Munson’s sign is a V-shaped indentation of the lower eyelid when a patient with advanced keratoconus looks down.

The cone pushes the lid forward, so the lid margin follows the shape of the cornea.

Therefore, you can suspect advanced disease before you reach the slit lamp.


What Munson’s Sign Shows

Normally the lower lid sweeps in a smooth curve over the cornea on downgaze.

In a steep, protruding cone, the apex presses against the lid and creates an angle.

Because the cone has to be large and steep, the sign appears late in the disease.

Munson’s sign is specific for advanced ectasia but not sensitive for early disease.

How to Check Munson’s Sign

Seat the patient and ask them to look down slowly while you watch from the front and from the side.

Look at the lower lid margin, and note whether it forms a V instead of an even curve.

Also, check each eye separately, because keratoconus is often asymmetric.

Photograph the finding for the record.

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Munson’s Sign and Other External Signs

Several bedside signs accompany advanced keratoconus.

Rizzuti’s sign is a sharp conical light reflex near the nasal limbus when you shine a penlight from the temporal side.

The scissoring reflex on retinoscopy and the oil droplet reflex on distant direct ophthalmoscopy point to irregular astigmatism.

Because these signs depend on a steep cone, their absence says little in early disease, so use topography and tomography.

Management After Munson’s Sign

Advanced disease often needs rigid gas-permeable or scleral lenses, because spectacles cannot correct irregular astigmatism (see scleral lens fitting).

Corneal transplantation comes into play when lenses fail or scarring limits vision (see deep anterior lamellar keratoplasty).

Counsel the patient to avoid eye rubbing, since rubbing is linked to progression.

History Clues Behind the Sign

Ask about progressive blur, frequent spectacle changes, monocular diplopia and glare.

Also, ask about eye rubbing, atopy and family history.

Because Munson’s sign appears late, most patients report years of symptoms.

Examining the Cone

After you see the sign, look at the cornea for apical thinning, a Fleischer ring, Vogt’s striae and scarring.

Then measure keratometry and request tomography.

Also, check the fellow eye, because the disease is bilateral in most patients.

Documenting the Sign

A side-view photograph with the patient looking down records the angulation.

Also, a short video shows the lid movement.

Because the sign reflects the cone, a change over time suggests progression.

Risk of Hydrops

Advanced cones carry a risk of acute corneal hydrops, in which Descemet membrane breaks and the stroma swells (see acute corneal hydrops).

Therefore, tell patients to report sudden pain, redness or whitening of the cornea.

Munson’s Sign: Lid V-Shape in Keratoconus

Pitfalls

Do not rely on the sign for screening, since it has low sensitivity.

Also, do not confuse a V-shaped lid with ptosis or lid retraction, so compare lid position in primary gaze.

Remember that a thick lid fold in downgaze can mimic the angle in some patients.

What the Sign Means for Contact Lens Use

A steep cone makes spectacle correction poor, so many patients rely on lenses by this stage.

Rigid lenses vault the cone, and scleral lenses clear it entirely.

Also, watch for apical touch, which can scar the cone.

When to Consider Surgery

Surgery is considered when contact lenses fail, when scarring limits vision, or after hydrops.

Deep anterior lamellar keratoplasty keeps the host endothelium and reduces rejection risk.

Penetrating keratoplasty remains an option when scars are deep.

Cross-Linking at an Advanced Stage

Cross-linking aims to stop progression and does not restore the shape.

Thin corneas may limit treatment, so measure the thinnest point (see crosslinking criteria).

Also, early treatment gives the best chance to preserve vision.

Patient Education

Explain that the cone is a structural change of the cornea.

Because progression often slows after the third or fourth decade, set realistic expectations.

Then schedule regular reviews.

Munson’s Sign and Visual Symptoms

Patients with this sign usually report glare, ghosting and poor night vision.

Also, they often complain of monocular diplopia that spectacles do not fix.

Because the cornea distorts the image, acuity falls with glasses and rises with a rigid lens.

Therefore, test vision with a pinhole and with an over-refraction through a rigid lens.

Lower Lid Mimics of Munson’s Sign

Other conditions can distort the lower lid on downgaze.

A chalazion or a lid tumor creates a focal bulge, and scars from old trauma can tether the margin.

Therefore, look at the cornea at the slit lamp to confirm that the cone causes the contour.


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References

  1. Rabinowitz YS. Keratoconus. Surv Ophthalmol. 1998;42:297-319.
  2. Gomes JAP, Tan D, Rapuano CJ, et al. Global consensus on keratoconus and ectatic diseases. Cornea. 2015;34:359-369.
  3. Jhanji V, Sharma N, Vajpayee RB. Management of keratoconus: current scenario. Br J Ophthalmol. 2011;95:1044-1050.
  4. Krachmer JH, Feder RS, Belin MW. Keratoconus and related noninflammatory corneal thinning disorders. Surv Ophthalmol. 1984;28:293-322.

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Ahmed Ophthalmologist

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