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What is CUTIS APLASIA?

Cutis Aplasia is a rare disorder characterized by the absence of a portion of the scalp skin, typically in the midline at the top of the head. In severe cases, the underlying skull bone may be missing, leaving the outer covering of the brain (dura) exposed. The condition can be limited to a small area, have multiple small lesions, or be more extensive involving a large portion of the scalp.

Symptoms SYMPTOMS

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 & MANAGEMENT

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.

After the affected area of scalp heals, it is typically scarred and will not grow hair. Occasionally a skull defect remains. Secondary or later reconstruction may be necessary. Bone grafts taken from adjacent skull may be necessary to reconstruct a skull defect. Scarred scalp skin can be either directly excised, or often for larger areas of scarring, a procedure known as tissue expansion is warranted. In this situation, balloons placed below the adjacent scalp surface are slowly expanded to generate hair-bearing skin to resurface the scarred area.

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.

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.