A Case of Large Aplasia Cutis Congenita with Underlying Skull Defect: Effective Surgical Treatment with Integra® Dermal Regeneration Template

Abstract

<b><i>Introduction:</i></b> Aplasia cutis congenita (ACC) is a rare congenital abnormality characterized by the absence of a portion of skin at birth which most commonly involves the scalp and can affect the galea, the pericranium, the bone, and the dura mater. It can be an isolated condition or associated with other disorders. <b><i>Case Report:</i></b> We present a case of ACC with a large defect of the scalp and the underlying bone treated with the use of Integra® Dermal regeneration template. At 5 months of follow-up, the wound is completely healed and the bony defect greatly reduced. Contraction of the area of alopecia was observed. <b><i>Discussion:</i></b> Several surgical and conservative options have been described to treat this congenital condition: advanced dressing, skin graft, local flaps, free flaps, and other methods. In our case, we used Integra® Dermal templates which provide a barrier for infections, promote cellular activity for a rapid vascularization, and improve healing.

Challenges in the management of extensive aplasia cutis congenita

“…In terms of the extent of involvement, to the best of the authors’ knowledge, our patient possibly has one of the largest extent in the reported literature, among surviving patients with ACC. Although there are numerous case reports of ACC, only a handful describe ‘extensive ACC’, including seven that involve the scalp,21 26–31 four that affect the trunk,32–35 and three that affect both the head and trunk . Among these, only the latter three36–38 are nearly as extensive as our patient, with only one of them having survived 38.…”

“…There is no consensus in the management of extensive ACC. Different management strategies have been proposed, including conservative31 47–50 and surgical approaches 12 15 20 21 24 51–53…”

“…We decided to proceed with CEA to minimise morbidity in the baby’s parents, reduce risk of rejection and to circumvent ethical considerations. The use of Integra has been described in two ACC patients 20 21. It is a non-cellular artificial matrix comprising an outer silicone layer that simulates the epidermis and an inner three-dimensional porous matrix of cross-linked collagen and glycosaminoglycan of bovine origin which acts as a dermal regeneration template.…”

“…As a bridging measure, staged artificial dermal matrix (ADM)16 20–24 in the form of Integra was applied to her scalp on the third day of life to protect her scalp and provide a scaffold for cellular ingrowth while awaiting CEA, followed by application to the remaining involved areas on her trunks and limbs on day 10 of life under general anaesthesia (figure 2). A small amount of skin was harvested from her right groin for CEA preparation and the donor site was closed primarily at the same setting.…”

Repair of an Occipital Meningocele and Scalp Soft-tissue Reconstruction in a Newborn Patient

“…When complications develop related to the defect, surgery becomes the preferred choice of treatment. 6 However, consideration should be given to preemptive surgical intervention to avoid the risk of major complications.…”