Synonyms: macular cyst, retinal hole, retinal perforation
It runs from the internal limiting membrane to the outer segment of the photoreceptor layer. It involves the fovea, so affects central visual acuity (VA).
Prevalence is 3.3 per 1,000 >55 years in the USA. Studies in India and Denmark have shown prevalences of 0.14-0.17%. In the UK incidence is approximately 1 per 10,000 per year.
Women are more commonly affected than men. Peak incidence is in the age group of 70-80 years.
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Usually idiopathic (<10% have a history of trauma). The most widely accepted theory suggests that age-related focal shrinkage of the prefoveolar vitreous cortex causes traction on the foveal area, leading to foveal detachment and subsequent macular hole formation.
Other risk factors include cystoid macular oedema, retinal detachment, laser injury, hypertension, very high myopia and diabetic retinopathy.
It may rarely be an incidental finding. Symptoms appear gradually over days/weeks:
- Distorted vision as well as visual loss.
- Visual acuity (VA) will depend on the site of the hole, ie small eccentric holes may have little effect on acuity.
- Holes that are not full thickness also have less effect on acuity.
- Look for a tiny well-defined 'punched out' area of the macula which can be hard to detect. There may be yellow-white deposits at the base with a grey margin around it representing oedema.
- Slit lamp examination will show 'a round excavation with well-defined borders' interrupting the beam of the slit lamp.
- Most patients also have a semi-translucent tissue over the hole, which may be surrounded by a grey halo caused by detachment of the retina.
|Stage 1a||Seen as a yellow spot. This is not specific for macular hole - can be associated with central serous chorioretinopathy, cystoid macular oedema, and solar maculopathy.|
|Stage 1b||Occult hole: doughnut-shaped yellow ring (approximately 200-300 μm) centred on the foveola. Approximately 50% of holes progress to stage 2.|
|Stage 2||Full thickness macular hole (<400 μm). Prefoveolar cortex usually separates eccentrically creating a semi-transparent opacity, often larger than the hole, and the yellow ring disappears. These generally progress to stage 3.|
|Stage 3||Holes >400 μm associated with partial vitreomacular separation.|
|Stage 4||Complete vitreous separation from the entire macula and optic disc.|
Diagnosis is usually made clinically; however, the following may be useful:
- Ocular coherence tomography (OCT) provides high-resolution cross-sectional imaging of the retina and is useful in predicting prognosis.
- Fluorescein angiography (FA), although not usually necessary, may be useful in differentiating macular holes from cystoid macular oedema and choroidal neovascularisation (CNV).
- It typically shows a window defect early in the angiogram that does not expand with time, and there is no leakage or accumulation of dye.
- There may be Amsler grid abnormalities. However, plotting small central scotomas is often difficult.
Check local protocol (units are usually happy to have a chat on the phone and take things from there).
Ultimately, the patient will need to be seen by a vitreoretinal surgeon. Not all units have one so, if your local unit is small, it is worth mentioning to the patient that they may well be sent to the nearest bigger unit for further assessment and treatment. This has the dual effect of helping the patient feel that they are going through a normal process of referral, rather than feeling that they are being passed from pillar to post. It also prepares them for the fact that they have a bit of a long day ahead - important especially for diabetic or elderly patients - and so helps preparations. That is also where surgery will be carried out.
- About 50% of stage 1 holes resolve spontaneously, but almost all stage 2 (and above) progress without surgery.
- The chosen surgery depends on the staging of the hole. Surgical closure of the hole is considered up until stage 3 or 4 associated with a visual acuity (VA) of 6/18 or worse. If the macular hole has been present for 1-3 years, then surgery is likely to work. If it has been present for 5 years or longer, then results may be more variable.
- Vitrectomy may relieve traction on the edge of the hole. The vitreous ± internal limiting membrane are removed and a long-acting gas bubble is introduced to tamponade the macula back into position. Short periods of postoperative prone posturing are being used in some centres in selected patients to aid surgical procedures associated with intraocular gas tamponade to achieve macular hole closure and there is increasing evidence to support its use. The procedure has been shown to be safe and effective.
- Vitrectomy (with fluid-gas exchange for stage 2, 3, and 4 holes) improves vision compared with conservative treatment. Series of patients have been variously reported with hole closure rates of 73-95%. Most patients' VA improves by two lines of the Snellen chart.
- Success is also possible if the hole is long-standing (6 months-2 years) or if the patient is aged >80 years.
- Occasionally more than one operation is required to close the hole.
- Standard vitrectomy with internal limiting membrane (ILM) maculorrhexis (peeling) has been performed in patients with stage 3 or 4 idiopathic macular holes. The retina is massaged to approximate the edges of the hole as closely as possible. This technique gives a good anatomical and functional result.
- For those patients who have a condition that precludes surgery, visual rehabilitation may be the only option with, for example, referral to low visual aids clinics if need be. These patients will not have ongoing ophthalmological review but are followed in the community by an optician.
- Zinc and beta-carotene replacement have been advocated (Ocuvite®). These are unproven and contra-indicated with actively bleeding ulcers.
Many patients develop cataracts. 76% of cases require extraction within two years of macular surgery.
Other problems include:
- Retinal detachment.
- Iatrogenic retinal tears.
- Macular retinal pigment epithelium changes.
- Enlargement of the hole.
- Macular light toxicity.
- Postoperative intraocular pressure spikes.
- Stage 1 holes spontaneously resolve in 50% of cases.
- Stage 2 holes almost always progress without surgery. With surgery, early stage 2 holes show anatomical closure in >90% of cases and a two or more line improvement on the Snellen chart in 80% of cases. NB: repair and visual acuity (VA) not necessarily correlated.
- 10% stay the same and 10% lose some VA.
There is a 12% chance (this may increase to 20% if other risk factors are present) of a similar hole developing in the other eye.
Further reading & references
- Oh KT et al, Macular Hole, Medscape, Jul 2011
- la Cour M, Friis J; Macular holes: classification, epidemiology, natural history and treatment. Acta Ophthalmol Scand. 2002 Dec;80(6):579-87.
- Sen P, Bhargava A, Vijaya L, et al; Prevalence of idiopathic macular hole in adult rural and urban south Indian population. Clin Experiment Ophthalmol. 2008 Apr;36(3):257-60.
- Ho AC, Guyer DR, Fine SL; Macular hole. Surv Ophthalmol. 1998 Mar-Apr;42(5):393-416.
- Gass JD; Idiopathic senile macular hole: its early stages and pathogenesis. 1988. Retina. 2003 Dec;23(6 Suppl):629-39.
- Ruiz-Moreno JM, Staicu C, Pinero DP, et al; Optical coherence tomography predictive factors for macular hole surgery outcome. Br J Ophthalmol. 2008 May;92(5):640-4.
- Malik A, Dooley I, Mahmood U; Single night postoperative prone posturing in idiopathic macular hole surgery. Eur J Ophthalmol. 2011 Aug 1:0. doi: 10.5301/ejo.5000039.
- Lange CA, Membrey L, Ahmad N, et al; Pilot randomised controlled trial of face-down positioning following macular hole Eye (Lond). 2011 Sep 23. doi: 10.1038/eye.2011.221.
- Haritoglou C, Reiniger IW, Schaumberger M, et al; Five-year follow-up of macular hole surgery with peeling of the internal limiting membrane: update of a prospective study. Retina. 2006 Jul-Aug;26(6):618-22.
- Chew EY, Sperduto RD, Hiller R, et al; Clinical course of macular holes: the Eye Disease Case-Control Study. Arch Ophthalmol. 1999 Feb;117(2):242-6.
- Freeman WR, Azen SP, Kim JW, et al; Vitrectomy for the treatment of full-thickness stage 3 or 4 macular holes. Results of a multicentered randomized clinical trial. The Vitrectomy for Treatment of Macular Hole Study Group. Arch Ophthalmol. 1997 Jan;115(1):11-21.
- Kim JW, Freeman WR, Azen SP, et al; Prospective randomized trial of vitrectomy or observation for stage 2 macular holes. Vitrectomy for Macular Hole Study Group. Am J Ophthalmol. 1996 Jun;121(6):605-14.
- Scott RA, Ezra E, West JF, et al; Visual and anatomical results of surgery for long standing macular holes. Br J Ophthalmol. 2000 Feb;84(2):150-3.
- Thompson JT, Sjaarda RN; Results of macular hole surgery in patients over 80 years of age. Retina. 2000;20(5):433-8.
- Alpatov S, Shchuko A, Malyshev V; A new method of treating macular holes. Eur J Ophthalmol. 2007 Mar-Apr;17(2):246-52.
|Original Author: Dr Hayley Willacy||Current Version: Dr Gurvinder Rull||Peer Reviewer: Dr Helen Huins|
|Last Checked: 17/11/2011||Document ID: 2412 Version: 22||© EMIS|
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