Aplasia cutis congenita of the scalp: How much therapy is necessary in large defects?

Abstract

Here we have shown that conservative treatment might be an option, even if the wound diameter is greater than 1 cm(2), to prevent infants from any untimely operative procedure with an elevated operative risk if any additional risk factors are excluded.

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

“…The sporadic cases may characterize incomplete penetrance of the autosomal-dominant mode of transmission. 4,14 Our patient had no known family history of congenital deformities of the scalp and extremities, and results of her chromosomal study were normal.…”

“…13,17 Determination of appropriate clinical management during the early stages of life improves survival 8 and is based on the size of the defect, presence of an underlying skull defect, the child's general condition, and his/her associated life expectancy. 4,18 Conservative treatment is advocated by some authors because of its simplicity. 4,16 As initial management of the condition in newborns, dressing changes avoid the possibility of surgical complications and the potential failure of skin flap or heterologous graft coverage.…”

“…4,18 Conservative treatment is advocated by some authors because of its simplicity. 4,16 As initial management of the condition in newborns, dressing changes avoid the possibility of surgical complications and the potential failure of skin flap or heterologous graft coverage. 2 Conservative treatment of cutis aplasia also maintains dural induction of osseous regeneration.…”

“…16 Typical dressing regimens in conservative treatment include daily wound care with moist sterile gauze and topical application of the antimicrobial agents silver sulfadiazine or bacitracin ointment, in conjunction with a systemic antibiotic therapy for the first several weeks, treatments that reduce the bacterial load and avoid dehydration of the wound. 4,7,18 Use of nonadherent, atraumatic, and relatively cheap dressings such as Mepitel has reportedly been successful for the initial conservative treatment to manage large defects in AOS. 12 Conservative therapy complications include meningitis, significant hyponatremia with seizures, and brain herniation.…”

Two different management modalities in a two sibling case report of Adams Oliver syndrome

“…An optimal conservative management plan has to provide protection against infection, keep the wound sufficiently moist and supply the optimum wound environment 17. An example of this would be Omiderm (Taureon, The Netherlands),13 which is made of a permeable polyurethane film which minimises fluid loss, but is permeable to oxygen and antibiotic solutions and prevents microbial invasion.…”