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Refractory Noninfectious Anterior Uveitis Associated With Systemic Mastocytosis
Journal of Pediatric Ophthalmology and Strabismus
|October 2, 2009
Summary
Systemic mastocytosis can cause severe eye inflammation (uveitis) and high intraocular pressure. This case highlights a rare association requiring medical and surgical glaucoma management.
Area of Science:
- Ophthalmology
- Hematology
- Immunology
Background:
- Systemic mastocytosis (SM) is a rare disorder characterized by mast cell accumulation.
- Ocular manifestations of SM are uncommon, with anterior uveitis and glaucoma being rare complications.
Purpose of the Study:
- To report a case of bilateral nongranulomatous anterior uveitis and high intraocular pressure in a pediatric patient with systemic mastocytosis.
- To discuss the diagnostic challenges and management strategies for this rare ocular presentation of SM.
Main Methods:
- A 10-year-old boy with biopsy-proven systemic mastocytosis presented with ocular symptoms.
- Anterior chamber paracentesis was performed for cytological evaluation.
- Ocular management included topical steroids, oral methotrexate, topical antiglaucoma medications, and deep sclerectomy.
Main Results:
- The patient presented with bilateral nongranulomatous anterior uveitis and elevated intraocular pressure.
- Anterior chamber fluid analysis showed monocytes and macrophages, but no mast cells.
- Uveitis was partially controlled with medical therapy; glaucoma required surgical intervention in one eye.
Conclusions:
- This case represents the first reported association of systemic mastocytosis with bilateral nongranulomatous anterior uveitis and secondary glaucoma in a pediatric patient.
- Early diagnosis and multidisciplinary management are crucial for patients with SM and ocular complications.
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