Stage 0 macular holes: observations by optical coherence tomography

Abstract

Objective: To introduce the concept of a stage 0 macular hole based on optical coherence tomographic observations of the vitreoretinal interface in fellow eyes of patients with unilateral idiopathic macular holes, and to evaluate the subsequent risk of progression to a full-thickness macular hole.

Design: Retrospective observational case series.

Participants: Ninety-four patients with a unilateral stage 2, 3, or 4 full-thickness macular hole.

Methods: The medical records of patients with a unilateral macular hole diagnosed between 1994 and 2000 at the New England Eye Center were reviewed.

Main outcome measure: Development of a full-thickness macular hole in the fellow eye on biomicroscopic fundoscopy or optical coherence tomography (OCT).

Results: In 27 (28.7%) of 94 clinically normal fellow eyes, OCT detected an abnormality of the vitreoretinal interface but normal foveal anatomy. The vitreoretinal abnormalities were further subclassified into severe (4 eyes), moderate (8 eyes), and mild (15 eyes) based on the intensity and morphology of the OCT signal. One of the 4 (25%) severe cases progressed to a full-thickness macular hole, 4 of the 8 (50%) moderate cases became full-thickness macular holes, and no (0%) mild cases progressed to a full-thickness macular hole. Severe and moderate eyes seemed to share characteristic features on OCT that increased their risk of macular hole development (stage 0 macular hole). The macular hole-free survival at 48 months was 94% for stage 0-negative patients, versus 54% for stage 0-positive patients. Univariate analysis revealed that the presence of a stage 0 macular hole was significantly associated with an almost 6-fold increase in the risk of macular hole formation (relative risk: 5.8, 95% confidence interval: 1.16-28.61, P = 0.03).

Conclusions: A stage 0 macular hole has a normal biomicroscopic appearance clinically, but has salient features on OCT as a result of oblique vitreous traction. Optical coherence tomographic findings consist of a normal foveal contour and normal retinal thickness and must include the presence of a preretinal, minimally reflective, thin band inserting obliquely on at least one side of the fovea. The presence of a stage 0 macular hole in the fellow eye is a significant risk factor for the development of a second macular hole.

Figure 1

Optical coherence tomography of fellow eyes with abnormalities at the vitreoretinal interface, classified according to the morphology and severity of the signal from the visible posterior hyaloid. Top, Severe case: prominent insertion of posterior hyaloid on both sides (superiorly and inferiorly) of the perifoveal region. Middle, Moderate case: prominent insertion of posterior hyaloid on only one side (nasal) of the perifoveal region. There is no distinct point of insertion on the other side (temporal). Bottom, Mild case: a preretinal signal corresponding to the posterior hyaloid is visible inferiorly, but there is no clear point of insertion. No posterior vitreous detachment was found on clinical examination.

Figure 2

Kaplan–Meier survival curve. A comparison of the proportion free from a macular hole in biomicroscopically normal fellow eyes between patients with a stage 0 macular hole and those without.

Figure 3

Evolution of a stage 0 macular hole to a stage 2 macular hole and subsequent surgical repair (case report). Top, Normal color photograph. Optical coherence tomography (OCT) showing normal foveal contour and thickness, a partially separated posterior hyaloid with persistent adherence at the fovea. Middle, Six months later, a color photograph reveals an eccentric full-thickness defect. Optical coherence tomography shows a new disruption in the inner layer of the retina, constituting a stage 2 macular hole. Bottom, Two months after macular hole surgery, a color photograph reveals resolution of the macular hole. The postoperative OCT image demonstrates reapproximation of the edges of the macular hole.