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Maria Carolina Ortube, Federico Velez, Joseph Demer; Orbital imaging in craniosynostosis: Understanding the pathophysiology of complex strabismus. Invest. Ophthalmol. Vis. Sci. 2013;54(15):1936.
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© ARVO (1962-2015); The Authors (2016-present)
To evaluate the strabismus patterns, orbital imaging, and surgical results in a large cohort of pediatric patients with complex craniofacial disorders.
A retrospective review was performed of 52 children with confirmed craniofacial disorders referred to Jules Stein Eye Institute between 1995 and 2009.
The cohort included 14 patients with syndromic craniosynostosis (Apert, Crouzon, Saethre Chotzen, Antley-Bixler, Pfeiffer, Jacobsen, Frontonasal dysplasia), 9 with non-syndromic synostosis and 8 with hemifacial microsomia. Other diagnosis included plagiocephaly, Treacher Collins, Mobius, Dandy-Walker, Di George and Nager syndromes. Strabismus was detected in 37/52 children (71%, 22 female/ 15 male). Mean (±SD) age at initial examination was 6±5 years. A total of 18/37 strabismic children (49%) presented with craniosynostosis, most commonly involving the bicoronal and sagittal sutures. There was developmental delay in 51 % of cases, and 14% required VP shunt placement for hydrocephalus. Exotropia was detected in 18 cases (49%), esotropia in 11 cases (30%), pulley heterotopy in 7 cases (19%), Duane syndrome and hypertropia in 5 cases each (3.5 %), superior oblique palsy in 3 cases (8%), ophthalmoplegia due to Mobius syndrome in 2 cases (5.4%), and double elevator palsy in 1 case. Fourteen patients (38 %) presented with incomitant horizontal strabismus, with V pattern in 9 cases, A pattern in 4 cases, and Y pattern in 1 case. Orbital imaging by CT or MRI was performed in 10/37 strabismic patients (27%). Imaging demonstrated excyclotorsion with abnormal position of the extraocular muscles (10 cases), superior oblique hypoplasia (2 cases), and optic nerve hypoplasia (1 case). Fifteen children (40 %) underwent strabismus surgery with mean follow of 70±60 months. Mean (±SD) initial angle of deviation in primary position was 19 ± 12 Δ in 14/15 cases. In fourteen cases (93%), post-operative alignment in central gaze was satisfactory.
Orbital imaging in patients with craniofacial anomalies demonstrates highly varied and complex anatomical pathophysiology of complex strabismus. The frequent finding of orbital excyclorotation can produce incomitant strabismus in oblique gaze positions that may masquerade as oblique muscles dysfunction, but superior oblique muscle hypoplasia may also occur. Clinical imaging may guide surgical therapy for strabismus.
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