Visual outcomes and amblyogenic risk factors in craniosynostotic syndromes: a review of 141 cases.
OBJECTIVE
To determine the visual outcome and prevalence of amblyogenic risk factors in children with craniosynostotic syndromes.


METHODS
The case notes of 141 children seen within the craniofacial unit were reviewed and information retrieved 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 were 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 (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.


CONCLUSIONS
In the largest study to date a poor visual outcome was shown 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.
Khan
S H
SH
Department of Paediatric Ophthalmology, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK.
Nischal
K K
KK
Dean
F
F
Hayward
R D
RD
Walker
J
J
D000293:Adolescent; D000550:Amblyopia / Q000209:etiology; D001251:Astigmatism / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003398:Craniosynostoses / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D013285:Strabismus / Q000209:etiology; D013577:Syndrome; D014786:Vision Disorders / Q000209:etiology*; D014792:Visual Acuity