Spontaneous calvarial regeneration after craniectomy for sickle cell-related skull infarction and spontaneous epidural hematoma: implications for delayed cranioplasty.

backgroundSpontaneous epidural hematoma is a rare but recognized neurological complication of sickle cell disease and is often associated with infarction of the calvarial bone. Surgical evacuation may necessitate craniectomy when the involved bone is infarcted and nonviable. Spontaneous regeneration of calvarial defects after craniectomy is uncommon but has been reported in children. CASE PRESENTATION: We report a 10-year-old boy with homozygous sickle cell disease (HbSS), diagnosed at the age of three years, who presented with headache and progressive right hemiparesis two weeks after a vaso-occlusive crisis. Cranial computed tomographic scan demonstrated a left parietal epidural hematoma. Emergency surgery was performed. Intraoperatively, the overlying calvarial bone was infarcted and nonviable, necessitating craniectomy and evacuation of the hematoma. The postoperative course was uneventful, and the patient was scheduled for delayed cranioplasty. However, two years later, preoperative cranial computed tomographic scan revealed substantial spontaneous ossification across the previous craniectomy defect, resulting in near-complete restoration of the calvarium thus obviating the need for cranioplasty.

conclusionThis case illustrates the remarkable osteogenic capacity of the pediatric calvarium and highlights the importance of repeat imaging prior to delayed cranioplasty in children, as spontaneous calvarial regeneration may eliminate the need for reconstructive surgery.