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Keratoconus: Picture 1
Keratoconus: Picture 1
Author:
Laura L Wayman, MD
Section Editor:
Jonathan Trobe, MD
Deputy Editor:
Jane Givens, MD
Contributor Disclosures
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jun 2020. | This topic last updated: May 12, 2020.
RISK FACTORS
with blurry vision or a sudden decrease in visual acuity. Symptoms progress until the
fourth decade. While vision is initially corrected by spectacles, as the disease
progresses, patients may develop irregular astigmatism and require contact lenses
for refractive correction.
Clinical features which may more specifically suggest the diagnosis include the
following:
As the disease progresses, slit lamp findings become common and include (see "Slit
lamp examination"):
●Fleisher ring – Brown-colored staining around the base of the cone is a subtle
early sign and can be noted even when the cone is minimally elevated. It is
composed of iron deposited within the epithelium by the tear film. The iron flows
with the tear film around the base of the cone. The ring is best seen with the
cobalt blue filter on the slit lamp.
●Vogt striae – Vogt striae are vertical stress lines in the thinnest part of the
cornea at the level of the posterior stroma and Descemet membrane. Unlike
Haab striae and folds secondary to corneal edema, Vogt striae disappear when
gentle pressure is applied to the cornea.
●Central and inferior paracentral corneal thinning – Central and inferior
paracentral corneal thinning, along with mild cone formation and protrusion, is
an early manifestation of keratoconus.
●Corneal scarring – Corneal scarring is the result of spontaneous breaks in
Bowman's layer as the cornea thins (picture 2). It is estimated that 20 percent of
patients with keratoconus will have corneal scarring [16].
The ophthalmologist may also consider other ectatic disorders of the cornea in the
differential diagnosis of keratoconus. These include keratoglobus and pellucid
marginal degeneration [18].
Correction of vision
●Spectacles – In the early stages, the treatment of choice is spectacle
correction. These are used as long as the visual acuity allows the patient to
function.
●Contact lens – As the disease progresses and irregular astigmatism
increases, contact lenses may become necessary to maximize the patient’s
visual acuity. A large portion of patients with keratoconus can be managed
conservatively with contact lenses for an extended period of time [23].
(See "Overview of contact lenses".)
Rigid gas-permeable lenses are the most common contact lens used in
keratoconus. The contact lens smooth out the irregularity of the cornea with its
rigid, regular optical surface. In some patients, particularly if the scarring is
irregular or elevated, rigid gas-permeable contact lens wear can become
intolerable. If significant corneal irregularity due to scarring, surface nodules, or
severe conical shape is present, contact lens correction will likely fail.
●Surgical interventions – Intrastromal corneal ring segments were approved
by the US Food and Drug Administration (FDA) in 2004 for the management of
keratoconus. This technique has also been studied in combination with collagen
cross-linking [24]. These thin, semicircular plastic inserts are implanted into the
mid-corneal layers to flatten the cornea. The goal is to improve the patient’s
visual acuity by reducing the amount of astigmatism. Several authors have
reported flattening of the cornea and significant improvement of refractive errors
[25,26]. However, this treatment is not useful in patients with more advanced
vision loss.
Keratectomy is uncommonly used in the treatment of corneal scars and nodules
to improve contact lens tolerance. However, recurrence is possible and can
stimulate progression [27,28].
●Keratoplasty (corneal transplantation) – Keratoplasty is the procedure of
choice when contact lenses are no longer helpful. Approximately 10 to 15
percent of patients with keratoconus will require keratoplasty; penetrating
keratoplasty (full thickness corneal transplant) is the most commonly used
procedure [29,30]. This procedure has a success rate of greater than 90 percent
in patients with keratoconus [31]. Studies have reported visual acuity of 20/40 or
better in 80 to 90 percent of patients [31-33]. Of those patients, 15 percent
obtain that visual acuity without correction, 40 percent with spectacle correction,
and 26 percent with contact lenses. Graft rejection has been reported in 20 to 35
percent of cases in the first 12 months after surgery. It is associated with loose
sutures, trauma, and large grafts [31]. The majority of cases are treated
successfully with corticosteroids. A more common complication of penetrating
keratoplasty is astigmatism. Treatment options for astigmatism include contact
lens correction, refractive surgery, relaxing incisions, wedge resection, and
compressive sutures.
Deep anterior lamellar keratoplasty (partial thickness corneal transplant) is
another option for keratoplasty that may be used in the treatment of keratoconus
[30,34]. It is used infrequently in the United States.
After an episode of corneal hydrops, the cornea is left with a scar [3,9].
Paradoxically, the severity of the cone may be reduced as the cornea flattens. This
may result in reduced and more manageable astigmatism. Eventually, however,
patients may require keratoplasty because of corneal scarring as a result of hydrops.
PROGNOSIS Keratoconus is a progressive condition. In a 2019 meta-