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Neovascular glaucoma in a child: an unusual presentation of medulloepithelioma
Enoch Kassa1, Helen Li1, Yang Sun1
1Glick Eye Institute, Indianapolis, USA.
Insights
A rare ciliary body tumor, medulloepithelioma, caused neovascular glaucoma in an infant. Early diagnosis and treatment, such as enucleation, are crucial for pediatric eye conditions.
Area of Science:
- Ophthalmology
- Pediatric Oncology
Background:
- Medulloepithelioma is a rare intraocular tumor originating from the primitive medullary epithelium of the ciliary body.
- It can present in early childhood and is a significant cause of secondary glaucoma.
Observation:
- A 12-month-old infant presented with unilateral red eye and elevated intraocular pressure.
- Ophthalmic examination revealed iris neovascularization, optic nerve cupping, and a non-pigmented ciliary body mass.
Findings:
- Biopsy confirmed a diagnosis of medulloepithelioma, characterized by primitive medullary epithelial structures.
- The tumor was associated with significant intraocular pressure elevation and neovascular glaucoma.
Implications:
- This case highlights the importance of considering medulloepithelioma in the differential diagnosis of pediatric intraocular masses and neovascular glaucoma.
- Prompt recognition and management, including enucleation, are essential for managing this condition and preventing vision loss.
Abstract:
A healthy 12 month old infant without significant medical history presented with left eye redness for one week. Ophthalmic examination showed elevated intraocular pressure with iris neovascularization in the affected eye with increased optic nerve cupping. Scleral depression revealed a ciliary body mass in the supratemporal quadrant. A large, non-pigmented, vascular mass was noted; biopsy results showed multilayered cords, tubules, and sheets resembling primitive medullary epithelium arising from the ciliary body. The patient was diagnosed with medulloepithelioma. The patient underwent enucleation of the affected eye. Medulloepithelioma is a rare but important cause of neovascular glaucoma in the pediatric population. This case will focus on the characteristics of medulloepthelioma and the differential diagnosis for a non-pigmented ciliary body mass in a child.
