Surgical management of the cutaneous manifestations of linear nevus sebaceus syndrome.

Abstract

Linear nevus sebaceus syndrome is characterized by the association of nevus sebaceus covering extensive areas on the head and scalp with abnormalities of the central nervous system, ophthalmologic and skeletal changes, and malignancies. The incidence is approximately one per 10,000 live births, and there is no sexual predilection reported. The original description of this syndrome was followed by extensive literature describing the dermatologic, neurologic, and ophthalmologic manifestations of this disease. The objective of this report is to describe the surgical approach for the excision and reconstruction of giant sebaceous nevi of the face and scalp in children with linear nevus sebaceus syndrome on the basis of a consecutive series of five patients treated over 10 years in the same institution. To the authors' knowledge, this report represents the largest surgical series and suggests a reliable approach to the treatment of the cutaneous manifestations of this syndrome.