Hyphaema, heterochromia and hyperpigmented cutaneous nodules.

Area of Science:

• Ophthalmology
• Pediatrics
• Dermatology

Background:

• Juvenile xanthogranuloma (JXG) is a rare, benign granulomatous disorder.
• It primarily affects children, often presenting with cutaneous lesions.

Observation:

• An 11-month-old girl presented with systemic and ocular JXG manifestations.
• Ocular findings included hyphema, elevated intraocular pressure (IOP), and glaucomatous optic neuropathy.

Findings:

• Histopathology confirmed JXG with systemic involvement.
• The patient experienced recurrent hyphema despite initial topical steroid and antiglaucoma treatment.

Implications:

• This case highlights the challenges in managing JXG ocular complications, including rebleeding and refractory glaucoma.
• A multidisciplinary approach involving chemotherapy was effective in managing severe JXG manifestations.