Parental allografts in the management of Adams–Oliver syndrome

Abstract

Dear Editor: We read with interest the report of Udayakumaran et al. [5]. The case they present is strikingly similar to that of a girl with Adams–Oliver syndrome (AOS) who has been under our care since 2005. Our patient was born with a 10×12 cm full-thickness scalp defect with present but abnormal underlying dura. We elected to cover the defect with a scalp transposition flap but, as in Udayakumaran’s case, the flap failed. Likewise, we covered the defect with a paternal split thickness skin graft which was repeated on three occasions over the following 2 months until the wound had developed a sufficiently robust vascularised connective tissue bed to receive a meshed split thickness autograft. The graft matured well and over the following 7 years, new bone continues to form beneath it, closing the calvarial defect. Both cases illustrate key points in the management of this very rare condition which we wish to emphasise. Although scalp flaps appear to be an attractive option for achieving rapid closure of aplasia cutis congenita and AOS scalp defects, they are prone to a high rate of failure probably due to abnormal vascular anatomy [4]. Although free transfer techniques would avoid this problem, successful microsurgery in the neonate remains a considerable challenge. Conservative approaches employing repeat dressings are well described [3], but are slow to achieve epithelialisation in large defects. Repeated dressing changes risk infection and haemorrhage [1], which may be fatal, as well requiring extended nursing involvement and likely distress to the infant. Early skin grafting offers a middle course between these two approaches but can be impeded by the lack of available donor skin. Further, the initial wound bed may well be fragile and poorly vascularised, resulting in a poor quality, unstable graft with likely areas of loss, if an autograft is applied immediately. Allografts initially survive and provide temporary wound closure, reducing dressing demands along with the associated risk of bleeding and serious infection. Further, allografts promote development and maturation of the underlying wound bed [2]. Temporising with parental allografts also allows the infant to grow sufficiently to provide an adequate autograft. Overgrafting the final autograft with parental allograft also enables a thin widely meshed autograft to be used, minimising donor site morbidity. In retrospect, we would suggest that in patients with a large scalp defect, parental allografts should be considered as a first-line measure rather than as a salvage procedure pending definitive autografting. Lastly, we note the authors’ plan to evaluate the need for cranioplasty at a future date. Our and others’ [6] experience of new bone formation beneath skin grafts in calvarial defects in young children with intact dura suggests that a cranioplasty may well not be required.