Intracranial hypertension after cranial vault decompression for craniosynostosis

A 3-year-old boy with chronic papilledema, bilateral coronal synostosis and Pfeiffer syndrome underwent an orbitofrontal advancement. One month postoperatively, his papilledema had resolved, however, his vision progressively deteriorated over the ensuing 3 months to legal blindness. He had no symptoms or signs of increased pressure. Multiple imaging studies were unremarkable except for dilatation of the optic nerve sheaths. A lumbar puncture revealed intracranial hypertension, which resolved after treatment with a lumboperitoneal shunt. Intracranial hypertension may persist after craniofacial reconstruction. In patients who have progressive neurological deterioration after craniofacial reconstruction, direct measurement of intracranial pressure is indicated, despite a lack of clinical signs or symptoms of intracranial hypertension and normal imaging studies.