Keratoconus bcsc

• Keratoconus (KC), a common disorder, occurs in approximately 1 in 2000

individuals.

• However, the incidence rate varies based on geographic location and the
testing methodology. Typically, the central, inferior paracentral, or
inferior midperipheral cornea undergoes
• progressive thinning and protrusion, resulting in a cone- shaped cornea
(Figs 9-1, 9-2).
• There is a slight female preponderance, and a higher incidence has been
reported in South Asian
• and the Middle Eastern countries.
• Although a hereditary pattern is not commonly observed, positive family
histories have been reported in 6%–8% of cases.
• Considerable variations can occur in clinical findings within an affected
family.
• The prevalence of KC in first- degree relatives is 3.34%.
• Clinically unaffected first- degree relatives have a higher chance of
showing subclinical tomographic abnormalities associated with KC than
the general population.
• Although multiple chromosomal loci for KC have been reported, the
identification of specific genes remains elusive.
• The development of sophisticated topographic (power mapping) and
tomographic (elevation and thickness mapping) devices has
dramatically improved the detection of corneal ectasia, particularly KC.
• Factors that may play a role in the onset and progression of KC include •
• genetic predisposition
• • eye rubbing
• • atopic disease and allergic keratoconjunctivitis
• • contact lens wear
• • abnormalities in collagen that result in
hyperelastic joints
• • keratorefractive surgery in patients with
preoperative high myopia or thin corneas
• • oxidative stress
• CLINICAL PRE SEN TA TION
• Although KC is typically bilateral, asymmetry is
commonly observed.
• Apical thinning with protrusion or anterior bowing of
the inferior paracentral or midperipheral cornea
worsens with progression, as does the degree of
irregular astigmatism.
• Generally, keratitis or corneal neovascularization is not
observed; however, they may occur due to contact
lens wear.
• Common signs of KC include
• • scissoring of the light reflex on retinoscopy:an early but nonspecific sign of KC; commonly
associated with irregular astigmatism
• • Munson sign:a late- stage nonspecific sign involving inferior deviation of the lower eyelid
contour on downgaze (Fig 9-3)
• • Rizzuti sign:focusing of the light within the nasal limbus when a penlight is shone from the
temporal side; an early but nonspecific sign
• • partial or complete Fleischer ring:a circle formed due to iron deposition within the basal
epithelium at the base of the cone, which becomes narrower and increasingly well- defined with
disease progression .
• It is best seen with the slit lamp using a broad, oblique beam or diffuse illumination with the
cobalt blue filter.
• • Vogt striae:fine, parallel lines observed in the posterior stroma at the apex of the cone, which
may dis appear with the application of external pressure (Fig 9-5) • apical scarring:types
commonly seen include ■ reticular scarring related to breaks in Bowman layer (Fig 9-6) ■
• nummular scarring at cone apex related to contact lens wear■
• deep stromal scarring related to prior corneal hydrops
• Topographic findings, obtained through Placido- based imaging
(reflection of concentric rings), slit- scanning, or Scheimpflug-
based devices (see Chapter 2), include
• • increased overall steepening (>47.20 diopters [D])
• • inferior steepening, especially when coincident with abnormal
elevation and/or abnormal inferior thinning (Fig 9-8)
• • superior flattening (see Fig 9-8)
• • increase in inferior- superior (I- S) ratio (I/S >1.2 is considered
significant)
• • skewing of the radial axes (SRAX) >21° is considered suggestive
of KC, while an orthogonal bow tie is typical of regular astigmatism
(Fig 9-9)
• Tomographic findings, obtained with slit- scanning devices, Scheimpflug imaging, or anterior segment optical
coherence tomography (OCT; see Chapter 2), include
• • isolated islands of elevation anteriorly and/or posteriorly: the elevation is relative to
• a computer- generated best fit sphere placed behind the anterior or posterior surface
• • asymptomatic posterior elevation: an early sign of subclinical KC
• • abnormal thinning (<500 µm): indicates ectasia, especially if coincident with islands of elevation
• • I- S difference of >30 µm in thickness at the 5 mm ring around the fixation
• • difference of >30 µm between the 2 eyes in the thinnest pachymetric reading
• • decentration of the thinnest part of the cornea on a pachymetry map typically, the thinnest cornea is
centrally located
• • enhanced ectasia risk, detected by comparing the difference between standard anterior and posterior
elevation maps and maps in which the best-fit sphere is determined, excluding a 4-mm area around the
thinnest cornea
• • change in corneal thickness, evaluated from the area of minimum pachymetry to the limbus (occurs more
dramatically in eyes with KC compared with eyes without)
• Anterior segment OCT is used to map corneal epithelial thickness for early diagnosis and follow-up of KC.
• The normal corneal epithelium is thicker centrally and thinnen superiorly superiorly, while the keratoconic
cornea is thinner at the apex of the cone, typically inferotemporally, and thicker superonasally.
 MANAGEMENT
• Some cases of mild KC can be successfully managed
with glasses.
• It is impor tant to counsel patients regarding the risk of
disease progression with continued eye rubbing, which
in turn, can be reduced with optimal management of
atopic diseases.
• As mentioned, KC progresses rapidly in young patients
(in their teens and twenties), and thus,
• they should be examined more frequently.
• The disease is more likely to be stable in patients
• over age 40, but progression can still occur. Contact lenses can
mask the associated irregular corneal astigmatism (Fig 9-11),
and most affected patients report a dramatic improvement in
vision with use.
• As the disease progresses, scarring and contour distortion can
reduce the contact lens– corrected vision and make it more
difficult to achieve a stable fit.
• Contact lens wear does not prevent KC progression. Although
most patients will require rigid gas- permeable (RGP) lenses,
some patients, particularly those with mild disease, may
achieve improved vision and comfortable wear with soft lenses.
• Contact lens options include
• • soft toric lens
• • RGP hard lens
• • bicurved hard lens
• • hybrid lens (hard center and soft skirt)
• • piggyback system (hard lens on top of a soft
lens)
• • mini- scleral lens with high oxygen permeabilit
• Surgical treatment for apical subepithelial scarring, a common
cause of contact lens intolerance, includes mechanical
debridement and phototherapeutic keratectomy.
• Treatment can improve patient comfort, but scarring may recur.
Intrastromal corneal ring segments can be implanted to center
the cone and facilitate successful contact lens wear.
• The procedure may not prevent disease progression, however,
and is not intended to reduce dependence on glasses or
contact lenses.
• Vision correction through glasses or contact lenses may allow
the patient to postpone or eliminate the need for keratoplasty
• Corneal crosslinking is used in patients who demonstrate
progressive disease or are at a significant risk of progression,
particularly adolescents.
• Older patients may benefit as well if they show signs of
progression.
• Corneal crosslinking is most effective in mild to moderate
• cases and may not work as well in cases of post- LASIK ectasia or
patients with advanced KC.
• It may be performed in combination with intrastromal ring
insertion.
• For a more detailed discussion of intrastromal ring segments and
corneal crosslinking, see the Appendices at the end of this chapter.
• Keratoplastybecomes an impor tant option under the following
circumstances:
• • poor vision even with a comfortable stable fitting contact lens
(usually due to scarring)
• • contact lens intolerance despite the good vision achieved with
the lens
• • unstable contact lens fit (even with good vision and lens
tolerance)
• • progressive thinning toward the corneal periphery approaching
the limbus, which requires a very large graft (associated with
increased risk of rejection)
• • corneal hydrops that fails to clear after several months
• Penetrating keratoplasty (PK) is currently the preferred cornea transplant procedure
• for the treatment of KC in the United States and is associated with an excellent prognosis.

• Alternatively, many surgeons prefer deep anterior lamellar keratoplasty (DALK) for KC.
• This is particularly true in places where high- quality donor tissue is not readily available.
• Although technically more challenging, there are several advantages of DALK over PK.
• At 6 months post– DALK, the endothelial cell counts are significantly higher compared
• with PK.
• Although endothelial rejection does not occur with DALK, stromal rejection is still pos si
ble. Stromal scarring can result in reduced vision and symptoms of glare.
• In the event of trauma, postoperative wound integrity is better with DALK than with PK
• Corneal hydrops is treated conservatively with topical
hypertonic agents and/or a soft contact lens for several months.
• A cycloplegic agent and topical steroid may provide short- term
relief by reducing inflammation and pain.
• Aqueous suppressants may decrease the flow of fluid into the
cornea.
• Intracameral injection of air or a nonexpansile concentration of
gas (SF6or C3F8) may help accelerate the resolution of hydrops.
• Pupil dilation and/or inferior peripheral iridectomy may reduce
the risk of pupillary block after intracameral gas injection.
• Hydrops is not an indication for emergent keratoplasty.