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Scleral Lens for Management of Ocular Surface Disease Following Penetrating Keratoplasty in a Patient with Stevens-Johnson Syndrome
Bita Asghari, OD, Chantelle Mundy, OD, FAAO
Havener Eye Institute, The Ohio State University Wexner Medical Center Department of Ophthalmology and Visual Science
INTRODUCTION
METHODS and RESULTS
Stevens-Johnson Syndrome (SJS) is a rare self-limiting immunemediated hypersensitivity complex with life-threatening
mucocutaneous reactions. SJS typically develops secondary to an
infection or adverse drug reaction to a medication. Severity of
ocular involvement may vary ranging from keratoconjunctivitis
sicca (KCS) to permanent tissue damage and corneal
blindness.1,4,5
The patient presents with high degree of corneal irregularity including central scarring and thinning (see figure 5).
The cicatricial entropion with trichiasis (see figures 1, 2, and 3) of the upper eyelid is causing mechanical stress to the
ocular surface and triggering continuous blink mediated micro trauma. A diagnostic scleral lens (ScCL) fit is
conducted. The goal of the ScCL is to improve quality of vision, provide ocular surface protection and improve the
patient’s dry eye symptoms.
Treatment of OSD
Final Scleral Lens Prescription OD
Brand
Overall
Diameter
(mm)
Base
Curve
(mm)
Power
(D)
CT
(mm)
Peripheral
Curve radius/
diameter (mm)
Material
Vault
Europa,
Visionary Optics
16.0
6.62
-5.75
0.40
PC1: 6.56 / 2.10
PC2: 9.00/ 0.75
PC3: 13.00/ 0.50
PC4: 14.50/ 0.40
Boston
XO
300
microns
(figure 6)
Background
A 73 y/o Caucasian female with history of SJS presents as a
referral from ophthalmology for a specialty contact lens
evaluation OD. Multiple treatments have been implemented for
management of ocular surface disease (OSD) including: ocular
lubrication, Restasis, doxycycline, acetylcysteine, and surgical
eyelid repair. Such treatments provided no long term success.
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•
•
•
•
•
•
•
•
•
Chief Complaint: longstanding severe dry eye and decreased
vision with no improvement OD
Ocular Hx: OD: KCS, decreased vision, recurrent corneal
ulcer, corneal perforation, persistent epithelial defect,
elevated IOP w/ secondary glaucoma, pseudophakia.
OS: cataract
Ocular Surgical Hx: OD: PK (7/14/2015), aqueous shunt
implant (4/6/2016), complex cataract extraction (4/6/2016),
brow ptosis repair (6/20/2013). OS: unremarkable
Ocular Medication(s): OD: prednisolone QID, ofloxacin QID,
Combigan BID, erythromycin ung QID. OS: none
Personal Medical Hx: h/o SJS
Systemic Medication(s): none
Unaided Visual Acuity (VA): OD: 20/350; PH 20/200
OS: 20/20
Pupils: OD: irregular, minimally reactive; mild APD.
OS: 3mm, 2+ response
IOP (Goldmann): OD: 14mmHg, OS: 16mmHg @ 10:36AM
SLE: OD: upper eyelid entropion w/ trichiasis, corneal graft
w/ 8 intact sutures, 2+ diffuse PEE, central corneal haze and
thinning, symblepharon superior temporal and inferior
temporal, mucous strands, filtering bleb w/ overlying patch
graft, PCIOL. OS: 2+ NSC
Fundus: OD: 0.50 C/D; thin temporally. OS: 0.30 C/D
Assessment
1. KCS, OD
2. s/p PK, OD
3. Corneal scarring, OD
4. Symblepharon, OD
5. Cicatricial entropion with
trichiasis, OD
6. Secondary glaucoma, OD
7. Mild APD OD
8. Cataract, OS
9. History of SJS
Plan
1-5. Continue using
medications as prescribed by
MD. Return to clinic for scleral
lens fitting.
6. Continue using Combigan as
prescribed.
7-8. Monitor annually.
9. No new symptoms
reported. Continue to followup with PCP.
Visual rehabilitation procedures include cataract extraction and in severe cases of visual morbidity,
keratoplasty or keratoprosthesis.2,5
ScCL in Ocular Surface Protection
The ScCL acts as a physical barrier to external mechanical risk factors. The fluid reservoir behind the ScCL
lubricates the ocular surface, provides protection of the fragile epithelium and promotes reepithelialization.7
ScCL in Vision Improvement
Following PK, the average amount of corneal astigmatism is 4 to 5 D.11 Prescription eyeglasses often do not
sufficiently correct for high degrees of corneal irregularity and astigmatism.4,6 Scleral lenses improve visual
acuity for patients with corneal surface irregularities by providing a smooth refractive surface. Scleral lenses
also contribute to a decrease in higher-order aberrations (HOA) in irregular corneas, resulting in improved
quality of vision.2,4,6
Due to severe corneal scarring and perforation, a penetrating
keratoplasty (PK) was performed OD. The patient has secondary
glaucoma from long term use of ophthalmic steroid drops.
Intraocular pressure of the right eye is managed with Combigan
and an aqueous shunt implant.
Case Presentation
Treatment of OSD in SJS aims to stabilize the ocular surface and provide visual rehabilitation. Procedures in
stabilization of the ocular surface include the use of amniotic membranes, lubrication, topical steroids, and
puncta cauterization. In cases of severe scarring of the fornix and conjunctiva, reconstruction surgery may
be indicated.8 Mucous membrane grafting (MMG) may be conducted to repair eyelid keratinization and
prevent blink related micro trauma.1,3,5,7,8,10
Figure 1. Corneal topography (Pentacam) of the right
eye demonstrating high degree of surface irregularity
with central corneal thinning.
Figure 2. Slit lamp photograph (Topcon, Nikon) of the right
eye. Note the corneal graft, irregular pupil, and mechanical
stress of the eyelashes to the ocular surface. HVID=
11.5mm.
CONCLUSIONS
Slit lamp photographs (Topcon, Nikon) show the superior temporal edge of the trial ScCL landing on the
conjunctival graft (figure 3, left) and placement of the ScCL notch (figure 4, right).
Office Visits for Scleral Lens Fitting OD
Chief Complaint
BCVA
Notes
Initial Fitting
Longstanding dry eye and
decreased vision
20/40-2
Lens edge landing on conjunctival graft (see
figure 3)
No change
Follow-Up #1
Patient unable to wear
ScCL due to unsuccessful
insertion and removal
20/50+2
Improvement in vision and
dry eye symptoms OD
20/50+2
Follow-Up #2
20/50+2
It has been demonstrated that scleral lenses are effective in the management of severe OSD.10 A ScCL may be
used as part of first line treatment in prevention of ocular tissue damage, act as a protective barrier and
provide improvement in VA by correcting for refractive error and varying degrees of corneal astigmatism.
Although SJS is self-limiting, damage to the ocular tissue may lead to chronic OSD and ophthalmic
complications. In summary, sclera lenses serve the dual purpose of managing OSD and improving quality of
vision. It may be integrated as part of management of OSD during any phase of the malady.
Office Visit
Initial ScCL
Dispense
OSD in SJS should be identified and treated early to prevent deterioration of the ocular surface and potential
corneal blindness.
Superior temporal ScCL notch in place (see
figure 4)
Improved insertion and removal with the See
Green Lens Inserter from Dalsey Adaptives
(dispensed to patient).
Patient fully adapted to ScCL with an average
wear time of 8-10 hrs/day.
Scleral Lens Fitting
Trial Lens
Lens Diameter
(mm)
BC (mm)
Power
(D)
OverRefraction
Vault
Europa,
Visionary Optics
16.0
6.62
-4.50
-1.25 sphere
350 microns
ACKNOWLEDGEMENTS
Special thank you to Dr. Andrew Hendershot, MD, Dr. Mark Slabaugh, MD and the staff of Havener Eye Institute at
The Ohio State University Wexner Medical Center Department of Ophthalmology and Visual Science.
Slit lamp photographs (Topcon, Nikon) show the central thinning of the corneal graft (figure 5, left) and the
vault of the prescribed ScCL (figure 6, right)..
DISCUSSION
Ocular Manifestations of SJS
Ocular manifestation of SJS can vary from 50% to 81%.8 Ocular sequelae can range from mild KCS (the most
common) to bilateral severe scarring and subsequent corneal blindness.8 Pseudomembranes, persistent
epithelial defects, corneal and/or conjunctival ulceration and perforation may also develop. Symptoms
commonly include significant irritation, ocular discomfort and photophobia .1,3,5,8
One of the most common ocular manifestations is keratinization of the eyelid margin which is causative of
continued blink mediated micro trauma to the ocular surface. Such keratinization adversely impedes surgical
reconstruction procedures and wound healing of the ocular surface; thus, potentiating epithelial keratitis,
persistent epithelial defects, and neovascularization.8
Patients with severe OSD are frequently linked to cataract formation and secondary glaucoma or exacerbation
of preexisting glaucoma subsequent to long term use of topical steroids.1,9
REFERENCES
1. Gregory, Darren G., MD. "New Grading System and Treatment Guidelines for the Acute Ocular Manifestations of Stevens-Johnson Syndrome."
Ophthalmology 123 (2016): 1653-658. Web. 13 Oct. 2016.
2. Keido, Minako, Masakazy Yamada, Chie Sotozono, and Et Al. "The Relationship Between Visual Performance and Clinical Ocular Manifestations
in Stevens-Johnson Syndrome." Am J Ophthalmol 154.3 (2012): 499-511. Web. 13 Oct. 2016.
3. Kolomeyer, Anton M., MD, PhD, Brian K. Do, MD, Yufei Tu, MD, and David S. Chu, MD. "Placement of ProKera in the Management of Ocular
Manifestations of Acute Stevens-Johnson Syndrome in an Outpatient." Eye & Contact Lens 39.3 (2013): E7-E11. Web. 13 Oct. 2016.
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Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis." Am J Ophthalmol 148 (2009): 852-59. Web. 13 Oct. 2016
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8. Iyer, G., Srinivasan, B., Agarwal, S., Kamala, M. S., & Arumugam, S. (March 01, 2014). Comprehensive approach to ocular consequences of
Stevens Johnson Syndrome - the aftermath of a systemic condition. Graefe's Archive for Clinical and Experimental Ophthalmology :
Incorporating German Journal of Ophthalmology, 252, 3, 457-467.
9. Tsai, J. H., Derby, E., Holland, E. J., & Khatana, A. K. (January 01, 2006). Incidence and prevalence of glaucoma in severe ocular surface disease.
Cornea, 25, 5, 530-2.
10. Rosenthal, P., & Croteau, A. (January 01, 2005). 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, 31, 3, 130-4.
11. Touzeau, O., Borderie, V. M., Allouch, C., & Laroche, L. (January 01, 2006). Late changes in refraction, pachymetry, visual acuity, and corneal
topography after penetrating keratoplasty. Cornea, 25, 2, 146-52.