Purpose
To report the outcomes of current vitreoretinal surgical management of proliferative sickle retinopathy and to compare current methods to previous studies.
Design
A retrospective, interventional case series.
Methods
Fifteen eyes of 14 patients with proliferative sickle retinopathy were managed with vitreoretinal surgery over a 12-year period at a single institution.
Results
Nine patients had a sickle cell–hemoglobin C (SC) profile, 1 was sickle cell–beta + thalassemia (S beta + -thal), and 4 were sickle cell trait (AS). All 15 eyes underwent pars plana vitrectomy (PPV): 6 for vitreous hemorrhage (VH), 1 for epiretinal membrane (ERM), and an additional 8 for tractional retinal detachment (RD) and/or rhegmatogenous RD. In addition, an encircling scleral buckle (SB) was used in 2 cases. In 7 cases, 20 gauge PPV was used; 23 gauge was used in 3; and 25 gauge was used in 5. All 7 eyes with VH or ERM had improved vision postoperatively. Four of the 8 patients with traction and/or rhegmatogenous RD developed recurrent detachments and required a second operation. All retinas were attached at last follow-up, and visual acuity was 20/400 or better in all eyes. No cases of anterior segment ischemia were encountered.
Conclusions
Anterior segment ischemia is no longer a common occurrence in eyes undergoing surgery for proliferative sickle retinopathy. Although PPV has replaced the use of SB in many situations, an encircling SB may still be used in this population when necessary. Surgery for VH and ERM generally results in favorable outcomes, but eyes undergoing surgery for traction/rhegmatogenous RD carry a more guarded prognosis.
Proliferative sickle retinopathy is characterized by the development of peripheral retinal neovascularization, vitreous hemorrhage, and tractional or combined tractional-rhegmatogenous retinal detachment. Natural history studies demonstrate a high rate of autoinfarction of neovascular complexes in the absence of treatment, but selected cases require vitreoretinal surgical management to improve or stabilize vision.
The earliest surgical series for proliferative sickle retinopathy was published prior to the era of vitrectomy and reported a high rate of anterior segment ischemia, prompting some authors to recommend preoperative exchange transfusion and avoidance of an encircling scleral buckle. Subsequent reports demonstrated both improved outcomes and a lower rate of complications in this disease, despite the use of encircling scleral buckles in some cases.
The present study describes the indications, treatments, and outcomes for surgically managed cases of proliferative sickle retinopathy at the Bascom Palmer Eye Institute over the past 12 years and offers updated treatment recommendations compared to a previous study from this same institution.
Methods
This study was a retrospective, interventional case series of all patients undergoing vitreoretinal surgery from 2001-2013 with an International Classification of Diseases (ICD)-9 diagnosis code of sickle cell disease (282.60-282.68) or proliferative retinopathy not elsewhere classified (362.29). Data collected included demographics, sickle cell status, clinical indication for surgery, best-corrected visual acuity (BCVA), lens status, operative procedure(s), postoperative outcome, and duration of follow-up. Indications for surgical management included vitreous hemorrhage (VH), epiretinal membrane (ERM), traction retinal detachment (RD), rhegmatogenous RD, or combined traction/rhegmatogenous RD. The operating surgeon selected the surgical approach for each individual case; there was no defined management protocol in this study. The institutional review board at the University of Miami approved the study protocol, and Health Insurance Portability and Accountability Act (HIPAA) compliance was maintained.
Results
One hundred and sixty-six records were identified, but only 15 eyes of 14 patients were operated for a vitreoretinal condition associated with proliferative sickle retinopathy. One case was excluded because the patient had a penetrating keratoplasty and corneal ulcer prior to his vitreoretinal complication, and it was felt that these pre-existing conditions would complicate the analysis of his vitreoretinal management. Three patients had diabetes mellitus, and 1 of these 3 patients had early changes of nonproliferative diabetic retinopathy (this patient underwent surgery for ERM only). The other 2 patients showed no signs of diabetic retinopathy. All patients had at least 2 months of follow-up. There were 8 male and 6 female patients with a mean age of 48 years (range: 23-69) during the study period. Six right eyes and 9 left eyes had surgery. Fourteen of 15 eyes were phakic at the time of primary surgery. All operations were performed under local anesthesia. Hemoglobin subtypes were as follows: sickle cell–hemoglobin C (SC, 9), sickle cell–beta + thalassemia (S beta + -thal, 1), and sickle cell trait (AS, 4). The mean postoperative follow-up was 26.7 months (range: 2-140 months, Tables 1 and 2 ).
| Case | Sex | Age (Y) | Sickle Type | Diabetes | Eye | Preoperative Visual Acuity |
|---|---|---|---|---|---|---|
| 1 | Male | 67 | SC | No | Right | HM |
| 2 | Male | 59 | AS | No | Right | LP |
| 3 | Female | 59 | SC | No | Left | 20/200 |
| 4 | Male | 39 | AS | No | Left | CF 5 feet |
| 5 | Female | 61 | SB + Thal | Yes, without NPDR | Left | CF 3 feet |
| 6 | Female | 42 | SC | Yes, without NPDR | Left | HM |
| 7 | Female | 69 | SC | Yes, with NPDR | Right | 20/200 |
| 8 | Female | 54 | AS | No | Both | HM OD 20/40 OS |
| 9 | Male | 23 | SC | No | Left | 20/200 |
| 10 | Male | 28 | SC | No | Left | 20/200 |
| 11 | Male | 33 | SC | No | Left | 20/400 |
| 12 | Male | 49 | SC | No | Right | 20/80 |
| 13 | Female | 53 | AS | No | Left | HM |
| 14 | Male | 29 | SC | No | Right | HM |
| Case | Indication | Surgery | Valved Cannula | Last Recorded Visual Acuity | Follow-up |
|---|---|---|---|---|---|
| 1 | VH | 20G PPV, EL | No | 20/40 | 3 months |
| 2 | VH | 20G PPV | No | 20/20 | 40 months |
| 3 | VH, ERM | 20G PPV, MP, EL, air | No | 20/30 | 63 months |
| 4 | VH | 20G PPV, MP, EL, SF6 | No | 20/20 | 4 months |
| 5 | VH | Phaco/IOL, 23G PPV, EL, SF6 | No | 20/40 | 39 months |
| 6 | VH | 20G PPV, Cryotherapy | No | 20/30 | 140 months |
| 7 | ERM | 20G PPV, MP, EL | No | 20/60 | 50 months |
| 8 (OD) | RRD | SB, 23G PPV, EL, C3F8 | Yes | 20/60+2 | 11 months |
| 8 (OS) | RRD | 25G PPV, EL, C3F8 | Yes | 20/200 | 2 months |
| 9 | VH/TRD/RRD | SB, 20G PPV, MP, EL, SO | No | 20/400 | 24 months |
| 10 | TRD | 23G PPV, MP, EL, SO | Yes | 20/80 | 9 months |
| 11 | TRD, ERM | 25 G PPV, MP, EL, C3F8 | Yes | 20/300 | 2 months |
| 12 | TRD/RRD | 25G PPV, MP, EL, SO | Yes | 20/400 | 7 months |
| 13 | VH/TRD/RRD | Phaco/IOL, 25G PPV, EL, SF6 | Yes | 20/400 | 3 months |
| 14 | VH/TRD/RRD | 25G PPV EL, C3F8 | Yes | 20/100 | 3 months |
Six vitreoretinal procedures were performed for the primary indication of VH, and 1 operation was performed for ERM. Of these 7 eyes, 6 underwent 20 gauge pars plana vitrectomy (PPV) and 1 underwent 23 gauge PPV. Each of these procedures was performed with nonvalved cannulas. One patient (Case 1) developed a postoperative hyphema and elevated intraocular pressure that was successfully managed with topical steroid and ocular antihypertensive medications. The preoperative BCVA of the patients ranged from 20/200 to light perception, and all 7 eyes had significantly improved BCVA at last follow-up, ranging from 20/20-20/60 ( Table 2 ).
Eight eyes underwent vitrectomy for traction RD and/or rhegmatogenous RD. Of these 8 cases of detachment, 7 involved the macula. The preoperative BCVA ranged from 20/40 to hand motions (HM). An encircling scleral buckle (SB) was employed in 2 cases in order to provide additional support for peripheral traction: a #41 band was used in 1 case and a #240 band was used in the second case. In 1 case, 20 gauge PPV was used; 23 gauge was used in 2 cases; and 25 gauge was used in 5 cases. Valved cannulas were employed in all cases except for the 20 gauge PPV. Silicone oil was used in 3 cases and gas was used in 5 cases. Final BCVA was improved in 5 of these 8 cases, with cataract limiting visual potential in 1 of the remaining 3 cases. All eyes achieved anatomic success at last follow-up, and all eyes achieved 20/400 or better vision ( Figures 1 and 2 , Table 2 ).
The surgical objectives (clearance of vitreous hemorrhage, improvement of macular pucker, complete retinal reattachment) were achieved by a single operation in 9 of 15 eyes (60%); 6 of 15 eyes underwent additional vitreoretinal surgery, including 4 for recurrent RD, 1 for silicone oil removal, and 1 for ERM peeling ( Table 3 ). One case with an initial indication of VH developed a giant retinal tear following the initial vitrectomy but was managed successfully with pneumatic retinopexy. In addition, 7 eyes underwent subsequent cataract extraction. Exchange transfusion was not performed for any patients and anterior segment ischemia did not occur in any study eyes postoperatively.
| Case | Indication for Second Surgery | Second Surgery | Indication for Third Surgery | Third Surgery |
|---|---|---|---|---|
| 2 | Giant retinal tear | Pneumatic retinopexy | Cataract | Phaco/IOL |
| 3 | Cataract | Phaco/IOL | None | |
| 5 | ERM | 23G PPV, MP | None | |
| 7 | Cataract | Phaco/IOL | None | |
| 8 (OD) | Cataract | Phaco/IOL | None | |
| 9 | Cataract, retained SO, PVR, recurrent RD | Phaco/IOL, 20G PPV, SO removal, MP, EL, C3F8 | PVR, Recurrent RD | IOL removal, 20G PPV, MP, EL, SO |
| 10 | Cataract, retained SO | Phaco/IOL, 25G PPV SO removal, MP, EL | None | |
| 12 | PVR, recurrent RD | 25G PPV, MP, EL SO | None | |
| 13 | PVR, recurrent RD | 25G PPV, MP, PFO, EL, SO | None | |
| 14 | Cataract, PVR, recurrent RD | Phaco/IOL, 25G PPV, MP, EL, SO | None |
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