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Prosthetic Replacement in
Steven-Johnson Syndrome
Varsha Rathi
Somasheila I Murthy
L V Prasad Eye Institute, Hyderabad, India
The authors have no financial interest to disclose
Introduction
•
Stevens Johnson disease (SJS) is assumed to be an
autoimmune response to an environmental factor such
as a drug or virus
• Conjunctival involvement and corneal involvement with
dry eye and reduced visual acuity
• Purpose: To study the role of scleral contact lens
(Prosthetic replacement of the ocular surface ecosystem
[PROSE]) in Stevens-Johnson Syndrome (SJS)
Scleral Contact lens
• Prosthetic replacement of the ocular
surface ecosystem PROSE, Boston
Foundation for Sight, Needham Heights
USA
How PROSE works!
• The fluid reservoir conserves moisture
Reduces pain
• Reduces photophobia
• Prevents rubbing of tarsus on cornea
• Aids in epithelization
• Masks irregular astigmatism
1.
2.
Romero-Rangel T, Stavrou P, Cotter J, Rosenthal P, Baltatzis S, Foster CS. Gas-permeable scleral contact lens
therapy in ocular surface disease. Am J Ophthalmol 2000;130:25-32.
Rosenthal P, Croteau A. Fluid-ventilated, gas-permeable scleral contact lens is an effective option for managing
severe ocular surface disease and many corneal disorders that would otherwise require penetrating
keratoplasty. Eye Contact Lens 2005;31:130-134.
Material and Methods
Patients having SJS who were dispensed with scleral contact
lens (PROSE, prosthetic replacement of the ocular surface
ecosystem, Boston Foundation for Sight, MA, USA) were
analyzed retrospectively
• Goal of Scleral lens fitting was to improve comfort and vision
• SJS was diagnosed by the presence of history of allergic
drug reaction
• All patients underwent comprehensive eye examination
including Slit lamp biomicroscopy
• Visual acuity was noted with and without PROSE lens wear in
eye
Statistical analysis
• Microsoft excel Windows version 7 was used
• Visual acuity was converted to LogMAR for statistical
analysis
• Paired t test was used to find significance p <0.05 was
termed statistically significant
Results
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Mean age, 28.86 years
n= 38 were <16 years
Gender: 116 M, 91 F
Mean follow up = 18.9 months
(3 sessions to 11 sessions) were
required to teach lens insertion
and removal
•
12 lenses were dispensed within •
6 weeks of having SJS
•
Diameter -<18 mm -69 lenses
Remaining were > 18 mm lenses
•
– most being spherical lenses
Average daily lens wear = 9h
Average training sessions = 3.89
•
Visual acuity
Average Uncorrected visual acuity
- 20/100
Average best corrected visual
acuity without PROSE– 20/80
Average Prose wear visual acuity
– 20/50
The p value was statistically
significant (p<0.05)
All these patients had photophobia and difficulty in day light with reduced visual acuity
and the symptoms reduced and visual acuity improved significantly
SJS in acute phase
PROSE was dispensed within 6 weeks of presentation of SJS
Complications
• Microbial keratitis – 1 eye – healed with
scarring and medical treatment
• Infiltrative keratitis – 2 eyes of one patient (
changed to new lens)
• Second order of lens was required in 26
eyes – either for lens breakage or changed
fitting over time ( third order was required
for 3 eyes – broken lens, changed fitting)
• Lens breakage - 15 patients
• Increased photophobia with PROSE wear =
2 eyes ( discontinued lens wear)
• Deposits on lens surface 4 lenses ( 2
lenses grew fungus)
• Lens intolerance 4 eyes ( underwent
Mucus membrane graft)
Deep vessels – 2 eyes of one
patient – whether it is due to
SJS per say or overuse?
The deep vessels regressed with
discontinuation of PROSE for a
while and then rewear with a
high Dk material lens
SJS
• Front surface drying
This required replacing the fluid daily in
between to improve vision
Debris collection in the
fluid
Challenges
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Shortened fornix
Symblepharon
Mucus discharge
Pediatric patients
Small apertures
Presence of
tarsorrhaphy
Small diameter lenses
• Creates more suction
• Close to limbus –
More focal scleral
compression –
blanching of
conjunctiva
Discussion
• All these patients had photophobia and difficulty in day
light with reduced visual acuity – symptoms reduced and
visual acuity improved significantly
• Lens breakage was noted. The newer lenses were
ordered only if lens broke or deposits developed over
lens over follow up.
• The mean follow up was 18.9 months and patient
continued to use the same lens over follow-up. This
indicates that though the cost of the lens is high, use of
the same lens over follow-up is cost effective provided
the condition of PROSE is good
Conclusion
• South east Asia – first series
• Statistically significant improvement in visual acuity was
noted with PROSE wear
• Using a small inventory of lenses, it is possible to fit most
of the patients with less reorder rate.
• Cost effective