Stickler Syndrome (SS): Laser Prophylaxis for Retinal Detachment (Modified Ora Secunda Cerclage, OSC/SS)

Abstract

Purpose: To introduce a novel technique of encircling laser prophylaxis (ora secunda cerclage Stickler syndrome, OSC/SS) to prevent rhegmatogenous retinal detachment (RRD) in Stickler syndrome eyes.

Patients and methods: After first eye RRD at age 50 and at age 18, respectively, a 53-year-old father and his 22-year-old son with type 2 SS (STL2) gave informed consent and underwent OSC/SS prophylaxis, performed in each fellow eye. A 26-year-old STL2 daughter then suffered first eye retinal detachment and similarly chose fellow eye OSC/SS prophylaxis. A second son, 28 years of age with STL2, chose OSC/SS prophylaxis in both eyes.

Results: The three OSC/SS treated fellow eyes have gone 12 years, 11 years, and 8 years without RRD. STL1 and less common STL2 eyes are known to have a similar rate of RRD, and 80% of STL1 fellow eyes develop RRD at a median of 4 years in the absence of prophylaxis. Moreover, five of six (83%) known STL2 family members suffered RRD, only the STL2 son with bilateral OSC/SS remaining bilaterally attached. All five OSC/SS treated eyes (average 8.7 years post-prophylaxis) retained preoperative visual acuity of 20/20 to 20/30, with an average, asymptomatic reduction of meridional field in each eye to 50 degrees. In contrast, in the three eyes having suffered RRD prior to presentation, visual acuity ranged from 20/125 to 8/200 and average meridional field was 29 degrees.

Conclusion: Encircling grid laser (OSC) modified in Stickler eyes to encompass the ora serrata and extend posteriorly to and between the vortex vein ampullae (OSC/SS) is a reasonable RRD prophylaxis option to offer STL1 and STL2 patients as an alternative to no treatment or less effective prophylaxis. Because of rarity and severity, the ultimate proof of safety and efficacy will likely come not from randomized trials, but from a non-randomized, prospective, cohort comparison study of such individual efforts.

Keywords: OSC; OSC/SS; Ora Secunda Cerclage; SS; STL1; STL2; Stickler syndrome; encircling laser prophylaxis; giant retinal tear; retinal detachment prevention.

Figure 1

Illustration of OSC. Laser burns of moderate intensity are placed in a grid pattern (one to two spot widths separation) extending from the ora serrata approximately 4 mm posteriorly, in effect producing a “second ora” posterior to the vitreous base. Used with permission of artist Stephen Gordon, copyright 2020.

Figure 2

(A) Final appearance of the father’s right eye fundus after repair of recurrent RRD/PVR in multiple procedures. Postoperative visual acuity is 8/200. Multiple causative tears with aberrant vitreous traction were noted at RRD repair. (B) Visual field of the right eye postoperatively.

Figure 3

(A) Final appearance of the son’s left eye after repair of recurrent RRD/PVR. Postoperative visual acuity is 20/125. Initial RRD was from multiple small defects with lattice. (B) Visual field of the left eye postoperatively.

Figure 4

(A) Step 1 (essential) of OSC/SS. Laser burns of moderately high intensity are placed in a tight grid pattern (one spot width separation) from 2 mm onto the pars plana to the ora serrata, and approximately 4 mm posteriorly, halfway to the vortex vein ampullae, achieving protection against GRT and anterior defects. Artist Stephen Gordon. (B) Step 2 (optional) of OSC/SS. Three months after initial treatment, the laser grid is extended posteriorly to and between the vortex vein ampullae, achieving maximum protection against both GRT and posterior defects throughout the peripheral retina. Used with permission of artist Stephen Gordon, copyright 2020.

Figure 5

(A) Fundus image of the daughter’s right eye after completed OSC/SS laser prophylaxis. Visual acuity is 20/30 (as preoperatively) corrected with −20.25 +3.75 × 091. (B) Visual field of the right eye post OSC/SS.

Figure 6

Fundus image of pigmented perivascular retinal degeneration extending radially and posteriorly, as seen in some Stickler syndrome pedigrees.

Figure 7

Fundus image of a giant retinal tear (GRT) extending from 9 to 5 o’clock in the left eye.  GRT is most commonly seen in childhood retinal detachments of Stickler syndrome patients.

Figure 8

(A) Intraoperative image of seven post equatorial retinal tears extending from 8 to 10 o’clock temporally along a prominent circumferential line of vitreous traction, causing total RRD in the right eye of a 14-year-old female with STL1, congenital extreme myopia (26 diopters), and abnormal vitreous (Video S3). These tears occurred well posterior to standard OSC prophylaxis performed 4 months previously. (B) Postoperative image of the same retina reattached under silicone oil, with corrected visual acuity in the eye of 20/30. Note the prominent line of vitreous traction from 5 to 11 o’clock that could not be safely removed during the initial repair despite retinal stabilization with perfluorocarbon liquid. Laser retinopexy extends quite posteriorly to encompass all tears and the traction line that was further reduced upon silicone oil removal.

Figure 9

Intraoperative image of vitreous veils in the left eye of the elder son, an STL2 patient (Video S2).