Incidence of and Risk Factors for Fellow-Eye Involvement in Sturge-Weber Syndrome Children With Unilateral Glaucoma.
Shin Young In, Ha Ahnul, Jeong Yoon, Huh Min Gu, Jeoung Jin Wook, Park Ki Ho, Kim Young Kook
AI Summary
This study found 15% of children with unilateral SWS glaucoma developed fellow-eye involvement, often in girls or with choroidal hemangioma, highlighting the need for vigilant monitoring.
Abstract
Précis: Among children with unilateral glaucoma associated with Sturge-Weber syndrome (SWS), 7 of 47 demonstrated involvement in the fellow eye, and that group had had earlier first-eye surgery relative to the noninvolvement group.
Purpose
The aim of this study was to determine the incidence of and risk factors for fellow-eye involvement in children with unilateral SWS-associated glaucoma.
Materials and methods
Children diagnosed with a unilateral facial port-wine stain and ipsilateral glaucoma before the age of 5 and followed up for at least 5 years were enrolled. The incidence rates of fellow-eye glaucoma involvement were estimated per 100 person-years, and factors associated with a higher incidence of fellow-eye involvement were investigated.
Results
A total of 47 children [24 (51.1%) girls] with unilateral SWS-associated glaucoma were included. All of them had facial port-wine stain involving ophthalmic division of the trigeminal nerve, and 18 (38.3%) had neurological comorbidities. The mean age at glaucoma diagnosis was 0.8±1.2 years [range, 0.08 (1 mo)-4.0 y]. Over a median follow-up of 8.4 years, glaucoma was diagnosed in the fellow eye of 7 of the children (14.9%; incidence rate of 1.8 per 100 person-years), 6 of whom were girls ( P =0.097) and 5 of whom were diagnosed before the age of 4 years ( P =0.508). The fellow-eye-involvement group showed significantly higher mean follow-up intraocular pressure in the fellow eye, older age at first-eye surgery (both P <0.005), and higher frequency of choroidal hemangioma both at first onset and in fellow eyes ( P =0.026 and 0.019, respectively).
Conclusions
In this cohort of SWS children diagnosed with unilateral glaucoma, the risk of fellow-eye involvement was higher in girls, within the first 4 years, and in cases with choroidal hemangioma. The fellow-eye-involved children underwent surgery on the first eye earlier than those without fellow-eye involvement.
MeSH Terms
Shields Classification
Key Concepts4
Among children with unilateral glaucoma associated with Sturge-Weber syndrome (SWS), 7 of 47 (14.9%) demonstrated involvement in the fellow eye, with an incidence rate of 1.8 per 100 person-years.
The fellow-eye-involvement group of children with unilateral SWS-associated glaucoma showed significantly higher mean follow-up intraocular pressure in the fellow eye (P <0.005) compared to the non-involvement group.
Children with unilateral SWS-associated glaucoma who developed fellow-eye involvement had an older age at first-eye surgery (P <0.005) compared to those without fellow-eye involvement.
The risk of fellow-eye involvement in children with unilateral SWS-associated glaucoma was higher in girls (6 of 7 fellow-eye involvement cases were girls, P =0.097), within the first 4 years (5 of 7 fellow-eye involvement cases diagnosed before age 4, P =0.508), and in cases with choroidal hemangioma both at first onset (P =0.026) and in fellow eyes (P =0.019).
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