The prevalence of macular hole (MH) coexistent with rhegmatogenous retinal detachment (RRD) with peripheral break (RRD-MH) is thought to be 2% to 8%, depending on the study.1,2 However, some have postulated that macular holes may go undetected upon initial evaluation of a patient with RRD and that the real prevalence of RRD-MH may actually be on the high end of the reported spectrum in macula-off retinal detachment (RD).
There is no consensus as to the preferred treatment for this clinical scenario. This review serves to elucidate some general principles in the management of this complex condition.
AT A GLANCE
• MH with RRD can present a complex surgical challenge, and no best practice patterns have been defined.
• It is feasible in most cases to peel ILM over detached retina using a pinch-and-peel technique. However, it may not be required for most MHs to close in the setting of RD.
• If peeling is performed, care should be taken to prevent subretinal migration of the staining agent and enlargement of the MH.
• Generally, high rates of MH closure and retinal reattachment can be achieved.
Is RRD-MH associated with proliferative vitreoretinopathy (PVR)? The pathophysiology of MH in RRD is not entirely clear, but one hypothesis is that retinal pigment epithelium (RPE) cells released from a peripheral break attach to the macular surface, contract, create tangential traction, and cause an MH, as in a PVR process.1
Another theory is that a posterior vitreous detachment, in addition to causing a peripheral break, may put tangential traction on the central macula, leading to an MH.2 In a retrospective study including 16 patients with RRD-MH, Najafi et al reported that 18% of the patients developed an RD in the fellow eye,1 an incidence slightly higher than the incidence of RD in the general population, of 2% to 11%.1
It is possible that patients with RRD-MH may have underlying vitreoretinal interface abnormalities that predispose to RRD and MH. Also, nine of the 16 eyes (53%) in the Najafi et al study had coexisting PVR. Along these lines, Cunningham et al reviewed 607 patients who underwent surgical repair for RRD and found that MH was present in 7.3% of cases of RRD with PVR, but only 1.4% of cases of RD without PVR.2 This finding suggested that, in the setting of RRD, MH and PVR may be significantly associated.
MANAGEMENT: SURGICAL STRATEGIES
The original strategy for repair of RRD-MH was to repair the RRD by creating adhesion of the peripheral breaks and to disregard the MH, as leaving the MH open only rarely prevents RRD from reattaching.3,4 However, Ah Kiné et al showed that closure of MH significantly improves final BCVA.3
By Lianna Valdes, MD; Patrick Oellers, MD; and John B. Miller, MD
Case No. 1
A 60-year-old patient presented with dialysis retinal detachment and associated macular hole (MH). He related a history of significant blunt trauma a few years earlier. There was a pigmented demarcation line associated with the dialysis, which was broken through with subretinal fluid on presentation. The patient underwent 25-gauge pars plana vitrectomy, scleral buckle, and internal limiting membrane (ILM) peeling aided by indocyanine green staining and C3F8 gas. The ILM was peeled using a pinch-and-peel technique with end-grasping forceps (Figure A, B). A broad sheet of ILM was then peeled across the MH (C) without causing any enlargement of the MH. At 9 months postoperative, the retina remains attached with closed MH and VA of 20/40 (D, E).
Case No. 2
A 53-year-old patient presented with total retinal detachment with MH. He had previous laser demarcation performed outside the country (Figure A, B). The patient underwent 23-gauge pars plana vitrectomy, scleral buckle, and ILM peel assisted with indocyanine green staining and C3F8 gas. At 3 months postoperative, his retina was attached with closed MH. BCVA was 20/25 (C).
With the goal of repairing the MH as well as the RRD in mind, two approaches can be taken. In a sequential approach, only the RRD repair is addressed in primary surgery, and after the retina is attached, a second surgery is performed to repair the MH if needed. In a combined approach, the RRD and MH are both addressed in one surgery with internal limiting membrane (ILM) peeling over the macula.
Subretinal fluid can be drained through the MH, a peripheral break, or a posterior retinotomy. What is the best route to use? In a study in which fluid was drained through a posterior retinotomy, the MH closure rate was 87%.5 In two studies in which fluid was drained through a peripheral break, the closure rate was 100%.3,6 In two studies in which fluid was drained through the MH, the closure rate varied greatly, at 30% and 91%.4,7
Drainage of subretinal fluid through the MH may enlarge the hole and disrupt photoreceptors and RPE cells. As a result, this approach may be associated with worse VA outcomes and closure rate, so we advise against it.
TO PEEL OR NOT TO PEEL?
In theory, peeling the ILM in surgery for RRD-MH can improve surgical outcomes by removing any remaining tractional forces across the macula. In 49 cases of surgical repair of RRD-MH included in one study, 39 of 43 (90.7%) holes closed with ILM peeling, compared with only two of six (33.3%) closing without ILM peeling.7 This finding suggests that ILM peeling increases the chance of MH closure in the setting of concurrent RD. However, with only six cases in which ILM was not peeled, the small sample size limits the strength of any statistical conclusion.
In a retrospective analysis of 10 patients by Ah Kiné et al,3 all MHs closed without ILM peeling. In a prospective study, Shukla et al separated patients undergoing RRD-MH repair into two groups: One underwent ILM peeling, and the other did not.5 In this study, 14 of 17 of the MHs treated with ILM peeling closed, compared with 13 of 14 of the MHs treated without ILM peeling. Of note, ILM peeling in this study was associated with worse final VA.
Overall, ILM peeling does not seem to be mandatory. Peeling the ILM in the setting of RD can be technically challenging, as the detached retina gives way in the direction of the peeling. Also, because of a lack of countertraction, a pinch-and-peel technique is usually required, and flap creation with diamond-dusted membrane scraper (multiple vendors) or Finesse Flex Loop (Grieshaber/Alcon) does generally not work well over detached retina.
Care must be taken not to enlarge the macular hole during membrane peeling. Use of perfluorocarbon liquid (perfluoro-n-octane, PFO) to provide countertraction has been suggested by several authors. However, this may have its own associated difficulties, as there is a risk for subretinal retention of PFO and because PFO flattens elevated ILM flaps toward the retinal surface.
In a recently introduced technique, Chirag D. Jhaveri, MD, suggests that a PFO “marble,” 1 to 2 disc areas in size, can be used to provide countertraction, can allow manipulation of the flap under balanced saline solution, and can be more easily kept away from the MH.8
Staining agents may be used, but they must be kept from going to the subretinal space. In this regard, indocyanine green (ICG) dye can be toxic to the retina and RPE with high concentrations or prolonged exposure. It is likely that brilliant blue G dye has a better safety profile, but no formulation approved by the FDA is available, and it cannot be easily obtained by all retina specialists in the United States.
We believe that ILM peeling is feasible in most cases without the use of PFO. We prefer a pinch-and-peel technique, with cautious staining using ICG or brilliant blue G. However, in cases in which ILM peeling is technically challenging, for example due to poor surgical view or a bullous nature of the macular detachment, it is often advantageous to prioritize the surgical steps to repair the RRD without peeling of the ILM, given the high closure rate of MH in RRD even without ILM peeling.
WITH COEXISTING PVR
There is a high prevalence of PVR in RRD-MH, and we recommend aggressive surgical treatment overall, with possible consideration of adding an encircling scleral buckle. In cases with definite PVR present, we believe that ILM peeling can have an added benefit to MH closure. In these cases, the edges of peeled ILM can be peeled further toward the periphery to remove concurrent PVR membranes and thereby remove any scaffold for PVR regrowth.
We believe that C3F8 gas is favorable over silicone oil in most cases, due to a higher likelihood of MH closure. As for any MH cases in general, optimal gas fill is desired. We usually reserve silicone oil for patients who need a large peripheral relaxing retinectomy or in selected cases for patients who are unable to maintain facedown positioning.
RRD-MH can present a complex surgical challenge, and no best practice patterns have been defined. There is no strong evidence in favor of ILM peeling, but, when peeling is performed, care should be taken to prevent subretinal migration of the staining agent—especially ICG—and enlargement of the MH.
There seems to be an association of RRD-MH with PVR, and we recommend a low threshold for surgical strategies to treat PVR such as scleral buckle and membrane peeling. Generally, high rates of MH closure and retinal reattachment can be achieved. As always, the best strategies depend on the individual surgeon’s experience and the specific parameters of the case (see Case Examples).
1. Najafi M, Brown JS, Rosenberg KI. Increased reoperation rate in surgical treatment of rhegmatogenous retinal detachment with coexistent macular hole. Ophthalmology Retina. 2017;2(3):187-191.
2. Cunningham MA, Tarantola RM, Folk JC, et al. Proliferative vitreoretinopathy may be a risk factor in combined macular hole retinal detachment cases. Retina. 2013;33(3):579-585.
3. Ah Kiné D, Benson SE, Inglesby DV, Steel DH. The results of surgery on macular holes associated with rhegmatogenous retinal detachment. Retina. 2002;22(4):429-434.
4. O’Driscoll AM, Goble RR, Kirkby GR. Vitrectomy for retinal detachments with both peripheral retinal breaks and macular holes. An assessment of outcome and the status of the macular hole. Retina. 2001;21(3):221-225.
5. Shukla D, Kalliath J, Srinivasan K, et al. Management of rhegmatogenous retinal detachment with coexisting macular hole: a comparison of vitrectomy with and without internal limiting membrane peeling. Retina. 2013;33(3):571-578.
6. Singh AJ. Combined or sequential surgery for management of rhegmatogenous retinal detachment with macular holes. Retina. 2009;29(8):1106-1110.
7. Ryan EH, Bramante CT, Mittra RA, et al. Management of rhegmatogenous retinal detachment with coexistent macular hole in the era of internal limiting membrane peeling. Am J Ophthalmol. 2011;152(5):815-819.e811.
8. Jhaveri CD. PFO marble for macular hole retinal detachments. American Academy of Ophthalmology website. Accessed February 14, 2019. www.aao.org/clinical-video/pfo-marble-macular-hole-retinal-detachments.
John B. Miller, MD
• Assistant Professor of Ophthalmology, Harvard Medical School, Boston
• Director of Retinal Imaging, Massachusetts Eye and Ear, Harvard Medical School, Boston
• Financial disclosure: Consultant (Alcon, Allergan, Heidelberg, Optovue, Zeiss)
Patrick Oellers, MD
• Retina Surgeon, Retina-Vitreous Surgeons of Central NY, PC; Assistant Professor of Ophthalmology, State University of New York, Upstate Medical Center; both in Syracuse, New York
• Financial disclosure: None
Lianna Valdes, MD
• Chief Ophthalmology Resident, State University of New York, Upstate Medical Center, Syracuse, New York
• Financial disclosure: None