A Case Report of Postoperative Cystic Hydrocephalus Following Duroplasty and Scalp Reconstruction in Neonatal With Aplasia Cutis Congenita and Extensive Craniodural Defect
TL;DR
A full‐term female newborn with a large midline parietal scalp defect associated with absence of the underlying calvarium and dural aplasia is reported, representing a severe and potentially life‐threatening variant of the disease.
Abstract
Background Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by localized absence of skin, most frequently involving the scalp. In severe cases, the defect may extend to the skull and dura mater, exposing intracranial structures and increasing the risk of infection, hemorrhage, and venous sinus injury. Although early surgical reconstruction with duraplasty and scalp flap coverage is often successful, delayed complications such as cystic hydrocephalus may develop months after surgery. Extensive ACC with both cranial and dural defects remains exceedingly rare, and optimal management strategies are not well established. Case Presentation We report a full‐term female newborn with a large midline parietal scalp defect measuring 7 cm × 6 cm, associated with absence of the underlying calvarium and dural aplasia. The lesion was covered by a thin membranous tissue with visible brain pulsation and exposure of the superior sagittal sinus (SSS). Initial management included sterile dressings and broad‐spectrum antibiotics. On day 8 of life, surgical reconstruction was performed using synthetic duraplasty followed by rotational scalp flap closure. Postoperative recovery was uneventful, and the wound healed satisfactorily. At 6 months postoperatively, the patient developed cystic hydrocephalus, which was managed with ventriculoperitoneal shunt placement. At 24‐month follow‐up, the patient achieved normal developmental milestones with a good cosmetic outcome. Conclusion Extensive ACC involving the skull and dura mater represents a severe and potentially life‐threatening variant of the disease. Early multidisciplinary management with synthetic duraplasty and scalp flap reconstruction can provide effective protection of intracranial structures and yield favorable long‐term outcomes. Nevertheless, long‐term follow‐up is essential, as delayed complications such as cystic hydrocephalus may arise months after initial surgical repair.