What is complex cutis aplasia

Complex cutis aplasia is a rare disorder characterized by the absence of a portion of the skin on the scalp. In most cases, the disorder occurs as a solitary defect, but it can also appear as multiple lesions.

Diagnosis

Complex cutis aplasia is diagnosed at birth when babies are born with an absence of skin and possibly underlying soft tissue and skull bone which may leave the dura (the outer covering of the brain) exposed. The absence of skin and underlying tissues may vary from small defects less than one inch to massive defects that involve much of the scalp.

Treatment

Treatment of cutis aplasia depends on the degree of involvement. For small areas with minimal exposure to underlying tissues, dressings alone may be all that is required until the area heals on its own.

For larger areas in which the underlying brain tissues and veins are at risk of exposure, more aggressive treatment is necessary. In some cases, bone grafts taken from elsewhere in the skull will be used in addition to large rotation flaps of adjacent scalp. Biological dressings or skin grafts may also be used.

The goal of treatment is to protect the cerebral and venous structures in order to prevent infection, cerebral spina fluid (CSF) leaks, or significant bleeding.

In more complicated cases, your child may electively undergo secondary reconstruction at a later stage once the wound is stabilized. In these reconstruction procedures, any missing bone may be transplanted from an adjacent side on the cranium.

Subsequent procedures after your child is 18 months old may include tissue expansion of the adjacent scalp to address soft tissue deficiencies, removal of the skin grafts, and repair with expanded hair-bearing scalp.

Why choose us

Cutis aplasias are a rare congenital anomaly. In a newborn patient with a large wound, exposure of the brain and venous drainage pose a surgical emergency that must be dealt with in an intensive care setting with input from plastic surgeons, neurosurgeons, and other pediatric specialists. Your child should be treated at a pediatric institution that offers a high level of expert care in all of these specialties. At CHOP, the multidisciplinary Craniofacial Program will coordinate your child’s care.

Reviewed by: Scott Bartlett, MD and Jesse Taylor, MD
Date: March 2014