Visual preservation in congenital orbital fibrosis

Congenital orbital fibrosis (COF) is a nonprogressive, unilateral, congenital process in which variable fibrosis is demonstrated in the orbit, resulting in restrictive strabismus, upper eyelid malposition, and axial displacement of the globe. We present 4 new pediatric cases of COF and discuss factors that impact visual development. We also describe a patient with local compressive optic neuropathy/edema who underwent optic nerve sheath fenestration (ONSF) for visual preservation.

Literature review and retrospective case series.

Four male COF patients (mean age of 11 months) were examined. Two patients presented with decreased ocular motility of the affected eye. Two patients presented with exophthalmos, and one presented with enophthalmos. Two patients presented with ptosis, and one presented with eyelid retraction. Two patients presented with optic nerve atrophy, and one presented with optic nerve edema. Magnetic resonance imaging demonstrated involvement of the superior, medial, and inferior rectus and superior oblique muscles in 3 patients and the lateral rectus and inferior oblique muscles in 2 patients. Three patients underwent orbitotomy. Histology was consistent with fibrosis. Three patients demonstrated amblyopia, and 2 responded to treatment. The patient with optic nerve edema underwent ONSF. At 4 months' follow-up, the edema had resolved.

COF can present with either anterior or posterior globe displacement. Patients must undergo a complete ophthalmic evaluation to identify modifiable factors. Strabismus and ptosis should be addressed for optimal visual development. Amblyopia therapy should be instituted quickly. Patients who present with active optic nerve edema may benefit from ONSF for local compressive optic neuropathy.