This study presents the orbital and cranial magnetic resonance imaging (MRI) findings of patients diagnosed with congenital fibrosis of the extraocular muscles (CFEOM) along with the surgical procedures and outcomes for ocular motility and strabismus. Nine patients are described: three with type 1, one with type 2 and five with type 3. The condition was bilateral in four and unilateral in five cases. Infraducted primary deviation was reported in eight cases and supraducted in three. Seven cases had abnormal head posture. Eight had severe limitation of horizontal ocular movements and all had limited vertical ocular movements. Exotropia was present in four cases and esotropia in three. All unilateral cases were hypotropic. Six had aberrant movements, binocular vision was absent in eight, amblyopia was present in seven and eight had moderate / severe refractive errors. MRI revealed atrophy of at least one extraocular muscle supplied by the IIIn in all cases. Five had hypoplasia of the IIIn and one had a reduced VIn. Strabismus surgery was undertaken for four cases and two required more than one procedure. Favourable outcome was obtained in two cases. The authors conclude orbital and cranial MRI provides useful information on extraocular muscles and nerves, and surgery should be chosen based on individual findings after treatment of amblyopia and refractive error.
CFEOM outcomes
Reviewed by Fiona Rowe
Congenital fibrosis of the extra ocular muscles: magnetic resonance imaging findings and surgical treatment.
CONTRIBUTOR
Fiona Rowe (Prof)
Institute of Population Health, University of Liverpool, UK.
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