Acutis Dermal Dysplasia: a review

Abstract

Aplasia Dermal Dysplasia (ADD) is an etiologically heterogeneous congenital abnormality having a circumscribed area of absent skin that involves any region of the body mainly the cranial vertex. The condition may involve only the superficial skin but also the subcutaneous tissue, bone, or even the dura. ADD can be isolated or associated with other malformations in the context of a genetic disease. Numerous alterations of skin detectable at birth might be confused with aplasia cutis congenita. Birth trauma, injuries from obstetrical procedures and intrauterine trauma such as from mechanical deliveries are some examples. Histologic analysis can make the proper diagnosis. Here we describe the surgical strategies to treatment of ADD, including patient selection, properative preparation, the operative procedure and Post-operative management. A multidisciplinary team including neurosurgery and plastic surgery, among others, is optimal for the treatment of these patients. Nevertheless, intensive care must be undertaken during treatment to detect potential life-threatening complications.

Keywords

• acutis dermal dysplasia
• skin
• adams-oliver syndrome
• cranioplasty
• plastic surgery