Aplasia cutis cerebri with partial acrania--total reconstruction in a severe case and review of the literature

Abstract

Background: Aplasia cutis cerebri with partial acrania is a rare anomaly that can involve the epidermis, dermis, and subcutaneous tissues of the scalp with significant bone defect.

Methods: The authors present the first successful report of providing one-stage complete cover in a case of aplasia cutis cerebri with major skull defect by using local rotational flaps. Also presented is a review of literature.

Results: The newborn had a receding forehead and a large scalp defect from frontal to occipital bone measuring 10 x 8 cm2 involving full thickness of cranium centrally, thereby exposing the dura. Local rotation flaps were raised from the scalp to cover the 10- x 8-cm2 defect. The edges of the defect were showing granulation tissue with ingrowth of epithelium. Partial-thickness skin grafts were used to cover the raw area left at the nape of the neck after raising the rotation flaps. The child was discharged on the 19th postoperative day.

Conclusions: Aplasia cutis cerebri with partial acrania, as in our case, has a high mortality rate secondary to infection or to hemorrhage from ulceration of the sagittal sinus. The successful outcome of our one-stage local rotational scalp flap technique provided complete cover to this major scalp defect. This technique will definitely improve the management of this complex disorder keeping the morbidity to the minimum.