Early Ophthalmology Findings in Nonsyndromic Craniosynostosis

Abstract

Craniosynostosis (CS) occurs 1 in 2500 births and surgical intervention is indicated partly due to risk for elevated intracranial pressure (EICP). Ophthalmological examinations help identify EICP and additional vision concerns. This study describes preoperative and postoperative ophthalmic findings in CS patients (N=314) from chart review. Patients included nonsyndromic CS: multisuture (6.1%), bicoronal (7.3%), sagittal (41.4%), unicoronal (22.6%), metopic (20.4%), and lambdoidal (2.2%). Preoperative ophthalmology visits were at M =8.9±14.1 months for 36% of patients and surgery was at M =8.3±4.2 months. Postoperative ophthalmology visits were at age M =18.7±12.6 months for 42% with follow-up at M =27.1±15.1 months for 29% of patients. A marker for EICP was found for a patient with isolated sagittal CS. Only a third of patients with unicoronal CS had normal eye exams (30.4%) with hyperopia (38.2%) and anisometropia (16.7%) at higher rates than the general population. Most children with sagittal CS had normal exams (74.2%) with higher than expected hyperopia (10.8%) and exotropia (9.7%). The majority of patients with metopic CS had normal eye exams (84.8%). About half of patients with bicoronal CS had normal eye exams (48.5%) and findings included: exotropia (33.3%), hyperopia (27.3%), astigmatism (6%), and anisometropia (3%). Over half of children with nonsyndromic multisuture CS had normal exams (60.7%) with findings of: hyperopia (7.1%), corneal scarring (7.1%), exotropia (3.6%), anisometropia (3.6%), hypertropia (3.6%), esotropia (3.6%), and keratopathy (3.6%). Given the range of findings, early referral to ophthalmology and ongoing monitoring is recommended as part of CS care.