Article

Surgical Indications for Nonrhegmatogenous Retinal Detachment

How acting sooner than later may preserve vision in selected cases.

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Rhegmatogenous retinal detachment is considered a sight-threatening emergency that generally necessitates timely surgical repair to achieve the best visual outcomes. Histopathologic studies in animals and humans have confirmed photoreceptor death secondary to the detachment.1-3 Macular height of macula-off retinal detachment correlates with worse visual outcomes due to possible suboptimal diffusion of nutrients from the retinal pigment epithelium (RPE) to the detached retina.4 Furthermore, the duration of macula-off retinal detachment has been shown to result in an exponential decay in final visual acuity (VA).5

The same factors influence final visual acuity in other types of retinal detachment. For instance, in central serous chorioretinopathy, the duration of macular detachment correlates with abnormal optical coherence tomography findings and final vision.6 This makes it very clear that a long-standing detachment of any kind will lead to poor long-term visual outcomes. So, why is it that we put less emphasis on intervening for serous retinal detachments?

Serous retinal detachment has many different causes, and the treatment varies depending on the specific pathology. Most cases do well with medical treatment for the underlying cause.7 However, cases that persist with submacular detachment can end up with poor VA and may even develop hypotony, leading to phthisis bulbi. For these cases, we believe that a surgical approach is the better option for achieving timely final retinal reattachment.

We share a series of cases and our experience in the treatment of nonrhegmatogenous retinal detachment with vitreoretinal surgical intervention. The study was performed in accordance with the United States Health Insurance Portability and Accountability Act (HIPAA) of 1996 and was approved by the Office of the Human Research Protection Program (Institutional Review Board) of the University of California, Los Angeles. The records of all patients who were managed with vitreoretinal surgical intervention with a retinal detachment of nonrhegmatogenous etiology were extracted from a surgical database of retinal detachment cases. To be considered for vitreoretinal surgery, the etiology of the patient’s illness had to be medically controlled; the patient was experiencing a deterioration in vision with worsening pathology despite efforts to manage the underlying condition; or the etiology was uncertain and no directed medical therapy was recommended.

Baseline patient and retinal detachment configuration; baseline, presurgical, and follow-up VA; surgical procedural details; and complications were tabulated. Surgery was performed by 1 of 2 surgeons. Six eyes of 5 patients underwent vitreoretinal surgical repair of a retinal detachment of nonrhegmatogenous etiology. Table 1 summarizes the cases.

CASE 1

A 66-year-old Hispanic male presented with unilateral uveitis of unknown etiology with exudative shallow macula-off retinal detachment and vision of counting fingers in the affected eye. While undergoing a systemic work-up, including neuroimaging and lumbar puncture, followed by a 1-month course of oral steroids, his vision declined to light perception. Six months after presentation, he underwent vitrectomy with retinotomy, perfluorocarbon liquid, endolaser, air-fluid exchange, and placement of 1,000-centistoke silicone oil. Three months after surgery, his macula remained attached under silicone oil, with persistent inferior retinal detachment with VA of counting fingers.

CASE 2

A 58-year-old white female presented with a diagnosis of bilateral panuveitis with dense vitritis in the affected eye with total bullous retinal detachment observed on B-scan ultrasonography and vision of hand movements. After undergoing a comprehensive work-up for all possible causes, an inflammatory etiology was presumed. Despite treatment with infliximab (Remicade; Janssen), she experienced continued poor vision. She underwent scleral buckling and vitrectomy with retinotomy, perfluorocarbon liquid, endolaser, air-fluid exchange, and placement of 1,000-centistoke silicone oil at 2 months following diagnosis of retinal detachment in the affected eye. One month after surgery, her retina remained completely attached with VA of 20/300.

CASE 3

A 69-year-old white male with a diagnosis of granulomatous uveitis due to presumed sarcoidosis experienced gradual unilateral decline in vision and was found to have a shallow macula-off retinal detachment, with vitreous opacities and VA of 20/125. Within 1 week, he underwent scleral buckling with external drainage, vitrectomy, air-fluid exchange, endolaser, and intraocular C3F8 gas tamponade. His vision improved to 20/80 at 6 months postoperatively with an attached retina. He had a moderately advanced nuclear sclerotic cataract and epiretinal membrane. Despite being eligible for surgical intervention the patient decided not to go through a second procedure.

CASE 4

A 45-year-old Asian female with a diagnosis of protein-losing enteropathy presented with bilateral bullous retinal detachments and VA of 20/400 in both eyes despite a 3-week course of oral steroids (Figure 1). Over the next 6 weeks while continuing steroid treatment, she developed progressive massive diffuse subretinal fibrosis (Figure 2). She underwent vitrectomy, retinotomy, release of subretinal bands, and 5,000 centistokes of silicone oil in each eye. Her VA improved to 20/80 in both eyes at 1 month postoperatively, but by 3 months, it had declined to 20/200 in both eyes despite retinal reattachment (Figure 3). Six months after the initial surgery, she underwent phacoemulsification, IOL implant, and silicone oil removal, with final VA of 20/80 and 20/125, respectively.

Figure 1. Bullous retinal detachment in both eyes in patient 4, a 44-year-old Asian female with protein-losing enteropathy. Her visual acuity was 20/400 in both eyes.

Figure 2. Progression of retinal detachment in patient 4, resulting in massive diffuse subretinal fibrosis in both eyes.

Figure 3. One month following vitrectomy, retinotomy, release of subretinal bands, and silicone oil placement for patient 4, retinal reattachment has occurred in both eyes. The vision initially improved to 20/80 in both eyes but decreased to 20/200 in both eyes during follow-up.

DISCUSSION

We present the cases of 6 eyes in 5 patients who underwent vitreoretinal surgery as part of the management of nonrhegmatogenous retinal detachment. The retinal detachment in case 5 was believed to be nonrhegmatogenous in nature, but advanced PVR was discovered intraoperatively. Despite visual improvement in the short term in each of these cases, there was a substantial delay (up to 6 months) between the initial diagnosis and vitreoretinal repair of retinal detachment.

There is little in the literature regarding surgical management of nonrhegmatogenous retinal detachment. Galor et al published a series of 5 cases of surgical drainage of nonrhegmatogenous retinal detachment of uveitic origin. In their series, the detachment duration ranged from 3 to 18 months, and the final vision improved in only 2 patients (summarized as part of Table 1).8 Similar to our series, vitreoretinal surgical repair was feasible and with minimal risk to the patient experiencing progressive blindness due to the underlying condition. It is possible that our series had better visual outcomes due to the shorter duration of retinal detachment in the group as a whole.

Table 1. Patients Who Underwent Vitreoretinal Surgical Repair of a Retinal Detachment of Nonrhegmatogenous Etiology
CASE ETIOLOGY CONFIGURATION DURATION VA BEFORE SURGERY VA AFTER SURGERY
McCannel et al
1 Unknown Shallow 6 mo LP CF
2 Unknown Bullous 2 mo HM 20/300
3 Sarcoid Shallow 1 wk 20/125 20/80
4 Protein Bullous 2 mo 20/400 20/200
5 Protein Bullous 2 mo 20/400 20/200
Galor et al8
1 Sarcoid Shallow 9 mo 20/40 20/40
2 Sarcoid Bullous 7 mo 20/200 20/60
3 JRA Bullous 3 mo 20/400 20/40
4 Sarcoid Bullous 18 mo HM NLP
5 HLA-B27 Shallow 4 mo CF CF
Kim et al7
1 Unknown No data 117 mo 20/5000 20/3000
2 Choroiditis No data 2 mo 20/400 20/400
3 Uveitis No data 1 mo 20/400 20/600
4 Coats No data 5 mo 20/10000 20/2000
5 Unknown No data 6 mo 20/400 20/2000
6 Unknown No data 3 mo 20/2000 20/200
7 Unknown No data 1 wk 20/10000 20/200
8 Uveitis No data 10 mo 20/400 20/1000
9 Uveitis No data 3 mo 20/10000 20/3000
10 Coats No data 67 mo 20/2000 20/2000
11 Uveitis No data 12 mo 20/10000 20/10000
12 Unknown No data 9 mo 20/2000 20/60
13 Uveitis No data 4 mo 20/10000 20/2000
14 Unknown No data 3 mo 20/2000 20/200
15 Choroiditis No data 8 mo 20/3000 20/100
16 Unknown No data 11 mo 20/100 20/50
17 Uveitis No data 6 mo 20/2000 20/400
Note: Boldface font indicates cases where visual acuity improved following surgery.
CF, counting fingers; VA, visual acuity; HM, hand movements; JRA, juvenile rheumatoid arthritis; LP, light perception; mo, month; NLP, no light perception; PVR, proliferative vitreoretinopathy; wk, week.

In the second publication addressing this issue, Kim et al7 presented 17 eyes with serous retinal detachment treated with surgical drainage and silicone oil tamponade. The range of detachment varied from 0 to 117 months, and the authors improved vision in 11 of 17 patients. Main complications included 8 cases of cataract, 2 cases of secondary glaucoma, and 2 cases silicone oil emulsifications. Thus, although there are few data, the high likelihood of visual improvement suggests that vitrectomy is a reasonable consideration for some patients with nonrhegmatogenous retinal detachment.

Generally, there has been reluctance to consider surgery as a management option in cases of nonrhegmatogenous retinal detachment, perhaps in part because nonrhegmatogenous retinal detachment is generally considered to be a medical disease in which only the underlying cause must be identified and treated to achieve resolution. However, the belief that the retinal detachment is simply a manifestation of an untreated etiology, obviating the role of surgical intervention, may be problematic in some instances. Often, the medical evaluation in search of the underlying etiology and treatment initiation take prolonged periods of time, during which the retina is detached. Or, the patient may achieve medical control of his or her disease, without evidence of immediate retinal reattachment. In both of these scenarios, the delay in retinal reattachment can lead to compromised vision outcomes.

Although there is a risk of reaccumulation of subretinal fluid following vitreoretinal surgery with subretinal fluid drainage due to the underlying etiology, the placement of silicone oil can mechanically approximate the macula to the RPE, allowing for improved vision, as in case 1. In such instances, the rationale for surgery and macular attachment follows the practice pattern of managing cytomegalovirus retinitis-related retinal detachments with silicone oil to achieve macular attachment, which resulted in improved vision despite sometimes persistent inferior retinal detachment.9-13

In ophthalmic oncology, exudative retinal detachment is present in most, if not all, malignant tumors to some extent, and the larger the tumor is, the more extensive the detachment. The use of 1,000-centistoke silicone oil as a vitreous-attenuating substance not only limits the radiation to the healthy nontumor tissues of the eye, but it also results in macular reattachment from a secondary retinal detachment.14 At our institution, the silicone oil is removed only after complete resolution of the serous detachment to maintain macular attachment after therapy. We expect that keeping the macula attached also contributes to better visual outcomes when silicone oil is used. The development of a cataract is frequent after vitrectomy and silicone oil placement; however, given the advances in cataract surgery, we do not see this as a serious issue. Cataract surgery can easily be performed simultaneously with or without the removal of silicone oil.

Without the use of silicone oil, serous retinal detachment often persists following radiotherapy. Although retinal reattachment may eventually occur with watchful waiting, vision in eyes in which a large serous retinal detachment has taken months to resolve may be limited. Once local tumor control has been achieved with radiotherapy, we recommend considering vitrectomy in all cases in which the macula is detached to maximize visual outcomes for patients.

In cases in which external-beam irradiation has been used to treat a choroidal metastatic tumor, there may be significant persistent of the serous detachment. To offer terminally ill patients the best possible vision, we recommend vitrectomy to repair the detachment.

Among the methods to consider when performing vitrectomy, we recommend rendering the patient pseudophakic at the time of surgery. Doing so eliminates the need for subsequent cataract surgery. In eyes with serous retinal detachment, the RPE/choroidal function may be altered and inefficient, compared to an eye with a rhegmatogenous retinal detachment. We recommend silicone oil for tamponade with superiorly placed peripheral retinotomies when possible. If an inadvertent inferior retinotomy is created, we recommend generous external support with a scleral buckling element. Although less critical in rhegmatogenous retinal detachment, the importance of complete retinal flattening during the fluid-air exchange in serous retinal detachments will help to maximize the tamponade effect. Placement of a buckle can help in cases where reaccumulation of subretinal fluid may displace the drainage retinotomy, creating a mixed retinal detachment. The buckle can support the retinotomy site, making it less likely that fluid could reach the drainage site, as well as supporting the vitreous base further minimizing the likelihood of postsurgical complications.

CONCLUSION

There is anatomic and functional evidence to support urgent retinal detachment repair irrespective of etiology, particularly in macula-off cases. We propose that vitreoretinal surgical management be considered sooner rather than later in cases of nonrhegmatogenous macula-off retinal detachments to reduce photoreceptor apoptosis, minimize progressive subretinal fibrosis, and result in better visual outcomes for patients with nonrhegmatogenous retinal detachment. Timely surgical treatment may be an adjunctive vision-saving or -restoring intervention to maximize vision outcomes, while medical management that identifies and treats the underlying etiology of the nonrhegmatogenous retinal detachment remains of the utmost importance. RP

REFERENCES

  1. Arroyo JG, Yang L, Bula D, Chen DF. Photoreceptor apoptosis in human retinal detachment. Am J Ophthalmol. 2005;139(4):605-610.
  2. Zacks DN, Hanninen V, Pantcheva M, et al. Caspase activation in an experimental model of retinal detachment. Invest Ophthalmol Vis Sci. 2003;44(3):1262-1267.
  3. Besirli CG, Chinskey ND, Zheng QD, Zacks DN. Autophagy activation in the injured photoreceptor inhibits fas-mediated apoptosis. Invest Ophthalmol Vis Sci. 2011;52(7):4193-4199.
  4. Ross W, Lavina A, Russell M, Maberley D. The correlation between height of macular detachment and visual outcome in macula-off retinal detachments of < or = 7 days’ duration. Ophthalmology. 2005;112(7):1213-1217.
  5. Ross WH, Kozy DW. Visual recovery in macula-off rhegmatogenous retinal detachments. Ophthalmology. 1998;105(11):2149-2153.
  6. Hassan TS, Sarrafizadeh R, Ruby AJ, et al. The effect of duration of macular detachment on results after the scleral buckle repair of primary, macula-off retinal detachments. Ophthalmology. 2002;109(1):146-152.
  7. Kim PS, Choi CW, Yang YS. Outcome and significance of silicone oil tamponade in patients with chronic serous retinal detachment. Korean J Ophthalmol. 2014;28(1):26-31.
  8. Galor A, Lowder CY, Kaiser PK, Perez VL, Sears JE. Surgical drainage of chronic serous retinal detachment associated with uveitis. Retina. 2008;28(2):282-288.
  9. Davis JL, Serfass MS, Lai MY, et al. Silicone oil in repair of retinal detachments caused by necrotizing retinitis in HIV infection. Arch Ophthalmol. 1995;113:1401-1419.
  10. Lim JI, Enger C, Haller JA, et al. Improved visual results after surgical repair of cytomegalovirus-related retinal detachments. Ophthalmology. 1994;101:264-269.
  11. Regillo CD, Vander JF, Duker JS, et al. Repair of retinitis-related retinal detachments with silicone oil in patients with acquired immunodeficiency syndrome. Am J Ophthalmol. 1992;113:21-27.
  12. Azen SP, Scott IU, Flynn HW Jr, et al. Silicone oil in the repair of complex retinal detachments. A prospective observational multicenter study. Ophthalmology. 1998;105:1587-1597.
  13. Nasemann JE, Mutsch A, Wiltfang R, Klauss V. Early pars plana vitrectomy without buckling procedure in cytomegalovirus retinitis-induced retinal detachment. Retina. 1995;15:111-116.
  14. McCannel TA, Kamrava M, Demanes J. 23-mm iodine-125 plaque for uveal melanoma: benefit of vitrectomy and silicone oil on visual acuity. Graefes Arch Clin Exp Ophthalmol. 2016;254(12):2461-2467.