Case of the Month

Edited by Robert N. Johnson, MD

Figures 1A and B: Color fundus photographs of the right (A) and left (B) eyes.  Note the reddish discoloration of the macula in the right eye. The dark red spot temporal to each fovea is an artifact.

Case History

A 53 year-old woman presents with a 3-week history of increasing distortion and blurred central vision in her right eye.  She notes that these symptoms are associated with a dark spot in her vision and eye pain.   She denies any history of recent trauma.  Her past medical history is significant for prediabetes, alopecia, and hormonal disturbances related to menopause.  Her medications include estradiol transdermal patch and progesterone.  Her past ocular history, family history, and social history were noncontributory.

On examination, her best-corrected visual acuity was 20/40 in the right eye and 20/25 in the left eye.  Intraocular pressure (IOP) was normal in both eyes.  The anterior segment examination was unremarkable.  The posterior segment examination of the right eye revealed mild peripapillary atrophy, drusen in the peripapillary distribution, an area of mild RPE atrophy inferior to the fovea, and a red discoloration to the fovea (Figure 1A).  The fundus exam of the left eye showed mild peripapillary atrophy and drusen, mostly in the peripapillary distribution but also in the macula temporal to the fovea (Figure 1B).

 Spectral-domain OCT (SD-OCT) of the right eye (Figure 2A) revealed a full-thickness break in the retinal layers.  SD-OCT of the left eye (Figure 2B) was unremarkable.  Although faint, the hyaloid appears to be attached in both eyes.

Figures 2A and 2B: SD-OCT of the right (A) and left (B) eye. Note in the right eye, there is a small, full-thickness macular hole with flat edges. The left eye is unremarkable.

Differential Diagnosis

Full thickness Macular hole, Lamellar hole, Epiretinal membrane with a pseudohole, Vitreomacular traction syndrome

Additional Case History

The patient was observed.  Within a month, the hole began to close spontaneously (Figure 3) and by three months, the foveal contour had nearly returned to normal with restoration of the ellipsoid zone (Figure 4).

Discussion

A full thickness macular hole is a defect in the continuity of the retinal layers in the fovea.  Macular holes are divided into two categories: primary, which occurs due to abnormal posterior vitreous detachment and was formerly known as idiopathic, and secondary, which results from trauma or macular edema.  Of the two, primary is the more common but secondary holes are more likely to close spontaneously.

OCT has been extremely helpful not only in the diagnosis of macular holes, but also in understanding the mechanism of spontaneous closure.  In a case series of 10 eyes undergoing spontaneous closure, serial OCTs documented the process of glial cell bridging, as evidenced by a “thread” binding the edges of the hole, starting in the outer nuclear layer before full hole closure occurred.¹  The same study found that nearly half of their cases had a PVD with vitreomacular traction, indicating that release of traction may be less significant to hole closure than previously postulated.  OCT analysis can also help predict visual acuity improvement with hole closure, as regeneration of the interface layer between the inner and outer segments of the photoreceptors has been shown to lead to the greatest visual acuity improvement.¹

Several factors, including a shorter duration of existence of the hole and a smaller hole size (usually <250 microns), have been previously reported to indicate a higher probability of hole closure.¹   Michaelewska et al showed that sharp, non smooth edges, also promote spontaneous closure even in larger holes, presumably by providing a scaffold for the bridging process.²

Macular holes can occur in any age, sex, or race but is most commonly found to affect females between 50 and 70 years of age.  Most commonly unilateral, bilateral holes can occur between 10-15% of the time.  Patients most commonly present with blurred vision, visual distortion, a dark spot in the vision or the perception of a bend in straight lines.

Management options for a macular hole include observation, chemical vitreolysis, intraocular gas injection and vitrectomy.  Several studies of ocriplasmin have reported a non-surgical full thickness macular hole closure rate of up to 40% attributable to the treatment.  Intravitreal injection of an expansile gas with facedown positioning has a similar closure rate. Pars plana vitrectomy is the primary surgical intervention for full thickness macular holes and has been found, even in aggregated reviews,³ to be most effective in improving visual acuity and achieving hole closure.  The adjunctive use of internal limiting membrane (ILM) peeling has been shown to increase the likelihood of primary anatomic closure without unwanted side effects, but no difference in final visual acuity was found.³  The traditional teaching of long-acting C3F8 tamponade and face down positioning after repair has also been called into question with recent studies.  A recent retrospective study demonstrated similar closure rates with SF6 and C3F8, irrespective of hole size, stage, or duration, and found that the SF6 group was less likely to develop cataract or ocular hypertension as a result of the procedure.4 Another randomized control trial found nonsupine positioning was noninferior to face down positioning, with equal closure rates and final visual acuity.⁵

References

1. Morawski K, Jedrychowska-Jamborska J, Kubicka-Trzaska A, Romanowska-Dixon B. An analysis of spontaneous closure mechanisms and regeneration of retinal layers of a full-thickness macular hole: relationship with visual acuity improvement. Retina. 2016;0:1-8.
2. Michalewska Z, Cisiecki S, Sikorski B, Michalewski J, Kaluzny JJ, Wojtkowski M, Nawrocki J. Spontaneous closure of stage III and IV idiopathic full-thickness macular holes – a two-case report. Graefs Arch Clin Exp Ophthalmol. 2008;246:99-104.
3. Parravano M, Giansanti F, Eandi CM, Yap YC, Rizzo S, Virgili G. Vitrectomy of idiopathic macular hole. Cochrane Data Syst Rev. 2015;5:1-35.
4. Modi A, Giridhar A, Gopalakrishnan M. Sulfurhexafluoride (SF6) versus perfluoropropane (C3F8) gas as tamponade in macular hole surgery. Retina. 2016;0:1-8.
5. Alberti M and La Cour M. Nonsupine positioning in macular hole surgery: a noninferiority randomized clinical trial. Retina. 2016;0:1-8.