D30. Orbital And Ocular Outcomes Of Rare Craniofacial Clefts: A Single Center’s 30-year Experience with 147 Patients

PURPOSE: Tessier craniofacial clefts are rare malformations that may carry significant functional and aesthetic pathology. This study examines orbital and ocular outcomes of craniofacial clefts. METHODS: We performed a retrospective cohort study of children with rare craniofacial clefts from 1993-2023. Primary outcomes were surgical burden, perioperative complications, ocular findings, and visual acuity. Clefts were grouped into superior periorbital (Tessier #9-11), inferior periorbital (3-6, 8), superior midline (12-14), inferior midline (0-2, 30), and lateral (7). RESULTS: Among 147 children, 75 (51%) had peri-orbital, 40 (27%) medial, and 65 (44%) lateral clefts. Mean age at first surgery was 3.6 ± 5.0 years. 26% underwent eyelid surgery, 25% underwent orbital surgery, and 25% underwent medial or lateral canthopexy. Periorbital cleft was associated with eyelid coloboma (17%) and retraction (12%); superior cleft with ocular surface pathology (88%), nasolacrimal duct obstruction (63%), hypertelorism (38%), lagophthalmos (25%) and ectropion (25%); inferior cleft with eyelid coloboma (18%), eyelid retraction (13%), lagophthalmos (11%), and ectropion (11%); medial cleft with microphthalmos/anophthalmos (25%), hypertelorism (22%); and superior medial cleft with entropion (14%). Among 254 eyes of 127 patients (mean age 9.8 (SD 7.3) years), 72% had "Good", 11% "Fair", and 18% "Poor" acuity. Primary causes of poor acuity included microphthalmia/anophthalmia (46%), optic nerve hypoplasia/atrophy (14%), corneal scarring from exposure/entropion (13%), optic nerve/retinal coloboma (9%), strabismus/refractive amblyopia (9%), post-operative orbital apex syndrome (5%), cataract (2%) and cryptophthalmos (2%). CONCLUSION: Children with rare craniofacial clefts commonly have orbital or ocular disease that may require multidisciplinary treatment, including eyelid, strabismus, or orbital surgery.