BASICS
DESCRIPTION
Partial or full-thickness retinal defect in the central macula.
EPIDEMIOLOGY
Incidence
• 7.8–8.7 cases/100,000 persons/year
• Peak incidence in seventh decade of life
Prevalence
• 0.02–0.8%
• Women affected more than men
• Typically affects patients over age 55 years
RISK FACTORS
Recent trauma
PATHOPHYSIOLOGY
Vitreoretinal traction contributes to macular hole formation.
ETIOLOGY
• Most macular holes are idiopathic
• Trauma may also cause a macular hole
DIAGNOSIS
HISTORY
• Patients notice blurry central vision, metamorphopsia, or a central scotoma in one eye.
• Acute or subacute onset
– Symptoms often first noted when fellow eye is incidentally covered.
• History of trauma may be elicited.
PHYSICAL EXAM
• Visual acuity ranges from 20/25 to 20/400 depending on the size and stage of hole.
– Average acuity is 20/200 for a full-thickness macular hole.
• Diagnosis primarily based on biomicroscopic examination
– Hand-held indirect lens or fundus contact lens
– Typically dark, round defect noted in central fovea
– Small neurosensory detachment may be present as a fluid “cuff” surrounding the hole.
– Yellow–white dots may be present at the level of retinal pigment epithelium.
– Pseudo-operculum may be visible overlying hole
• Biomicroscopic (Gass) classification (1)[C]
– Stage 1: Impending hole
Central yellow spot (Stage 1-A) or yellow ring (Stage 1-B) with loss of foveolar depression
– Stage 2: Small (<400 micron) full-thickness defect visible in the fovea
– Stage 3: Large (>400 micron) full-thickness defect
– Stage 4: Full-thickness defect with posterior vitreous detachment present
• Watzke-Allen test utilized to differentiate full-thickness from lamellar hole and other lesions
– Macular lens and slit lamp utilized to focus thin beam through central macula. Patients with macular hole will note a break or “compression” in the slit beam.
DIAGNOSTIC TESTS & INTERPRETATION
Lab
Laboratory testing not indicated
Imaging
• Optical coherence tomography (OCT)
– Shows full thickness defect involving the macula.
– May show vitreoretinal adhesion
– A pseudo-operculum may be visible
Diagnostic Procedures/Other
• Laser aiming beam test
– 50-micron laser aiming beam focused in center of lesion. Patients with full-thickness macular hole will be unable to see the spot, whereas patients with other lesions typically are able to see the spot.
Pathological Findings
Surgical specimens are typically not obtained.
DIFFERENTIAL DIAGNOSIS
• Epiretinal membrane with pseudohole
• Cystoid macular edema
• Macular cyst
• Lamellar macular hole
• Pigment epithelial detachment
• Central serous retinopathy
• Vitreomacular traction
TREATMENT
MEDICATION
No medications indicated
ADDITIONAL TREATMENT
Issues for Referral
Referral to vitreoretinal surgeon indicated.
COMPLEMENTARY & ALTERNATIVE THERAPIES
None indicated.
SURGERY/OTHER PROCEDURES
• Surgery is the only successful treatment option.
• Pars plana vitrectomy with gas tamponade (2)[C]
– Internal limiting membrane peeling in many cases (3)[B]
• Non-emergent or urgent, but surgery typically performed within 1–2 months of diagnosis
• Extensive postoperative face-down positioning, lasting several days to weeks, is usually required.
• Subsequent cataract extraction may be required in phakic patients.
IN-PATIENT CONSIDERATIONS
Initial Stabilization
Outpatient management indicated.
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
• Referral to a vitreoretinal surgeon is indicated.
• Routine postoperative examinations on postoperative day 1, 1 week, 2–3 weeks, and 6–12 weeks after surgery.
PATIENT EDUCATION
• Fellow eye develops a macular hole in 5–20%
– Educate patient to monitor symptoms in fellow eye
• Educate patient on signs and symptoms of retinal tear and detachment postoperatively and need for urgent evaluation if symptoms develop.
PROGNOSIS
• Hole closure rates >90% with surgery (3)[B], (4)[C]
• Postoperative visual acuity variable but can be good with successful hole closure
COMPLICATIONS
• Surgical complications
– Cataract (common)
– Retinal tear or detachment (uncommon)
• Macular hole associated retinal detachment (rare)
REFERENCES
1. Gass JD. Reappraisal of biomicroscopic classification of stages of development of a macular hole. Am J Ophthalmol 1995;119:752–759.
2. Kelly NE, Wendel RT. Vitreous surgery for idiopathic macular holes. Results of a pilot study. Arch Ophthalmol 1991;109:654–659.
3. Christensen UC, Kroyer K, Sander B, et al. Value of internal limiting membrane peeling in surgery for idiopathic macular hole stage 2 and 3: A randomised clinical trial. Br J Ophthalmol 2009;93:1005–1015.
4. Kumagai K, Furukawa M, Ogino N, et al. Long-term outcomes of internal limiting membrane peeling with and without indocyanine green in macular hole surgery. Retina 2006;26:613–617.
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