Unusual neurosurgical emergency in Paget’s disease of the skull: a case report and review of the literature

Abstract

Introduction: Paget's disease of bone is usually asymptomatic in most cases. The neurosurgeon should be familiar with the common presentation and complications. The rare presentation of this case of Paget's disease of the skull raised our attention to report it. Case description: A Paget's disease patient presented in the emergency department with a disturbed conscious level (GCS 8) without any history of trauma or seizures. CT showed severe hypertrophied left frontal, temporal, and parietal bones with midline shift. The patient underwent an emergency left decompressive craniectomy. Postoperative CT scan was done and showed frontoparietal epidural hematoma on the opposite side which was evacuated immediately. The patient started to improve and became fully conscious in few days with marked improvement of the motor power of her right side. Discussion and evaluation: Severe skull involvement and impending brain herniation may occur in Paget's disease of the skull which needs urgent decompressive surgery. Contralateral epidural hematoma after decompressive surgery may rarely occur and should be suspected in case of intraoperative brain swelling and postoperative failure of improvement. Neurosurgeons and radiologists should recognize the rare phenomenon of periosteal bone formation in the pagetic bone and its extension into surrounding tissues. Conclusions: In our case, we faced an unusual life-threatening condition in a patient of Paget's disease with severe skull involvement leading to rapid deterioration of consciousness and impending brain herniation. The neurosurgeon should be aware of this rare emergency condition.