Bilateral Spontaneous Hyphema in Juvenile Xanthogranuloma: A
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Juvenile xanthogranuloma (JXG) is a rare, benign, inflammatory histiocytic disorder that presents primarily in infancy. JXG can present at birth, but more than 80% of the cases present later, within the first year of life. Cutaneous lesions are the most common manifestations, which usually present as brownish papules, mostly in the head and neck region. The most frequent extracutaneous site of involvement in the eye.

Here reported a case of a 3-month-old baby girl who presented to the emergency department with bilateral hyphema that started 3 weeks ago. She was medically free with no history of any recent trauma or preceding febrile illness. General physical examination showed a quiet baby with multiple hyperpigmented macules over the inner thigh and right upper arm, with one pinkish nodule over the occiput. She also had high intraocular pressures. Ophthalmologists admitted the baby and she was started on topical antiglaucoma drugs, including timolol 0.25%, dorzolamide, latanoprost, and apraclonidine for both eyes. She was also administered steroid (Predforte) drops every 2 hours.

Two days later, the IOP normalized. At that time, the diagnosis of Juvenile Xanthogranuloma was confirmed based on histological findings of the biopsied tissue which showed Touton giant cells. The patient was then discharged, and a follow-up was given within 3 weeks. Yet, she presented again with high IOP and rebleeding in both eyes. On admission, a single dose of peribulbar steroid injection in the form methylprednisolone 10 mg was administered. The IOP normalized and the bleeding resolved after 3 days and the baby was sent home with topical 0.5% timolol, prostaglandin (Xalatan), apraclonidine (Iopidine), and steroid (Predforte) drops for a 1-month duration.

After 1 month, the bleeding had resolved completely in both eyes and the IOP had normalized with antiglaucoma drops given for 3 months. After about 5 months of being admitted and readmitted again, the bleeding had now resolved and the IOP is normal. Histopathologic examination of the biopsied tissue showed a cross-section of the skin lined by the hyperkeratotic epidermis. The dermis was infiltrated with histocytes highlighted by CD 68. Few Touton giant cells were observed.

Source:https://www.karger.com/Article/FullText/510905
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