Complete osseous regeneration of a large skull defect in a patient with cutis aplasia: a conservative approach

Abstract

Cutis aplasia (or aplasia cutis congenita) is a congenital absence of all skin layers, often extending through bone. This defect usually occurs in the scalp and can be extensive, exposing the dura mater, and deeper meninges. Treatment regimens for cutis aplasia have included early operative intervention, including skin and bone grafts, local scalp flaps, or free flaps to close the defect. In addition to the significant perioperative risks, these invasive procedures may inhibit the osteogenic potential of the dura to initiate and sustain bony closure of the defect. We report a case of an infant with Adams-Oliver syndrome and cutis aplasia involving a large portion of the skull that was treated conservatively with topical Silvadene dressings. No surgical treatment of bone or soft tissue reconstruction was necessary. This case report is the first to our knowledge to document complete bony restoration of the cranial vault through serial three-dimensional CT scans. The intensive therapeutic intervention in this case report allowed early discharge from the hospital, a gradual amelioration of the patient's alopecia as the hair-bearing scalp slowly covered the defect, and precluded the need for any subsequent bony reconstruction of the cranial vault. We hypothesize that conservative treatment of cutis aplasia maintains dural induction of osseous regeneration, and any treatment plan for bony defects of cutis aplasia should consider maintenance of dural integrity. Although further investigation is warranted, an initial trial of antimicrobial dressing care might optimally promote secondary closure of the cranial vault without the need for surgical intervention.