Chronic Pediatric Retinal Detachment with Multiple Macrocysts

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Retin Cases Brief Rep


PURPOSE: To describe a case of chronic pediatric retinal detachment with multiple macrocysts, its surgical management, and a review of the literature.

METHODS: Case report with fundus photography and optical coherence tomography.

RESULTS: We describe a case of an asymptomatic, 11-year-old male with a chronic rhegmatogenous retinal detachment with multiple peripheral macrocysts. The patient had count fingers visual acuity upon presentation. The detachment was successfully surgically repaired with scleral buckling, subretinal fluid drainage, cryotherapy, with a SF6 tamponade. At the 12-month follow-up, the retina remained attached with improvement of visual acuity to 20/100 with resolution of the cysts. Optical coherence tomography revealed loss of macular ellipsoid zone. Genetic testing revealed a heterozygous dominant COL11A1 mutation.

CONCLUSION: To the authors' knowledge, this is the first reported case of chronic retinal detachment presenting with multiple peripheral macrocysts in a pediatric patient with Stickler's Syndrome. More research is needed into the cause and significance of retinal macrocysts, particularly in the pediatric population.

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ePub ahead of print