広範囲頭蓋骨欠損を伴った先天性皮膚欠損症の１例

抄録

　　Aplasia cutis congenita of the scalp is a relatively rare disease. Bleeding from the superior sagittal sinus and intracranial infection indicate fatal complications. We report a case of aplasia cutis congenita of the scalp with a large cranial bone defect. A girl weighing 1484 g was born by cesarean section at 35 weeks of gestation due to severe fatal growth retardation. Aplasia cutis congenita consisted of a large scalp defect measuring 8.0 × 6.0 cm at the parietal region and 2.0 × 2.0 cm at the occipital region involving the full thickness of the cranium. Conservative treatment was performed and the skin defect was covered with white petrolatum and film to protect from drying after spraying basic fibroblast growth factor. The wound was fully epithelialized within 12 weeks. The scalp defects were treated by conservative methods without major complications. We describe the course of treatment in detail.