Delayed Onset Craniosynostosis Presenting as Idiopathic Intracranial Hypertension

Craniosynostosis refers to premature fusion of one or more cranial sutures, and typically presents with abnormal head shape within the first year of life. Management includes cranial vault reconstruction (CVR) or observation. Rarely, suture fusion may occur later in childhood or go unnoticed. These delayed cases may not be associated with changes in head shape and come to attention only because of an elevation in intracranial pressure. We present five children referred for presumed pediatric idiopathic intracranial hypertension (IIH) who fulfilled the modified Dandy criteria for the disease but were ultimately found to have sagittal craniosynostosis as the cause of papilledema. We review presenting symptoms and signs, associated radiological findings, management and clinical course.