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

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Collected 460.00 (paid by check)

ACGME considerations…check please

• PKP CPT 65730– Facility charge $19,326.00– Professional charge $

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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