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Peiquan Zhao; Ocular Anomalies and Treatments of Pediatric Incontinentia Pigmenti Patients. Invest. Ophthalmol. Vis. Sci. 2016;57(12):2057.
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© 2017 Association for Research in Vision and Ophthalmology.
To describe the ocular manifestations and treatments in a pediatric cohort of incontinentia pigmenti (IP) patients and to define the guidelines for staging and follow-up of vitreoretinal changes of IP.
This retrospective review was performed on patients under the age of 18 with definite diagnosis of IP. Data included demographics, medical history, ocular examination, assistant examinations, medical interventions performed, and final anatomical and visual outcomes. Vitreoretinal changes were divided into five stages.
Sixty-one consecutive children including 3 males and 58 females with median age of 3.7 months (range:0.2,72.0 months) were observed. The median follow up duration 6.5 months (range:0.5~69.0 months). Ocular anomalies were found in 77.05%(47/61) patients. 45.90% (28/61) cases presented with bilateral changes and 31.15%(19/61) with unilateral ones. 60.66%(74/122) eyes were affected and 59.85%(73/122) eyes had vitreoretinal changes. RPE defects (stage 1) were found in 8 eyes. Various retinal vascular anomalies were found in 22 eyes and macular ischemia in 3 eyes. Epi–retinal membranes (stage 3) were found in 6 eyes. Vitreous hemorrhage (stage 3) was found in 9 eyes. Partial retinal detachment (RD) (stage 4a) was seen in 10 eyes and 4 eyes were falciform-like. Total RD (stage 4b) was seen in 15 eyes. Phthisis bulbi or secondary glaucoma (stage 5) was seen in 8 eyes. 36.89% (45/122) eyes had vision-threatening anomalies. Sixteen eyes with secondary glaucoma or cataract received combined vitrectomy and lensectomy. Two eyes with ERM and two with vitreous hemorrhage received lens-sparing vitrectomy. Eighteen eyes received laser ablations for non-perfusion areas or abnormal vessels with fluorescein leakage. Five cases received bilateral intravitreal injection of Ranibizumab (0.25mg/0.025ml) for retinal neovascularizations.
High prevalence of various ocular lesions can be detected in IP patients, especially retinal vessel changes and consequential RD. Early-stage ocular lesions of IP may progress to more advanced stages, resulting in vision loss. Early screening, regular follow-up and timely treatments contribute to vision preservation for IP patients.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
Figure1. A.B Stage1:Retinal Pigmentation & hypo pigmentation ; C&D. Stage 2: Retinal vasculopathy; E.F Stage 3: ERM, retinal NV and NPA; G.H Stage 4a, 4b: Partial & total RD; I. Stage 5:Phthisis bulbi.
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