I.Clinical features

A.Synonyms.

1.Full-thickness macular hole.

2.Impending macular hole.

3.Lamellar or partial-thickness macular hole.

4.Foveal hole.

B.Ocular features.

1.Signs and symptoms.

a.Idiopathic full-thickness macular (foveal) holes occur more commonly in women (70%), generally in sixth to seventh decade of life (perhaps due to survival advantage).

b.Visual acuity ranges from 20/40 to 20/200; patients note blurred vision and central scotoma.

           c.Fully developd  holes are generally 500 microns in diameter; epiretinal membrane may be associated.

d.Vitrectomy surgery with gas tamponade shown to benefit vision.

e.Initial features.

(1)Stages of macular hole development described extensively.

f.Chronic features.

(1)Variable.

II.Basics

A.Pathogenesis.

1.Stages of macular hole development

a.Stage 1—yellow spot or halo, vitreous attached.

b.Stage 2—small early full-thickness hole (500 mm), vitreous attached.

c.Stage 3—full-thickness hole, vitreofoveal separation.

d.Stage 4—full-thickness hole, vitreous separation.

2.Reclassification of macular hole (Gass 1995).

3.  Further reclassification based on OCT findings (Gaudric 1999)

a. First stage of macular hole formation: intraretinal split in the inner part of the foveola, which evolves into a cystic formation.

b. Once this formation has extended posteriorly, disrupting the photoreceptor layer, and vitreous traction has resulted in the opening of the roof of the cyst, a full-thickness macular hole occurs.

c.Change from stage 1-A to 1-B caused by centrifugal displacement of retinal receptors following dehiscence at umbo.

d.Stage 1-B may become manifest stage 2 hole.

(1)Either after early separation of the contracted prefoveolar vitreous cortex, or

(2)As eccentric can opener-like tear in contracted prefoveolar vitreous cortex at edge of larger holes.

             e. If separation of the prefoveolar vitreous cortex occurs, the retina will return to place and the macular contour return to normal with restoration of vision 

B.Risk factors.

1.Medical disorders.

a.Pathologic myopia, vitelliform dystrophy.

2.Physical factors.

a.Trauma.

b.Lightning.

3.Iatrogenic factors.

a.After cataract surgery.

b.After intense retinal laser or cryopexy.

4.Genetic factors—nonfamilial.

C.Epidemiology

1.Prevalence—unknown.

a.When 1 eye involved, risk to fellow eye ranges between 3% and 30% (average ~ 5%), depending on the prefoveolar vitreous cortex attachment.

2.Occurs in all races.

3.Inheritance—nonfamilial.

4.Seen more commonly in women (70%) (perhaps due to survival advantage).

5.Onset occurs in sixth to seventh decade of life, although traumatic holes generally occur at earlier age and tend to be larger.

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III.Diagnosis

A.Imaging.

1.Optical coherence tomography.

a.Imaging technique of choice- usually reveals in great detail exact stage of macular hole formation and risk of macular hole development in fellow eye.( figure)

B.Pathologic findings.

1.Light microscopy.

a.Cortical vitreous contains retinal pigment epithelial cells and glial cells.

C.Clinical course/prognosis.

1.Natural history varies; in many holes in an early stage, prefoveolar vitreous cortex will detach and macula returns to its normal anatomic state

2. Early stage holes may not.progress

2.Indications for surgery depend on individual patient; generally, surgery performed only when visual acuity less than 20/60.

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IV.Management/treatment

A.General health care.

1.No limitations or precautions.

B.Medical therapy.

1.No medical treatment.

C.Surgical interventions.

1.For impending macular holes, vitrectomy shown to be no better than natural history; approximately 40% of impending holes progress to full-thickness holes with or without surgery.

2.For full-thickness macular holes, approximately 80% to 90% can be closed with vitrectomy surgery and gas tamponade.

3. Role of internal limiting membrane peeling on success of macular hole surgery  controversial, mainly due to traumatic effect of retinal manipulation and the toxic effects of dye used to identify the internal limiting membrane.

3.Postoperative positioning crucial during early post operative period.

4.With hole closure, vision generally improves by 2 to 4 Snellen lines.

       D. Precautions.

1.Patients must be selected for surgery carefully.

2.If patient unable to maintain face-down positioning for gas tamponade during first 2 postoperative weeks, silicone oil must be considered for tamponade, otherwise operation will usually fail.

E.Monitoring.

1.Complications.

a.Rhegmatogenous retinal detachment due to peripheral retinal tear (7% to 15%), peripheral retinal tears (3% to 6%), peripheral visual field loss (16%), transient elevated intraocular pressure (52%), enlargement of macular hole (2%), late reopening of hole (2% to 33%), photic (microscope) toxicity (1%), and endophthalmitis (1%)

2.Patient monitoring.

a.Patient should monitor central vision on daily basis with use of Amsler grid and report substantial changes promptly.

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