Granular Corneal Dystrophy
-
Upload
pinchasmd -
Category
Health & Medicine
-
view
7.305 -
download
0
description
Transcript of Granular Corneal Dystrophy
Grand Rounds
Eric K. Chiu, M.D.Department of Ophthalmology and Visual Science
University of Chicago2/2/05
Case Presentation
• 43 y.o. male presenting with blurred vision bilaterally
• Pt states he is tired of glasses and contacts and is interested in corrective surgery
• No pain/redness/flashes/floaters
Clinical Presentation
• PMH: none
• All: NKDA
• Meds: none
• Past ocular history:– myopia
Clinical Presentation
• SLE– See photo
• DFE– wnl
Case Presentation
Clinical Course
• What would you advise the patient?
Differential Dx.
• Granular dystrophy– Stromal dystrophy– Autosomal dominant
• Reis-Bücklers dystrophy– Anterior membrane dystrophy – Affects Bowman’s layer and anterior stroma– Autosomal dominant
• Macular dystrophy– Stromal dystrophy– Dense diffuse stromal opacities– Autosomal recessive
Clinical Course
• Pt underwent LASIK surgery bilaterally
• 9 years later pt presents to U. Chicago with blurred vision RE>LE
Clinical Course
• VASC
– OD 20/400 PH 20/80– OS 20/80 PH 20/50
• IOPGA
– OD 17mmHg– OS 17mmHg
Clinical Course
• PKP recommended
• Risks/benefits/alternatives discussed
Clinical Course
• One year later pt returns to clinic p/w decreased vision RE and LE
• Pt notes increased light sensitivity
• VASC
– OD 20/300 PH 20/200– OS 20/200 PH 20/60
Clinical Course
• Pt decided to undergo PKP in RE
• Last seen 3 weeks post-op
• VASC
– OD 20/300 PH 20/70– OS 20/80 PH 20/50
Clinical Pathology
Clinical Pathology
Clinical Pathology
Clinical Pathology
Clinical Pathology
Clinical Pathology
Clinical Course
• Pt underwent PKP left eye for decreased vision secondary to stromal opacities
Granular Dystrophy
• What is the definition of a corneal dystrophy?
Granular Dystrophy
• Dystrophy– Bilateral– Progressive– Genetic component– Little no relationship to
environmental or systemic factors
Granular Dystrophy• Dystrophy
– Bilateral– Progressive– Genetic component– Little no relationship to environmental or systemic factors
• General– Stromal dystrophy
• Genetics– Autosomal dominant– Chromosome 5q31
• Lattice • Avellino• Reis-Bücklers
– BIGH3 gene • Formation of keratoepithelin
Clinical
• Type I– Most frequent– Early onset in life with crumblike opacities
• Broaden into disciform appearance in teens
– Do not extend to limbus– Can extent anteriorly through breaks in
Bowman’s layer– Slowly progressive
• Vision rarely drops to 20/200 after age 40
Clinical
• Type II– Presents in 2nd decade– Fewer, larger ring/disc-shaped deposits in
anterior stroma– Clear areas– Deposits progressively deeper with age– Erosions infrequent– Vision usually better than 20/70
Clinical
• Type III– Presents in infancy with epithelial erosions– More superficial– Granular deposits confined to Bowman’s layer
or anterior stroma– Resembles Reis-Bücklers but distinct
mutation of BIGH3 gene
Management
• Early in disease process no treatment needed
• Recurrent erosions– Contact lenses– Superficial keratectomy– PTK
• Decreased VA– Good prognosis with PKP
Management
• PKP– Recurrence in graft may occur after many
years as fine subepithelial opacities varying from original presentation
Pathology
• Pathogenesis– Granular material consists of hyaline– Stains bright red with Masson trichrome stain– Electron dense material made up of rod-
shaped bodies immersed in an amorphous matrix
– Noncollagenous protein from corneal epithelium and/or keratocytes
ACGME considerations…check please
• New patient Comprehensive Eye Exam-No referral 48056– Facility charge 152.00– Professional charge 201.00
• External slit lamp photos 48034– Facility charge 75.00– Professional charge 32.00
-------------------------------------------
Collected 460.00 (paid by check)
ACGME considerations…check please
• PKP CPT 65730– Facility charge $19,326.00– Professional charge $
------------------------------------
Collected pending
References
• Ophthalmology. Yanoff. 2nd edition, p 439-440• Akhtar S et al. Deposits and proteoglycan changes in primary
and recurrent granular dystrophy of the cornea. Archives of Ophthalmology. 1999;117:310-321
• Rapuano et al. Recurrence of corneal dystrophy after excimer phototherapeutic keratectomy. Ophthalmology. 1999 Aug; 106(8):1490-7
• Marcon et al. Recurrence of Corneal Stromal Dystrophies after Penetrating Keratoplasty. Cornea. Jan. 2003 22(1):19-21
• Seitz et al. Morphometric analysis of deposits in granular and lattice corneal dystrophy: histopathologic considerations for phototherapeutic keratectomy. Cornea. 2004 May;23(4): 380-385