Keratometry
Keratometry is beneficial in the diagnosis and monitoring of keratoconus. However, the limitations of keratometry, including the measurement of only a few paracentral points on the cornea, can make early diagnosis difficult, especially if the patient has a decentered apex of the cone.
What is often observed in early keratoconus is a lack of parallelism of the keratometric mires. The mires are somewhat distorted, and one may not completely overlap the other. The corneal astigmatism may increase and shift toward an oblique axis. A steepening of the corneal curvature may be observed. If the condition is incipient, the keratometry readings may be in the 43 to 48 D range. Once the condition advances beyond the curvature values of the keratometer, a +1.25 D ophthalmic lens can be mounted over the objective (patient end) to add approximately 8 D to the keratometer reading (Appendix 1).
Videokeratography
The use of videokeratography (VKG) has been an outstanding tool for the diagnosis and monitoring of patients with keratoconus. The location of the apex and the progression of the condition can be observed via evaluation of the color map. The localized area of steepening can easily be observed with a color map (
Fig. 18.1).
Although definitive keratoconus cannot be diagnosed in the absence of several slit-lamp or retinoscopic findings, a keratoconus suspect can be determined quite easily via the appearance of the color map. These individuals can then be monitored to determine if further progression and diagnosis of the condition occurs. In addition, a compression of the mires will be present in the affected region via the reflection of the photokeratoscopic rings. Also, as the unaffected corneal region (typically superior) will not change, an inferior-superior dioptric asymmetry will be present.
1
The impacted region or “cone” has traditionally been categorized as “nipple,” oval (i.e., sagging), or globus.
49,
50,
51 The nipple cone is smaller and more centralized than the other two types. The oval cone is more inferior, whereas the globus cone is quite large in diameter. McMahon
52 reported on results from the CLEK study in which the impacted areas were divided into nipple (
Fig. 18.2A), oval (
Fig. 18.2B), globoid (
Fig. 18.2C), and marginal (
Fig. 18.1), with the latter group pertaining to a nonround or nonoval cone located in the periphery of the cornea (often inferior).
As shown in
Table 18.1, most of the cones can be described as nipple or oval. It was also reported that 12.2% have an apex above horizontal with an average location at 262 degrees (inferior-temporal).
53
Several VKG systems have developed applications for the screening and diagnosis of keratoconus.
48,
54,
55,
56,
57 This is particularly important, as the presence of keratoconus is a contraindication
for refractive surgery and it has been found that as many as 5% to 7% of refractive surgery candidates have subclinical keratoconus.
58,
59
In research clinics, keratoconus has been defined using topographic indices only. VKG readings and differences in corneal shape have been used to diagnose keratoconus. Mandell et al.,
4,
60 with the cone apex aligned with the optical system of a VKG, determined that a true apex power reading can be obtained and therefore compared to the normal range in the detection of keratoconus. It was concluded that if the cone apex power is 48 to 49 D, the patient should be considered a keratoconus suspect. For powers of 49 to 50 D, there is a very high likelihood of keratoconus, and for powers above 50 D, the diagnosis is almost certain. The modified Rabinowitz-McDonnell method
1,
54,
61 uses the following guidelines: if the central corneal power is >47.2 D or if the difference between the inferior and superior paracentral corneal regions (i.e., I-S value) is >1.4 D, then the cornea is considered keratoconus suspect. If the central corneal power is >48.7 D or the I-S value is >1.4 D, then the cornea is classified as keratoconus. Rabinowitz and Rasheed have also developed an index, the KISA%, to grade the presence or absence of keratoconus.
62 Although this index has the potential to define disease severity, the developers have only described its role in defining normals, keratoconus suspects, and those with the disease. The KISA index has been used to monitor changes in normal eyes of unilateral keratoconus patients
63 and genetic screening where KISA was used to distinguish keratoconus from normal individuals.
38
The difficulty in using topographic-only methods to define keratoconus is that early forme fruste conditions—which may never progress—can be labeled as keratoconus with unnecessary worry imposed on the patient. Although topographic evidence of keratoconus should be a strong reason to avoid ablative refractive surgery, otherwise asymptomatic patients may not need any special therapeutic treatment other than monitoring. For that reason, a definitive diagnosis of keratoconus is usually made clinically by requiring either a slit-lamp sign of the disease (as discussed below), refractive and visual acuity changes suggestive of keratoconus, or distortion of the anterior cornea as measured by the red reflex.