Management of craniosynostosis at an advanced age: Clinical findings and interdisciplinary treatment in a 17 year-old with pan-suture synostosis

Abstract

Craniosynostosis is the premature fusion of cranial sutures, occurring at a rate of approximately 1 in 2000 live births; it is usually diagnosed and treated within the first year-of-life. Some diagnoses are delayed and only detected later in childhood or adolescence when symptoms of increased intracranial pressure (ICP) arise such as headaches and vision changes. We present a case of occult craniosynostosis in which a relatively normocephalic 17-year-old male presented with debilitating headaches, optic nerve edema, and developmental delay consistent with probable ICP elevation. CT scan demonstrated pan-suture craniosynostosis. Invasive monitoring confirmed increased ICP for which he underwent cranial remodeling and expansion. While the functional benefits of cranial remodeling are still vigorously debated, this patient’s headaches resolved postoperatively. Clinicians should be cognizant of cases of occult craniosynostosis, obtain the appropriate preoperative evaluations, and recognize the utility of cranial remodeling in appropriately selected patients.

Keywords

• Craniosynostosis
• Cranial vault remodeling
• Elevated intracranial pressure