Ocular surface XXXX in xeroderma pigmentosum: clinical spectrum and outcome.

@entity1 @entity5 @entity957 @entity6391 @entity735 @entity3169 @entity5436
BACKGROUND: To study the clinical spectrum and therapeutic outcome of ocular surface @entity5 (OSSN) in @entity1 with @entity6391 ( @entity6391 ). METHODS: The authors performed a retrospective review of records of @entity1 with @entity6391 referred to Cornea Services for management of OSSN. Presenting symptoms, clinical features, @entity5 morphology and location, treatment modalities, recurrence rates and visual outcome were noted. RESULTS: Seven @entity1 of @entity6391 (six males; one female) with bilateral OSSN were included in the study. All @entity1 were less than 15 years of age at presentation, with 13 of 14 lesions of OSSN (93%) occurring at the limbus. Associated ocular features were limbal stem cell @entity735 (64.3%), @entity735 (100%), @entity3169 in @entity735 (50%), pseudopterygium in two eyes (14.3%), anterior symblepharon in three eyes (21.4%) and conjunctival inflammatory granuloma in one eye (7.1%). Unaided visual acuity in the 14 eyes ranged from 6/6 to 6/36 at presentation. Six of the 14 eyes (42.9%) had histopathological features of @entity957 , and eight eyes (57.1%) demonstrated features consistent with @entity5436 . Recurrence was seen in nine of 14 eyes (64.3%). CONCLUSIONS: OSSN occurs predominantly in the elderly, but in @entity1 of @entity6391 it tends to occur at a younger age (6-22 years). The disease appeared to be more aggressive (recurrence rate = 64.3%) than usual. Awareness and prompt management with close follow up is warranted in these @entity1 .
