Purpose: To report a pediatric case of exudative bullous retinal detachment and uveal effusion syndrome (UES) in Kenny-Caffey syndrome (KCS) complicated by nanophthalmos. Methods: Multimodal imaging evaluation and clinical outcome of a pediatric patient presenting emergently with decreased visual acuity. Results: A 13-year-old girl with KCS presented with 2-day blurred vision in the LE (20/100, +17 D; RE 20/20, +15 D). Fundus exam revealed exudative bullous retinal detachment involving the macula and temporal periphery. Ultrasound showed scleral and choroidal thickening with short axial length, consistent with nanophthalmos. The patient was treated with oral prednisone and acetazolamide. Complete resolution of subretinal fluid was observed at 6-week follow-up, with improved visual acuity. Conclusion: We describe a rare association of KCS, nanophthalmos, and UES. Conservative medical therapy may be an effective alternative to surgery mostly in pediatric cases.
Kenny-Caffey syndrome: nanophthalmos complicated by uveal effusion, presentation and treatment / Romano, A., Bellucci, C., De Santis, A., Spaggiari, E., Mora, P.. - In: RETINAL CASES & BRIEF REPORTS. - ISSN 1935-1089. - (2026). [10.1097/icb.0000000000001950]
Kenny-Caffey syndrome: nanophthalmos complicated by uveal effusion, presentation and treatment
Romano, Alessandra;Bellucci, Carlo;De Santis, Antonio;Mora, Paolo
2026-01-01
Abstract
Purpose: To report a pediatric case of exudative bullous retinal detachment and uveal effusion syndrome (UES) in Kenny-Caffey syndrome (KCS) complicated by nanophthalmos. Methods: Multimodal imaging evaluation and clinical outcome of a pediatric patient presenting emergently with decreased visual acuity. Results: A 13-year-old girl with KCS presented with 2-day blurred vision in the LE (20/100, +17 D; RE 20/20, +15 D). Fundus exam revealed exudative bullous retinal detachment involving the macula and temporal periphery. Ultrasound showed scleral and choroidal thickening with short axial length, consistent with nanophthalmos. The patient was treated with oral prednisone and acetazolamide. Complete resolution of subretinal fluid was observed at 6-week follow-up, with improved visual acuity. Conclusion: We describe a rare association of KCS, nanophthalmos, and UES. Conservative medical therapy may be an effective alternative to surgery mostly in pediatric cases.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
