Grand Rounds
Amir R. Hajrasouliha, M.D.
University of Louisville
Department of Ophthalmology and Visual Sciences
Friday, June 20th, 2014
Case Presentation
CC: Gradual blurred vision OU
HPI: 26 y/o otherwise healthy caucasian male
present with progressive decrease of vision OU
(OD>OS) in past year. The patient reported that
he had bilateral laser vision correction (LASIK) 4
years ago.
Case presentation: History

POHx:



LASIK 4 years ago
PMHx:


None
Eye med:


None
Meds:

None
FH:

Mother with unknown corneal dystrophy
20/40-2 -1.00 sphere
VA
20/20-1 sc
T
12
P
11
wnl
wnl
EOM and CVF Full
Right
Left
External
WNL
WNL
Lids
WNL
WNL
Conjunctiva
White and quiet
White and quiet
Cornea
s/p LASIK with multiple round/disc-shaped
intrastromal corneal opacities with
intervening clear stroma sparing the
peripheral corneal
s/p LASIK with few intrastromal
corneal opacities
Anterior Chamber
Deep and quiet
Deep and quiet
Iris
WNL
WNL
Lens
WNL
WNL
DFE
WNL
WNL
OD
OS
OCT OD
OCT OD
OCT OD
OCT OD
OCT OD
OCT OS
OCT OS
Assessment & Plan
26 y/o male with corneal deposits at interface post
LASIK OU ; OD>>OS
• Granular corneal dystrophy OU
Management

Observation
Laser refractive surgery (LRS) is contraindicated in
• Unstable refractive error
• Keratoconus
• Herpetic keratitis
• Significant cataract
• Uncontrolled glaucoma
• Uncotrolled diabetes
• Uncontrolled collagen vascular disease
• Pregnancy
• Pt on amiodarone and isotretinoin

LASIK is contraindicated in
Epithelial basement membrane dystrophy
(EBMD)
 Fuchs


PRK is treatment of choice for EMBD and
its safety in Fuchs is not established
Granular corneal dystrophy type I


Inheritance: Autosomal dominant
Pathology:
Hyaline material, stain bright red with Masson
trichrome stain.
 Hyper-reflective opacities are seen on
confocal microscopy.


Mutation: TGFBI gene on chromosome
5q31
Clinical finding
Onset early in life around teens
 Lesions do not extend to limbus but can
extend anterioly through focal breaks in
Bowman layer.
 Slowly progressive with vision only rarely
dropping to 20/200 after age 40.
 Recurrent erosions occur and vision
decreases as the opacities become more
confluent.

Treatment




Early in disease, no treatment is needed.
Recurrent erosions may be treated with contact
lens, superficial keratectomy, or PTK
When visual acuity is affected, DALK or PK has
a good prognosis.
Recurrence in the graft (anterior and
peripherally) may occur after many years as fine
sub-epithelial opacities varying from the original
presentation.
PTK treatment for GCD1
Clinical & Experimental Ophthalmology, 34, no. 8 (2006): 808-810

In 2002 and 2003 there were some reports from
Japan that demonstrated corneal electrolysis for
granular lesions at the interface
after LASIK surgery, resulted in
clearing of the cornea ; however,
the technique did not become
popular as the recurrence rate
was high.
Deposits of Transforming Growth Factor-[beta]-Induced Protein in Granular Corneal Dystrophy Type II After LASIK.
Kim, Tae-im; Roh, Mi; Grossniklaus, Hans; E MD, MBA; Kang, Shin; Jeong MD, PhD; Hamilton, Stephen; Schorderet, Daniel; Lee, W; Kim, Eung; Kweon MD, PhD
Cornea. 27(1):28-32, January 2008.
DOI: 10.1097/ICO.0b013e318156d36d
© 2008 Lippincott Williams & Wilkins, Inc. Published by Lippincott Williams & Wilkins, Inc.
2
Secondary wound healing process induce
TGF-β secretion which stimulates mutated
TGFβI protein product synthesis (keratoepithelin or TGFβIp). The misfolded protein
then accumulates as the insoluble protein
aggregates and results in clinical defect.
Munier FL, et al. Kerato-epithelin mutations in four 5q31-linked corneal dystrophies.
Nature Genetics. 1997; 15: 247-251.
Thank You