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 KERATOCONUS

Writer's picture: Munib ur RehmanMunib ur Rehman

Updated: Feb 17, 2024



The most common corneal disorder seen in examination.

 

FACE AND FACIAL SYMMETRY       

The patient may have high-powered spectacles.

 

EYELIDS AND ADNEXA

(Do not forget to Evert the eyelid in case of Keratoconus for signs of VKC)

Munson Sign

Floppy Eyelid Syndrome

 

CONJUNCTIVA

Papillae and their sequelae - VKC

 

CORNEA

Paracentral Stromal Thinning

Vogt's striae are fine, roughly vertical parallel striations in the stroma that disappear on applying pressure to the globe.

Fleischer Ring – hemosiderin ring in the epithelium surrounding the cone – brown in color and best seen with the blue light.

Prominent Corneal Nerves

Rizzuti's Sign - conical reflection on the nasal cornea when a penlight is shone from the temporal side.

Scars from old hydrops – breaks in the Descemet's membrane with overlying scarring of stroma.

ICRS placement

Keratometry

 

SCLERA

Blue Sclerae

 

IRIS AND PUPIL

+/- Aniridia

LENS

+/- Ectopia lentis

VITREOUS

 

POSTERIOR SEGMENT

Retinitis pigmentosa triad

 

 

IOP

Glaucoma –

Why is it challenging to measure IOP in Keratoconus?

 

Ophthalmoscopy

Distant direct ophthalmoscopy – Oil Droplet Sign (always seen with dilated pupil)

 

Retinoscopy

Irregular Scissor Reflex

 

FELLOW

Remember to follow the other eye, which may have some other stage of disease progression.

 

SYSTEMIC CONDITIONS

Down Syndrome

Marfan Syndrome

Atopic Eczema

Ehlers' Danlos syndrome

Osteogenesis imperfecta

 

QUESTIONS:

 

What is the histology feature of Keratoconus?

There is seen Fragmentation of Bowman's layer, thinning of the stroma and overlying epithelium, folds or breaks in Descemet's membrane, and variable amounts of diffuse scarring.

 

How would you treat a patient with Keratoconus?

The main problem is high astigmatism, which tends to be progressive, and treatment consists of glasses initially, then a hard contact lens, and eventually a corneal graft.

Hydrops are usually treated conservatively with antibiotics and eye patches. When edema settles, the scarring may interfere with vision. A corneal graft may be required.

 

What is the differential diagnosis of Keratoconus?

Pellucid marginal degeneration

Keratoglobus

Contact lens-induced corneal warpage

Corneal ectasia post-refractive laser treatment

Keratoconus (KC) is a progressive, asymmetrically bilateral ectatic corneal disease characterized by paraxial stromal thinning and steepening.

 

What is the grading of Keratoconus?

Keratoconus is classified into mild, moderate, and advanced cases.

Mild Keratoconus:

Absent or minimal external and corneal signs

Moderate-to-high myopia with oblique astigmatism on refraction

Irregular astigmatic keratometry values

Corneal inferior steepening, central corneal astigmatic steepening, or bilateral temporal steepening on corneal topography or tomography

Moderate Keratoconus:

  • Presence of one or more corneal signs of Keratoconus (e.g., enhanced appearance of corneal nerves, Vogt striae, Fleischer ring, corneal scarring)

  • Superficial corneal scarring (fibular, nebular, or nodular)

  • Deep stromal scarring

  • Scarring at the level of the Descemet membrane resembling posterior polymorphous corneal dystrophy

  • Paraxial stromal thinning

  • Keratometry values of 45-52 diopters (D)

  • "Scissoring" or the oil drop sign

  • Munson Sign

Advanced Keratoconus

  • Keratometry values greater than 52 D

  • Enhancement of all corneal signs, symptoms, and visual loss/distortion

  • Vogt striae; Fleischer ring and scarring

  • Acute corneal hydrops

 

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