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Diagnosis: Macular hole.
Differential Diagnosis:

1. Macular pucker (epiretinal membrane) with a pseudohole.
2. Cystoid macular edema.
3. Lamellar macular hole.
4. Age-related macular degeneration.
5. Central serous retinopathy.
6. Solar retinopathy.

Discussion:

I. Definition: Full-thickness defect (from the internal limiting membrane to the outer segment of the photoreceptor layer) that arises in the macula.
II. Demographics: Peak incidence is in the 7th decade of life; more common in women (3x more likely); occurs in <1% of the population; 10% bilateral.
III. Symptoms: Vision loss is variable depending upon size, location, and stage of defect. Metamorphopsia and/or a central scotoma may be present. The onset of symptoms is typically gradual. It may be asymptomatic and discovered incidentally or only noticed on covering the fellow eye.
IV. Staging:-Stage 1: An impending hole (premacular hole); a yellow spot (stage 1A) or yellow ring (stage 1B) is seen in the center of the fovea.

-Stage 2: Full-thickness hole less than 400 μm in diameter.
-Stage 3: Full-thickness hole larger than 400 μm in diameter and may have an associated cuff of subretinal fluid.
-Stage 4: Same as stage 3 except that a total posterior vitreous detachment is also present.
-Additional features: An operculum (retinal tissue attached to vitreous) may overlie the hole; yellow-white deposits may be present on the RPE at the base of the hole. V: Etiology: 80% are idiopathic; <10% associated with trauma to the eye.

-Theories:

1) Trauma: Force of trauma leads to immediate rupture of the macular retina.
2) Vitreous: A perifoveal vitreous detachment places tractional force on the retina; this leads to a full-thickness foveolar dehiscence; currently the leading theory.

-Macular holes are not associated with systemic disease.


VI. Diagnosis:

- Complete ocular exam: Will identify typical exam findings stated above.
- Watzke-Allen test: Differentiates true macular hole from pseudohole by directing thin, vertical slit beam across the area in question; patient will report break in the line if a true macular hole is present; the line may be distorted with pseudohole, but no break will be present.
- FA: Early foveal hyperfluorescence without evidence of late phase leakage is seen is stage 2-4 macular holes.
- OCT: Helps to determine stage, which will guide treatment; monitors progression of hole; determines degree of traction from epiretinal membranes.

VII. Treatment:

- Stage 1: 50% resolve spontaneously; remaining 50% progress to full-thickness holes; observation recommended.
- Stage 3-4: Surgery (pars plana vitrectomy with epiretinal membrane removal) considered in most eyes with moderate to large stage 3 or 4 holes that cause decreased VA (20/60 to 20/400); preoperative VA is inversely correlated with visual improvement; eyes with worse preoperative acuity gain the greatest improvement.
- Stage 2-3: Surgery may be indicated for eyes with small stage 2 or 3 macular holes causing decreased VA in the range of 20/40 to 20/60; preoperative VA is directly correlated with postoperative VA; eyes with better preoperative acuity end up with better postoperative acuity.
- Best to operate within 6 months of symptom onset; 80-90% rate of hole closure with surgery; holes with the shortest duration and < 500 μm in diameter have the best visual improvement; complications: retinal detachment, cataract, and visual field defects.

References:

Ehlers JP, Shah CP, Fenton GL, et al. The Wills Eye Manual.5th edition. Lippincott Williams & Wilkins, 2008. p. 315-6.

Ryan, SJ. Retina. 4th edition. Volume 3. Elsevier Mosby, 2006. p. 2527-41.

Quillen DA & Blodi BA. Clinical Retina. 2003. p. 104-5.

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