Surgical Management Of Recurrent Complex Epibulbar Choristomas In Organoid Nevus Syndrome

To report a case of recurrent complex epibulbar choristomas in a baby with Organoid Naevus Syndrome, with emphasis on diagnosis and surgical mangement  and to review the literature on this topic 

Interventional case report of a patient managed at a tertiary hospital with ophthalmology, radiology, paediatric dermatology, paediatric neurology input. Surgeries were done under general anesthesia at the hospital's day surgery centre. 

A 2 month old girl presented with right eye lumps rapidly growing towards the pupil. She was full-term with no parental consanguinity. Examination revealed an inferotemporal fleshy limbal dermoid (6mm diameter) and two adjoining superior lesions (10mm) encroaching 1 mm from the pupil margin. There was no coloboma or posteror segment anomalies. She also had yellowish scalp plaques with hyperpigmented eyelid and cheek lesions. She underwent lamellar keratoscleroplasty with excision of lesions down to clear stromal bed. A 6 mm and a 10 mm cornea graft were secured inferiorly and superiorly with 10 o nylon. Histopathology confirmed complex choristoma ( adipose tissue, fibrous tissue and ectopic lacrimal gland tissue) of the eye, organoid nevus ( naevus sebaceous) of the scalp and compound melanocytic naevus of the skin. Neuroimaging showed right temporal and parieto-occipital leptomeingeal angiomatosis, leading to diagnosis of organoid nevus syndrome. She was prescribed glasses for hyperopia/astigmatism and left eye patching, Despite initial recovery, the choristomas recurred 6 months postop, progressively enlarging to 6 mm superiorly and 7 mm inferiorly by 17 months. The lesions were almost encroaching the pupillary margins.Vision remained poor (6/60) with esotropia. She underwent repeat repeat excison and lamellar keratoscleroplasty with 6mm and 7 mm cornea grafts superiorly and inferiorly. Histopathology confirned choristoma with chronic inflammatory cells but no malignancy. 

Organoid nevus syndrome is a congenital, non hereditary phacomatosis with cutaneous, neurologic and ocular manifestations. Classified as choristomas, the different types of dermoids include well defined limbal dernoids, more diffuse dermolipomas or complex choristomas containing two or more ectopic tissue. Complex choristomas are typically stationary and non progressive but our case had rapid growth in infancy and recurrence in early childhood. Surgical excision is challenging due to deep corneal involvement, requiring careful dissection to avoid perforation. Postoperatove risks include inflammation, interface neovascularisation and graft opacification. Timely mangement of refractive errors and amblyopia is essential for visual outcomes.