ABSTRACT
The dislocation of a posterior chamber intraocular lens (PC IOL) because of constant eye rubbing is unusual and has never been reported in a child with Leber's congenital amaurosis (LCA). A 4-year-old full-term girl with an ocular history of LCA presented to the emergency room with a single-piece PC IOL dislocated into the anterior chamber (AC) of the left eye. There was no history of trauma or any other surgical intervention. A specific behavior known as Franceschetti's oculo-digital sign is a characteristic feature of LCA; this sign consists of repeated pressing, poking, and rubbing of the eyes with knuckles and fingers to stimulate the photoreceptors. This behavior caused the dislocation of the PC IOL into the AC. The dislocated IOL was explanted, and the patient was provided with aphakic glasses.
ABSTRACT
Juvenile xanthogranuloma (JXG) is an idiopathic granulomatous inflammatory condition that usually affects children. Intraocular involvement, especially bilateral, is rare in JXG. Most patients with ocular lesions are typically infants and usually present with hyphema, iridocyclitis, and secondary glaucoma. We report a case of a 3-month-old baby girl who presented to our 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. A detailed ophthalmic assessment was done under general anesthesia. The nodular lesion was excised and sent for histopathological evaluation, which confirmed the diagnosis of JXG. Treatment of JXG cases present a challenge to ophthalmologist due to rebleeding and refractory glaucoma. Our case was admitted multiple times for rebleeding and refractory glaucoma and was treated with full antiglaucoma drops, steroid drops and peribulbar injection of steroid.
