Management of large scalp and skull defects in a severe case of Adams-Oliver syndrome

Abstract

<jats:p>Adams-Oliver syndrome is a rare congenital disorder that includes congenital scalp and skull defects, variable degrees of terminal transverse limb anomalies, and cardiac malformations. Cutis aplasia occurring in 75% of patients is a potentially life-threatening condition. Large skin defects that cannot be closed primarily present a management dilemma, and may require skin grafting or flaps, or a combination of both operative and conservative modalities. The authors' experience in management of huge scalp and bone defects with the Integra Dermal Regeneration Template and regular dressing changes showed good scalp repair and no serious complications attributed to this approach.</jats:p>

Adams–Oliver syndrome

“…Cutis marmorata telangiectatica congenita occurs in about 25% of patients. It may be localized or generalized 1–3 . Other anomalies may include cranial arteriovenous malformation, cardiovascular diseases, and gastrointestinal and genitourinary malformations.…”

“…Most cases require conservative treatment, including wound dressings and serum physiologic and antibiotic ointments. Cases that involve large skin and skull defects require surgical management 3,11 …”

“…Additionally, if a newborn infant presents with aplasia cutis congenita and limb defects, dermatologists should consider Adams–Oliver syndrome and should evaluate the CNS and look for potential cardiovascular, gastrointestinal, and genitourinary malformations. Early management of these patients may reduce the risk for developmental delay and mortality caused by vascular pathologies and sepsis 2,3 …”

Different Clinical Phenotypes in Adams-Oliver Syndrome Conservative Approach to Aplasia Cutis: A Report of Two Cases

“…The management consists in a difficult multi-disciplinary approach aimed primarily at the treatment of the aplasia cutis. Large skin defects that cannot be closed primarily present a management dilemma, and may require skin grafting or flaps, or a combination of both operative and conservative modalities [3].…”

Aplasia Cutis Congenita of the Scalp, Composite Type: The Criticality and Inseparability of Neurosurgical and Plastic Surgical Management

“…Substitutes for split-thickness skin grafts have been used in dealing with the scalp defects but are most useful for secondary deformities resulting from scalp rotations, including: Biobrane®, cultured epithelial autographs, Dermal Regeneration Template [53,54], composite graft of cultured autologous fibroblasts and keratinocytes [55], acellular dermal graft with cultured epithelial autografts [56,57], recombinant human fibroblast graft (Trafermin®) [28], petrolatum dressings [28], and silicone elastomer membrane [39]. In both our patients, the donor sites resulting from the rotation of the scalp flaps and the original abdominal skin defects were managed temporarily with Biobrane® and later definitively covered with cultured epithelial cell autografts harvested from the groin.…”