Evolution and successful management of a large postvitrectomy macular hole

Abstract

We report the successful closure of a large postvitrectomy macular hole by temporal extension of internal limiting membrane (ILM) peeling. A 25-year-old man underwent pars plana vitrectomy, ILM peeling, and perfluoropropane gas tamponade for optic pit with chronic macular schisis and outer lamellar hole. The macular schisis deroofed into a large, full-thickness macular hole postoperatively. Optical coherence tomography revealed the horizontally oval shape of the secondary macular hole (658 × 824 μ). The best-corrected visual acuity (BCVA) was 20/200. Since ILM had already been peeled and the macular hole was widest horizontally, we combined a repeat gas tamponade with temporal extension of the ILM rhexis. Closure of the large macular hole with no central defect was observed and documented after the gas fill of the vitreous cavity was reduced sufficiently. BCVA remained unchanged for the initial months but gradually improved to 20/63 by the final follow-up visit at 5 years. A simple horizontal extension of ILM peel – in line with the shape of the hole – with repeat gas tamponade successfully closed a large secondary macular hole with progressive visual improvement.

Introduction

Optic pits are rare congenital anomalies of the optic nerve head (prevalence: 2 in 10,000) which cause visual loss only in the third and fourth decades of life due to waxing and waning accumulation of fluid within and under the macula.[1] The surgery is therefore usually performed on young adults with a variable history of visual loss. Subretinal and multilayered fluid in macula is associated with worse surgical outcomes and postoperative complications including macular hole formation.[2,3] When large, these macular holes are refractory to closure, more so when the internal limiting membrane (ILM) has already been peeled. We report the successful management of a large macular hole following vitrectomy for optic pit and long-standing maculopathy with a novel technique: Horizontal extension of the area of ILM peeling conforming to the shape of the macular hole. We are unaware of any report in the literature on matching the shape of ILM peeling to the shape of a macular hole.

Case Report

A 25-year-old man presented at a tertiary eye care hospital in India with gradual diminution of vision in the left eye for 5 years. His best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/120 in the left. Anterior segment biomicroscopy was unremarkable in both eyes. Fundus was normal in the right eye; the left eye had a temporal optic disc pit with macular elevation, and retinal pigment epithelial disturbance [Figure 1a and a]. Optical coherence tomography of the left eye revealed a multilayered macular schisis and outer lamellar hole, and central macular thickness was 647 μ. The patient was advised vitrectomy, but he sought further consultation and returned for surgery only after a year. The fundus picture remained largely unchanged, but the schisis had become extensive (central macular thickness 930 μ), with a near full-thickness outer lamellar hole and wafer-thin roof [Figure 1b]. He underwent vitrectomy, laser demarcation of the optic pit, ILM peeling, and nonexpansile perfluoropropane (C₃F₈ 15%) gas tamponade having been warned about the potential complications. A full-thickness macular hole was observed after the gas was absorbed. Since ILM had already been peeled, we waited for a month to observe for spontaneous reduction in the size of the hole. However, the macular hole enlarged from 518 μ to 824 μ (horizontal diameter) over this period [Figure 1c and d]. BCVA, which was unchanged postoperatively in spite of macular hole formation, had now dropped to 20/200. Since the ILM had already been peeled over the posterior pole, and the macular hole was observed to be horizontally oval (vertical diameter: 658 μ), the ILM rhexis was enlarged only in the horizontal direction (by approximately one-disc diameter) [Figure 2b]; C₃F₈ gas (15%) was repeated for tamponade. The macular hole was observed to be closed 1 month after resurgery [Figure 1e]. BCVA remained unchanged for about a year notwithstanding the cataract surgery at 10 months but gradually improved to 20/63 by the last follow-up over a period of 5 years [Figure 1f].

Figure 1.

(a): Left fundus shows temporal optic pit with serous retinal elevation and retinal pigment epithelial degeneration. (b) OCT shows multilayered retinal schisis, outer lamellar macular hole, and a wafer-thin roof. (c and d) Postoperatively, a full-thickness macular hole formed and enlarged to 824μ over a month. (e and f) One month after temporal extension of internal limiting membrane rhexis, the macular hole closed without central defect; note the residual bubble of perfluoropropane gas

Figure 2.

(a) Fundus autofluorescence image shows extensive submacular hypoautofluorescence due to preexisting retinal pigment epithelial degeneration. More intense signals at the macular hole and temporal to disc correspond to greater retinal pigment epithelial atrophy at the hole and postbarrage laser retinal pigment epithelial hypertrophy, respectively. (b) Intraoperative image clearly shows the fresh band of internal limiting membrane peeling temporally (~1 disc-diameter), contrasted against the dye-stained retina

Discussion

A conventional ILM peeling was performed for the optic pit with schisis, having observed excellent outcomes with this procedure previously in similar cases in spite of secondary macular hole formation.[3] However, the size of the macular hole was much bigger than those encountered previously;[3] and looked unlikely to respond to merely repeat gas tamponade. Since the hole was observed to be horizontally oval, we presumed the centrifugal tangential traction to be more predominant in the horizontal meridian. The temporal retina is also more compliant for centripetal movement after the release of traction.[4] Charles and colleagues have reported a temporal arcuate retinotomy for the treatment of large macular holes on the same principle.[5] Retinal autograft has also been proposed to close the defect in similar-sized macular holes.[6] A temporal extension of ILM rhexis appealed to us as a less aggressive alternative to the aforementioned procedures. Along with a long-acting gas tamponade, the procedure helped the extra-large macular hole to close rapidly and completely. Nonspecific enlargement of area of ILM peeling for refractory macular hole has been described previously with variable anatomic and functional success,[7] but in this case, ILM had already been peeled up to the arcades. Another surgical option suitable for a secondary macular hole is a free flap of ILM, harvested outside the arcades. This technique indeed reported 90% closure rates in a large case series.[8] The series included two macular holes secondary to ILM removal in optic pit maculopathy. While the smaller hole (281 μ) closed well with significant visual improvement, the larger macular hole (430 μ) showed a Type 2 (flat-open)[9] closure with no visual improvement. The free flap also required more manipulation and necessitated leaving viscoelastic substance in the eye.[8] Enlarging the previous ILM peel according to the shape of macular hole was simpler, less invasive, and made intuitive sense. In a chronic schisis with very large secondary macular hole and underlying retinal pigment epithelial degeneration, we obtained a Type 1 closure[9] with significant visual improvement. It is noteworthy that the visual improvement started only after a year but continued up to the last follow-up at 5 years. Such gradual incremental recovery is expected in a large macular hole.[10] The variable functional success in previous studies on large macular holes could partly be due to a shorter follow-up.[5,6,7] We report the successful closure of a large secondary macular hole with a simple, minimalistic procedure conforming to the shape of the macular hole.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.