Aknin J, Seguin P, Brunon J, Ouchchane M
Service de Stomatologie, Chirurgie Maxillo-Faciale et Chirurgie Plastique de la Face, Hôpital de Bellevue, Saint-Etienne.
Rev Stomatol Chir Maxillofac. 1992;93(4):267-72.
The authors report about a familial case of skin aplasia of the vertex cranii, of which they present a form that required emergent surgical repair with a favorable outcome. The investigation allowed tracing two relatives with minor forms expressed as alopecia of the vertex. Skin aplasia of the vertex cranii is an exceptional condition, which consists in the congenital absence of cutaneous, bony and/or dural tissue in the cranium. The various etiopathogenetic, clinical, diagnostic, evolutive and therapeutic aspects are reviewed.
作者报告了一例家族性头顶颅骨皮肤发育不全病例，其中呈现出一种需要紧急手术修复且预后良好的形式。该调查发现了两名表现为头顶脱发的轻微形式的亲属。头顶颅骨皮肤发育不全是一种罕见病症，其特征是颅骨先天性缺乏皮肤、骨骼和/或硬脑膜组织。本文对其各种病因、临床、诊断、演变及治疗方面进行了综述。
