Case #5

The patient

Patient right profile

Unilateral keratoconus with atopic eczema and ptosis

Identity : Mr. H.N
First visit : 02/14/2017
Last Visit : 12/11/2017

28
Age (years)
10
Follow-up (months)

Mr. H.N is a 28-year-old male without any family history of keratoconus. He was operated in 2013 for a ptosis in the right eye (drooping of the upper eyelid). He has an atopic background (atopic eczema).

He complained of a progressive decrease in visual acuity in the right eye which he noticed since 2014. Corneal topography at that time revealed a unilateral keratoconus in the right eye.

His refraction was: RE 20/60 with -6 (-6.5 x 80 °) and LE 20/20 with -7.25 (-1.25 x 115 °).

Clinical examination with the slit lamp revealed bilateral tarsal papillae, a mild irregular corneal bulge in the right cornea, associated with a Fleischer ring and superficial punctate keratitis. The left cornea was essentially unremarkable.

When asked about eye rubbing, he admitted to rubbing his eyes frequently, more often his right eye than his left. He attributed the preferential rubbing of his right eye to the eczema which was more marked in the right eye,  and the ptosis surgery, which caused additional discomfort and irritation.

We explained that chronic right eye rubbing had caused the right cornea to warp permanently, and subsequently advised him to abandon eye rubbing completely. We also advised this patient to have his eczema treated by a dermatologist.

The patients rubs his eyes vigorously and mostly with the tips of his index fingers. He claims to preferentially rub the right eye.
Note the ptosis in the right eye. In both eyes, periorbial dermatitis is obvious with Dennie Morgan infraorbital folds, which are hallmarks of atopic eczema.

Here are the Orbscan examinations (with SCORE Analyzer values) , OPDscan (topography and aberrometry) maps ,  ORA (Ocular Response Analyzer) (biomechanical evaluation) maps and Visante OCT (Optical Coherence Topography) imagery , all recorded at the first visit.

The Orbscan examination of the right eye reveals a particular "bow tie" pattern (bottom left, axial curvature map). This pattern is the consequence of a marked inferior decentration of the apex of the distorted cornea. Some may argue that the pattern suggests a "pellucid marginal degeneration" because of the "claw" -like pattern. However, the thinnest point of the cornea is relatively centered here, which makes keratoconus here more likely.
against the rule astigmatismThe Orbscan map of the left eye demonstrates the presence of a normal corneal pattern. The axial map (bottom left) shows the presence of a corneal astigmatism in the "against the rule" orientation.
SCORE Analyzer. From the Orbscan data, this software based on automated linear discriminant analysis for keratoconus detection computes a value, which is highly positive, confirming the presence of keratoconus.
The SCORE analyzer computed value is negative in this eye, contrasting with the very positive value of the right eye.
The aberrometry measurement allows to explore the optical quality of the eye. In such distorted cornea, high order aberration such as coma and trefoil are elevated. They explain the reduction in optical quality of the right eye, as they cannot be corrected with spectacles.
The aberrometric measurement of the left eye is unremarkable.
The biomechanical examination reveals low applanation peaks, indicating a "locally more deformable" cornea.
The applanation peaks are slightly higher in the left eye.
Cross sectional image of the right cornea. This examination does not contribute much diagnostic value to the right eye.
The cross sectional imaging of the left eye is unremarkable.

 Difference maps were performed at each subsequent visit at our institution. No evolution has been observed between the first and last visits. The keratoconus is stable, more than 10 months after the patient definitively stopped rubbing his eyes .

RIGHT EYE ORBSCAN (2nd VISIT)
LEFT EYE ORBSCAN (2nd VISIT)
The Difference maps with the Orbscan (axial curvature) demonstrates the stability of the deformation (no keratoconus evolution) since the patient stopped rubbing his right eye.
As expected, there is no change in the left eye corneal curvature over time.
RIGHT EYE ORBSCAN (3rd VISIT)
LEFT EYE ORBSCAN (3rd VISIT)
PENTACAM DIFFERENTIAL MAPS : RIGHT EYE.
PENTACAM DIFFERENTIAL MAPS : LEFT EYE.
RIGHT EYE ORBSCAN (4th VISIT)
LEFT EYE ORBSCAN (4th VISIT)
RIGHT EYE PENTACAM (4th VISIT)
LEFT EYE PENTACAM (4th VISIT)
PENTACAM DIFFERENTIAL MAPS : RIGHT EYE. (between 1st and 4th visits)
PENTACAM DIFFERENTIAL MAPS : LEFT EYE. (between 1st and 4th visits
RIGHT EYE ORBSCAN (5th VISIT)
LEFT EYE ORBSCAN (5th VISIT)
RIGHT EYE PENTACAM (5th VISIT)
LEFT EYE PENTACAM (5th VISIT)
PENTACAM DIFFERENTIAL MAPS : RIGHT EYE. (between 2nd and 5th visits)
PENTACAM DIFFERENTIAL MAPS : LEFT EYE. (between 2nd and 5th visits)

Unilateral keratoconus developed in the right eye of this patient with atopic eczema.  Allergy, which affects both eyes, would not alone explain the unilaterality of the keratoconus. The patient admitted to rubbing both eyes, but the right eye more aggressively.  In this patient, the left cornea could uphold the rubbing which was less frequent and vigorous. At the subsequent visit, the patient verbalized that he remembered rubbing his right eye very frequently as a child, but he could not explain why the right eye was itchier.

The unilateral asymmetric character of the keratoconus could also be correlated to the ptosis surgery, responsible for a greater discomfort in the right eye (dysfunctional palpebral dynamics), causing greater irritation and consequently more pronounced eye rubbing on this side. It is also possible that chronic eye rubbing could exacerbate the ptosis (chronic palpebral distension causing aponeurotic ptosis).

The cessation of eye rubbing has resulted in the stabilization of the keratoconus in the right eye.