BINDURA UNIVERSITY OF SCIENCE EDUCATION

P. BAG 1020 Tel. (263) 712 842 712-4

Bindura, 066 210 6505

Zimbabwe

FACULTY OF SCIENCE AND ENGINEERING

OPTOMETRY DEPARTMENT

NAME : RUSERE SUCCESS KUDZAI

REG NUMBER : B213298B

LEVEL : 2.1(CONVENTIONAL) YEAR: 2022

PROGRAMME : BACHELOR OF SCIENCE HONOURS DEGREE IN OPTOMETRY

MODULE CODE : OPTC 202

MODULE TITLE : OCULAR PATHOLOGY 1

LECTURER : DR. MASHOKO

DUE DATE : 06 DECEMBER 2022

QSN. Discuss the causes of keratoconus its evaluation and its

management (25)
Keratoconus is a non-inflammatory, bilateral (but usually asymmetrical) disease of the cornea
which results in progressive corneal steepening. It is characterized by para-central corneal
thinning and ectasia so that the cornea takes the shape of a cone. Visual loss occurs primarily
from myopia and irregular astigmatism and secondarily from corneal scarring. The cause of
keratoconus is not known. It may not be a single disorder, but rather a phenotypic expression of
perhaps many causes, both genetic and environmental.

A patient with protruding conical corneas and associated poor vision may have keratoconus.
Keratoconus is associated with round or nipple cones with a central conical protrusion, and oval
cones, often with inferior sagging. All layers of the cornea are believed to be affected by
keratoconus, although the most notable features are the thinning of the corneal stroma, the
fragmentation of the Bowman layer and the deposition of iron in the basal epithelial cells,
forming the Fleischer ring. Folds and breaks in the Descemet’s membrane result in acute hydrops
and striae, which produces variable amount of diffuse scarring.

Keratoconus can be categorized into clinically recognizable stages as:

Latent stage: Latent stage was recognizable by Placido disc only.
Early stage: Early stages were subdivided into two categories as:
 Keratoconus fruste, which entailed 1–4-degree horizontal axis deviation of Placido disc.
 Early or mild keratoconus, which entailed 5- to 8-degree deviation of horizontal axis.

The prevalence of keratoconus in general population appears to be relatively high and is likely to
be higher when examined with corneal topography. Corneal topography is a computer assisted
diagnostic tool that creates a three- dimensional map of the surface of the cornea. Keratoconus
occurs in all ethnic groups with no male or female preponderance. It is commonly an isolated
ocular condition, but sometimes coexists with other ocular and systemic diseases.

Commonly recognized ocular associations include vernal keratoconjunctivitis, retinitis

pigmentosa and Leber congenital amaurosis; many of the connective tissue disorders (e.g.
Ehlers-Danlos and Marfan syndromes), mitral valve prolapse, atopic dermatitis and Down
syndrome. Particular risk factors include atopic history, especially ocular allergies, rigid contact
lens wear and vigorous eye rubbing. Most keratoconus cases appear spontaneously, but few may
show evidence of genetic transmission.

Keratoconus generally manifests at puberty, and is progressive until third to fourth decade of life
when it usually arrests. Sometimes, however, it may commence later in life and progress or arrest
at any age.

Symptoms are highly variable and depend upon the stage of the disease. Early in the disease,
there may be no symptoms. In advanced disease, there is significant diminition and distortion of
vision. The symptoms include:

Monocular polyopia (perception of multiple ‘ghost’ images in the eye), decreased visual acuity,
and decreased contrast sensitivity may be seen in other disorders, especially early nuclear
sclerotic cataract. Blurring of vision. Decreased visual acuity. Progressively poor vision, not
corrected with eye glasses. Impaired visual perception. Decreased contrast sensitivity. Streaking
and flaring distortion around light sources. Marked anisometropia (difference in vision of two
eyes). Glare, photophobia (increased sensitivity to light). Eyestrain, in order to read clearly. Poor
vision at night. Itching.

Keratoconus may be associated with wide variety of systemic and ocular conditions. Systemic
associations include: Atopy (a genetic predisposition to develop an allergic reaction). One of the
most common associations of keratoconus is atopy and is seen in condition like vernal
keratoconjunctivitis. Eye rubbing seen in systemic atopy may play a role in the development of
keratoconus. Down syndrome (Trisomy 21) and In down syndrome the frequency of acute
hydrops is higher, perhaps because of eye rubbing and/or these patients are treated infrequently
with keratoplasty and their disease is allowed to progress further. Ehlers-Danlos syndrome,
Marfan syndrome, Crouzon syndrome and Apert syndrome. Ocular associations are Leber’s
congenital amaurosis and retinitis pigmentosa. Retinopathy of prematurity. Fuchs’ corneal
endothelial dystrophy and posterior polymorphous dystrophy.

There are also contributing factors of keratoconus. Enzyme abnormalities in corneal epithelium,
enzyme abnormalities such as increased expression of lysosomal enzymes (catalase and
cathepsin) and decreased levels of inhibitors of proteolytic enzymes (tissue inhibitor matrix
metalloproteinases), may play a role in corneal stromal degradation. Molecular defect, producing
unusual absence of water channel protein aquaporin in keratoconus as compared to normal
corneal epithelium. Gelatinolytic activity in stroma has been described, which may be due to
decreased function of enzyme inhibitors. Abnormalities in corneal collagen and its cross-linking
may be the cause of keratoconus. Eye rubbing, cytokine has been suggested as a mediator of eye
rubbing and stromal degradation. Keratoconus in patients with atopic dermatitis, down
syndrome, and Leber congenital amaurosis, a congenital photoreceptor degeneration, could be
related to forceful eye rubbing. The latter is a behavioral pattern seen in visually and mentally
handicapped children who repeatedly strike their eyes with hands and finally hard contact lens
wear.

Refraction, keratometry, corneal topography and slit- lamp (bio-microscopy) examination helps
in reaching to the diagnosis of keratoconus. Keratoconus is differentiated according to severity of
disease as well as shape of cornea.Medical therapy: Spectacle correction initially, attempt should
be made to correct myopic astigmatism by prescribing spectacle correction. Early keratoconus
patients may get satisfactory vision with spectacles. Development of irregular astigmatism often
limits the use of spectacle correction, and patients may require contact lenses.

Surgical therapy: Surgical therapy may be resorted to when patient does not improve with the
use of contact lenses. Contact lenses may fail due to: Inadequate acuity, inadequate lens
tolerance, frequent lens displacements, peripheral thinning. In surgical procedures, a standard
surgical treatment consists of keratoplasty. Recurrence of keratoconus after keratoplasty is rare.
Recurrence may be due to incomplete excision of the cone at the time of surgery. Unrecognized
keratoconus in the corneal donor. Host cellular activity that causes changes in the donor corneal
material. Lamellar keratoplasty: Lamellar keratoplasty is effective, but this is not preferred
because of the technical difficulties in the procedure and slightly reduced visual outcome.
Epikeratoplasty has been successful as well, but it has been abandoned due to suboptimal visual
outcome.

Penetrating keratoplasty: By far the most frequent procedure is penetrating keratoplasty. At the
time of keratoplasty, decreasing the donor/ recipient size disparity reduces post-keratoplasty
myopia. Complications of penetrating keratoplasty include corneal graft rejection, glaucoma,
cataract formation, anisometropia, astigmatism and infection. Deep anterior lamellar keratoplasty
(DALK): Deep anterior lamellar keratoplasty (DALK) has been proposed as an alternative to
penetrating keratoplasty.

Intra-corneal ring segments: Intra-corneal ring segments have achieved some success in patients
without corneal scarring in reducing the myopia and astigmatism and improving spectacle-
corrected visual acuity. These are intrastromal corneal ring segments, placed in the peripheral
cornea, either mechanically or with the femtosecond laser.
 REFERENCE

 Galloway N.R., Amoaku W.M.K., Galloway P.H. and A.C. Common Eye Diseases
and their Management. Third Ed. Springer-Verlag London Ltd, 2006

 BrowningDuane’s Clinical Ophthalmology. New York: Lippincott Williams &

Wilkins, 2005.

 Khurana A K: Comprehensive Ophthalmology. Fourth Edition. New Age

International (P) Ltd., Publishers, 2007.