OSLI Retina

November 2020

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612 Ophthalmic Surgery, Lasers & Imaging Retina | Healio.com/OSLIRetina ■ C L I N I C A L S C I E N C E ■ Long-Term Follow-Up of Retinal Detachment Repair in Patients With Stickler Syndrome Andrew C. Lee, MD; Giovanni H. Greaves, MD; Brett J. Rosenblatt, MD; Vincent A. Deramo, MD; Eric P. Shakin, MD; David M. Fastenberg, MD; Philip J. Ferrone, MD BACKGROUND AND OBJECTIVE: To report the long- term anatomic and visual outcomes of patients with Stickler syndrome undergoing retinal detach- ment (RD) surgery. PATIENTS AND METHODS: Retrospective, interven- tional, consecutive case series of patients with Stickler syndrome undergoing RD repair from 1999 to 2017 at the Long Island Vitreoretinal Consul- tants, New York. Retinal attachment status and vi- sual acuity (VA) at 1-year and last follow-up were assessed. RESULTS: Successful reattachment was achieved in 28 of 29 eyes (97%) with an average of 2.3 surger- ies (including silicone oil removal surgeries). Rede- tachment after the first surgery occurred in 13 eyes (45%). Mean Snellen VA at initial presentation, 1-year follow-up, and last follow-up were 20/289, 20/118 (P = .012), and 20/103 (P = .022), respec- tively. CONCLUSIONS: Anatomic success can be achieved in most eyes. However, redetachments are com- mon, and multiple surgeries are often required. Reasonable visual recovery is possible in many eyes. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:612-616.] INTRODUCTION Hereditary arthro-ophthalmopathy, more com- monly referred to as Stickler syndrome, is a connec- tive tissue disorder characterized by orofacial, mus- culoskeletal, auditory, and ocular abnormalities. 1 Four distinct subgroups of this disorder have been described, each corresponding to a different mutation in genes encoding various collagen polypeptides. 2 Ocular manifestations are most frequently seen in type 1 and type 2 Stickler syndrome, both of which are inherited in an autosomal dominant fashion. 3 Defects in collagen result in strong vitreoretinal adhesions predisposing patients to retinal tears and detachments. 4 The risk of rhegmatogenous retinal detachment (RD) has been cited to be as high as 70% in type 1 Stickler syndrome 5 and between 40% to 50% in type 2 Stickler syndrome. 6 In addition to the vitreoretinal abnormalities, the majority of pa- tients with Stickler syndrome have a high degree of myopia. 1 Congenital cataracts and glaucoma are also common. 7,8 RD repair in Stickler syndrome presents a unique challenge to the vitreoretinal surgeon because of the propensity for giant retinal tears and proliferative vit- reoretinopathy (PVR). 2 Recent case series of RD repair in the Stickler population have shown rates of rede- tachment to be much higher than in typical RDs. 9-12 This study aims to describe the anatomic and visual outcomes of RD repair in this condition. From Zucker School of Medicine at Hofstra/Northwell, Department of Ophthalmology, Great Neck, New York (ACL, VAD, EPS, DMF, PJF); UCLA Stein Eye Institute, Los Angeles, California (GHG); and Long Island Vitreoretinal Consultants, Great Neck, New York (BJR, VAD, EPS, DMF, PJF). Originally submitted May 19, 2020. Revision received June 19, 2020. Accepted for publication July 9, 2020. Presented at the 2018 Meeting of the Club Jules Gonin in Jersey, Channel Islands (GB). The authors report no relevant financial disclosures. Address correspondence to Philip J. Ferrone MD, 600 Northern Blvd, Ste. 216, Great Neck, NY 11021; email: pjferrone@gmail.com. doi: 10.3928/23258160-20201104-02

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