Congenital Cranial Dysinnervation Disorder Complicated by Ipsilateral Orbital Choriostoma

Abstract

A 20-year-old woman with right-sided congenital cranial dysinnervation disorder (exotropia, ophthalmoplegia, and ptosis) presented for strabismus surgery. Exploration of the right eye confirmed abnormal extraocular muscles. Lateral rectus recession with medial rectus plication and inferior oblique recession improved primary position ocular alignment. However, an unusual pattern of inferior chemosis developed postoperatively and persisted during the next month. Excision and histologic examination of this tissue revealed areas of lacrimal gland and cartilage with surrounding inflammation, suggestive of a disrupted orbital choriostoma. [ J Pediatr Ophthalmol Strabismus . 2022;59(6):e66–e68.]