EPOS

European Paediatric Ophthalmological Society

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VISUAL OUTCOMES AND ABLYOGENIC RISK FACTORS IN THE SYNDROMIC CRANIOSYNOSTOSES - A REVIEW OF 141 CASES

KHAN SAJID¹, NISCHAL KEN¹, DEAN FIONA¹, HAYWARD RICHARD¹, WALKER JANE¹
¹GREAT ORMOND STREET HOSPITAL, LONDON

Purpose: To determine the visual outcome and prevalence of amblyogenic risk factors in children with craniosynostotic syndromes. Methods: We reviewed the case notes of 141 patients seen within the Craniofacial Unit and retrieved information on date of birth, age at first and last examination, cycloplegic refraction at last visit, best corrected visual acuity at last visit, horizontal ocular deviation in primary position at first visit, and alphabet pattern if any. The presence of astigmatism, its magnitude and orientation of axis was determined. Results: 40.3% of patients had 1 Dioptre (D) of astigmatism or greater and of these 64% had oblique astigmatism in at least one eye. Anisometropia of 1D or more was found in 18% of patients ( c.f. age matched normals 3.5%). Horizontal strabismus was found in 70% ( 38% exotropia ,32% esotropia ).Visual outcome results showed 39.8% of patients(45 of 113) had visual acuity of 6/12 or worse in their BETTER eye. Conclusion: In the largest study, to date, we have shown a poor visual outcome in children with Crouzon's, Pfeiffer's, Apert's and Saethre-Chotzen syndromes (39.8% with 6/12 or worse in the better eye) together with significant prevalence of amblyogenic risk factors.