Risk and Prevention of Retinal Detachments in Patients with Stickler Syndrome

Abstract

BACKGROUND AND OBJECTIVE: To report the impact of prophylactic laser retinopexy in the prevention of retinal detachments (RDs) in patients with Stickler syndrome. PATIENTS AND METHODS: This was a retrospective, comparative case series of patients with Stickler syndrome from the year 2000 to 2019. We compared the rate of RDs between individuals who did and did not receive prophylactic laser therapy. In patients with an RD, we compared the rate of RD in the fellow eye with and without prophylactic laser treatment. RESULTS: A total of 95 eyes were identified. Fifty-four percent of the overall population was female. The RD rate was 26.7% among eyes without previous prophylactic laser retinopexy and 4.6% among eyes with previous prophylactic laser retinopexy. A Cox proportional model revealed that laser prophylaxis treatment had a statistically significant effect on the risk of RD or retinal tear during the 25-year survival period from birth ( P = .034). Eyes receiving treatment were 70% less likely to experience RD or retinal tear (hazard ratio, 0.297; 95% CI, 0.097 to 0.913). CONCLUSIONS: This study's results suggest a potential role for prophylactic laser retinopexy in the prevention of rhegmatogenous RD among patients with a clinical diagnosis of Stickler syndrome. [ Ophthalmic Surg Lasers Imaging Retina . 2022;53:7–11.]

Preventing Retinal Detachment: Where are We? Implications from Stickler Syndrome

“…Many reports of encircling laser prophylaxis give little description of technique. 4 , 5 , 36 , 37 But its safety and effectiveness can be quite technique dependent. We plan to publish a comprehensive description of optimal technique in a sequel to this article.…”

“…3,33 Naravane et al and Khanna et al subsequently reported similarly large reductions in RRD rates in (predominately clinically diagnosed) SS, in larger series, using a form of encircling laser retinopexy extending to approximately the equator (26.7% vs 4.6%, p = 0.034; 73% vs 3%, p = 0.001, respectively). 4,5 Linton et al recently reported a fourth encircling laser prophylaxis study for (predominately genetically confirmed) SS, confining treatment to the ora region as a GRT prophylaxis. 34 RRD was reduced from 23% to 9% (p = 0.057), with no GRT lesions seen in the eyes that detached 4318 despite prophylaxis (vs 42.9% GRT in control eyes detaching).…”

“…It then discusses the implications of recent successful prevention treatments in Stickler syndrome for high-risk eyes with other, non-syndromic conditions. [3][4][5] Without treatment, spontaneous detachment of the (neurosensory) retina from the eye wall is the leading cause of acute blindness in the aging eye. Treatment became possible only during the 20th century, after Jules Gonin proved that a full thickness defect in the retina allowed vitreous cavity fluid to enter the potential subretinal space (Figure 1).…”

Approaches to Retinal Detachment Prophylaxis among Patients with Stickler Syndrome

“…In 2022, two additional studies evaluating laser retinopexy were published. The first retrospective case series examined a cohort of 95 eyes from 48 patients and found that the retinal detachment rate was 26.7% among eyes without previous prophylactic laser retinopexy and only 4.6% among eyes with previous prophylactic laser retinopexy [25]. Laser burns of approximately 500 microns applied with a power titrated to a gray-white color in a nearly confluent pattern of 7-10 rows from the ora serrata for 360 degrees was performed in one session (Figure 1).…”

Quality of Life in Children and Adolescents with Stickler Syndrome in Spain

“…Regarding other usual problems, a total of 40–80% of patients with SS develop retinal detachments and tears. However, these alterations are corrected using prophylactic laser treatment, which was shown to be effective in the treatment of these disorders [ 12 ].…”