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[Juvenile xanthogranuloma with intraocular involvement. A case report]
M Hamdani1, A El Kettani, L Rais
1Service d'Ophtalmologie, Hôpital 20 Août 1953, CHU Ibn Rochd, Casablanca, Maroc.
Insights
Juvenile xanthogranuloma (JXG) is a rare childhood disease causing skin and eye lesions. This case highlights iris JXG in an infant, requiring aggressive treatment due to high intraocular pressure.
Area of Science:
- Ophthalmology
- Pediatrics
- Dermatology
Background:
- Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder typically affecting infants and young children.
- JXG commonly presents with cutaneous lesions but can involve extracutaneous sites, including the eye.
Observation:
- A 9-month-old infant presented with iris JXG, megalocornea, and severe ocular hypertension.
- Associated skin lesions were noted on the left eyelid and back.
- The ocular condition was refractory to medical and surgical interventions, necessitating cyclodestruction.
Findings:
- Iris JXG can lead to significant ocular complications, such as megalocornea and intractable glaucoma.
- Ocular hypertension in JXG may require aggressive management, including ablative procedures.
Implications:
- Early diagnosis and management of ocular JXG are crucial to prevent irreversible vision loss.
- Systematic screening for associated systemic conditions like neurofibromatosis and leukemia is essential in JXG cases.
- This case underscores the importance of recognizing rare presentations of JXG in pediatric ophthalmology.
Abstract:
Juvenile xanthogranuloma (JXG) is a rare and usually benign disease occurring in early childhood. It causes skin and deep seated lesions, notably in the eye. We report a case of JXG in the iris of a 9-month-old infant. Examination under general anesthesia revealed megalocornea, iris xanthogranuloma occupying the entire anterior chamber and high intraocular pressure. Skin lesions on the left lid and on the back were also found. Ocular hypertension resisted medical and surgical treatment. Cyclodestruction was necessary. The incidence of JXG is low (0.4%). The iris is the most frequently affected ocular tissue. Early diagnosis is necessary to avoid complications. Moreover, JXG can be associated in rare cases with neurofibromatosis or leukemia which must be systematically searched for.