Idiopathic Macular Holes
Idiopathic macular holes occur at a rate of approximately 8 per 100,000 persons per year and have a female-to-male ratio of 2 to 1. They occur mostly in the sixth through eighth decades of life and can appear at a younger age in myopic eyes. Idiopathic macular holes are bilateral in approximately 10% of patients. Investigations using OCT suggest that idiopathic macular holes are caused by the same tractional forces as the forces associated with perifoveal vitreous detachment and thus are likely an early stage of age-related PVD.
Figure 17-7 Macular holes. A, Color fundus photograph of stage 1A hole with horizontal splitting of retinal layers and corresponding OCT scan showing stage 1A hole. B, Fundus photograph and corresponding OCT scan of stage 1B hole. C, Fundus photograph of stage 2 hole with small opening in inner layer eccentrically and corresponding OCT scan of stage 2 hole. D, OCT scan of stage 3 full-thickness hole with elevation of adjacent retinal edges. E, OCT scan of stage 4 full-thickness hole with operculum.
(Courtesy of Mark W. Johnson, MD, and Peter K. Kaiser, MD.)
The following description of the stages of macular hole formation is useful in making management decisions (Fig 17-7):
A stage 0, or premacular, hole occurs when a PVD with persistent foveal attachment develops. Subtle loss of the foveal depression can be observed, and visual acuity is usually unaffected. Most stage 0 holes do not progress to advanced stages. This stage represents a VMA.
A stage 1 macular hole (impending macular hole) typically causes visual symptoms of metamorphopsia and central vision decline, usually to a visual acuity range of 20/25 to 20/60. The characteristic findings are either a small yellow spot (stage 1A) or a yellow circle (stage 1B) in the fovea. OCT examination reveals that a stage 1A hole is a foveal “pseudocyst,” or horizontal splitting (schisis), associated with vitreous traction to the foveal center. A stage 1B hole indicates a break in the outer fovea, the margins of which constitute the yellow ring noted clinically. Spontaneous resolution of a stage 1 hole occurs in approximately 50% of cases without ERM. This stage represents VMT syndrome.
A stage 2 macular hole represents an early full-thickness macular hole that is less than 400 μm in diameter. It results from the progression of a foveal schisis (pseudocyst) to a full-thickness dehiscence, as a tractional break develops in the “roof ” (inner layer) of the pseudocyst. Progression to stage 2 is accompanied by a further decline in visual acuity. OCT demonstrates the full-thickness defect and the continuing attachment of the posterior hyaloid to the foveal center. This stage represents VMT syndrome with a small- to medium-sized macular hole.
A stage 3 macular hole is a fully developed hole (≥400 μm in diameter), typically surrounded by a rim of thickened and detached retina. Visual acuity ranges widely. The posterior hyaloid remains attached to the optic nerve head but is detached from the fovea. An operculum suspended by the posterior hyaloid may be seen overlying the hole. On OCT, this stage represents a large macular hole with no VMT (Activity 17-3, Fig 17-8).
A stage 4 macular hole is a fully developed hole with a complete posterior vitreous detachment, as evidenced by the presence of a Weiss ring. On OCT, this stage represents a large macular hole with no VMT.
OCT Activity: Macular hole.
Courtesy of Colin A. McCannel, MD.
The fellow-eye risk of macular hole development depends on the vitreous attachment status. If a complete vitreous detachment is present in the fellow eye, there is little, if any, risk of macular hole development. If the fellow eye has stage 1A abnormalities, however, there is a substantial risk of progression to a full-thickness macular hole. When the fellow eye is normal and its vitreous is attached, the risk of developing a macular hole in that eye is approximately 10%, the rate of bilaterality.
For stage 2 or greater macular holes, surgical intervention is recommended—specifically, pars plana vitrectomy with gas tamponade; in most recent case series, the success rate of this procedure for closure and vision improvement was greater than 90% (see Chapter 20 in this volume). Modifications of routine macular hole surgery are usually reserved for very large, chronic, or nonclosing holes; these include inverted ILM flaps and autologous retinal grafts composed of peripheral retina. Although these techniques may achieve anatomical hole closure, visual acuity may not improve.
Figure 17-8 SD-OCT line cube scan of the left macula of a 62-year-old woman who suffered loss of vision 18 months prior to presentation. At the time of the scan, the visual acuity of this eye was 20/40 (eccentric fixation). The infrared reflectance image (left) shows a hole in the central macular retina. See also Activity 17-3; scrolling through the images, a discontinuity of the retina in the central macular scans can be seen, corresponding to the macular hole. The scan through the macular hole (scan 10) shows an operculum. This operculum represents a small glial plug that helps keep the fovea together; when it is pulled away by the posterior vitreous, a macular hole is facilitated. Histologic studies indicate that the number of photoreceptors attached to the plug can vary, from none to many. The prominent posterior vitreous face can also be seen; its contour suggests that it is likely attached at the optic nerve head and peripheral macula (dome-shaped configuration), which is typical in idiopathic macular holes.
(Courtesy of Colin A. McCannel, MD.)
Stage 1 macular holes have an approximate 50% rate of spontaneous resolution and thus are typically monitored. An alternative management option for stage 1 holes or holes that are less than 250 μm in diameter is the intravitreal injection of ocriplasmin. In the ocriplasmin phase 3 clinical trial, the closure rate of full-thickness macular holes was 40.6% overall and 58.3% for holes of less than 250 μm diameter. Ocriplasmin is most effective in eyes with focal adhesions and much less effective for broad vitreoretinal adhesions or adhesions associated with an ERM.
Duker JS, Kaiser PK, Binder S, et al. The International Vitreomacular Traction Study Group classification of vitreomacular adhesion, traction, and macular hole. Ophthalmology. 2013;120(12):2611–2619.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.