywords:
Cutis aplasia
;
Cutaneous agenesis
;
Skull agenesis
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract Aplasia cutis congenita of the scalp is a congenital anomaly, characterised by a lack of covering tissue on the vertex of the skull. Seventeen patients were treated in Lille CHR from 1964 to 1992. Three patients were of the same family (the father and his two sons). The frequency has probably been underestimated, due to the high under-diagnosis of 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 mm2 to 192 cm2. The main area of involvement was medial with parietal extension. Seven children had associated malformations: trisomy 13 with cardiopathy (2), cleft lip and palate (3), limb malformation (2), epilepsy (1), and abnormal ears (2). Three patients had been treated at the time of cicatricial alopecia by excision and suture or tissue expansion. Thirteen patients were treated in the neonatal period. The various methods used were spontaneous healing (1), skin graft (1), excision and suture (4), local T or W plasties (2), scalp rotation flap (5), and 1 patient died before any treatment. Four patients died in neonatal period: 2 trisomies 13, 1 meningitis with septicaemia, and 1 sagittal sinus rupture. One child required a secondary procedure at age 7; this was a cranioplasty and latissimus dorsi muscle free flap. The prognosis is highly conditioned by the associated pathology, e.g. trisomy 13, the size of the bone defect with, in addition, sagittal sinus exposure, and the rapidity of the treatment.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00209783
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