Morning report: january 28, 2015
Avni shah, pgy-2 preceptor: Dr. Erlanger
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H&P
• CC: blurry vision• HPI: 64 y/o AAM with DM2, h/o refractive error
presents to VA eye clinic with blurry vision in both eyes. Feels vision has gradually deteriorated in both eyes. No irritation, redness, or pain.
• PMH: HTN, DM2, no known history of retinopathy• POH: refractive error• Meds: HCTZ, lisinopril, metformin, glyburide• ROS: negative for joint pains, rash, and GI
complaints, no recent URI or sinus diseaseco
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H&P• VAcc: R 20/70+1 L 20/70+1• MRx:
R -1.75 +2.00 x 085 20/30-L -1.00 +1.25 x 110 20/40-
Add: +2.25 OU• Pupils: 3 mm > 2 mm OU, no APD• IOP: R 18 L 17• EOM full R and L.• VF full to confrontation R and L.
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Slit lamp exam
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topography
44.62D @9042.50D @180
44.37D @12043.37D @30
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Differential Diagnosis• Terrien’s Marginal Degeneration• Inflammatory PUK (RA, GPA/Wegener’s,
IBD, PAN, SLE, Sarcoid, Behçet’s)• Mooren’s Ulcer• Other infectious keratitis (Strep, Gonococcus,
Syphillis, TB, Moraxella, Haemophilus, HSV, VZV, fungal)
• Staph Marginal Keratitis• Senile Furrow Degeneration• Contact Lens use• Dellen
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Further workup
• RF – wnl• ANA – wnl• ANCA – negative• RPR – nonreactive• ACE – wnl
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Terrien’s Marginal Degeneration• First described by Terrien in 1900• Idiopathic, clinically noninflammatory, painless• Slowly progressive peripheral corneal stromal thinning• Typically unilateral or asymmetrically bilateral• Onset as early as second or third decade of life• Can be localized or involve extensive portions of
peripheral cornea• Typically begins superiorly and progresses
circumferentially, rarely involves inferior limbus or central cornea
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TERRIEN’s marginal degeneration:Clinical features
• Thinned edge with steep central wall and gradually sloping peripheral wall
• No epithelial defect• Fine vascular pannus
traverses thinned areas with lipid deposition at the leading edge of the pannus
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TERRIEN’S MARGINAL DEGENERATION:
PATHOLOGY• In vivo confocal microscopy of affected
corneas have shown distinct ultrastructuralchanges that support a subtle, subclinical mild inflammatory state
Decreased nerve fibers in sub-basal nerve plexus, dendritic cells
Irregularly organized Bowman’s
Activated keratocytes, honeycombing
Nonhomogenoushyperreflective material deposition
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TERRIen’s MARGINAL DEGENERATION: presentation
• Typical presentation - our patient• Other possible presentations:• Fuchs superficial marginal keratitis –
inflam variant– +/- pseudopterygium
• Spontaneous corneal perforation
• Spontaneous filtering bleb• Acute hydrops
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Differential diagnosis
Inflammatory PUK Systemic association: RA, GPA/Wegener’s, IBD, PAN, SLE, Sarcoid, Behcet’susually unilateral, de-epithelializedadjacent conjunctiva inflamedflares correlate with disease activity
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Differential diagnosis
Mooren’s Ulcer painful, progressive, idiopathicautoimmune, possible hx parasite, possible association with HCVleading edge de-epithelializedunilateral (older population), bilateral/rapid (West African males)
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Differential diagnosis
Staph Marginal Keratitis marginal infiltrates with peripheral clear zonechronic disease – stromal opacities, peripheral thinning, pannusfollowing resolutions of acute infiltrates
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Differential diagnosis
Senile Furrow Degenerationin lucid space peripheral to arcusThinning apparent more than realno inflammation or vascularizationvision not affected
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Management of TMD
• Surface lubrication• Correction of refractive error caused by
astigmatism with glasses or RGP lenses• Corneal collagen cross-linking – shown to
reverse thinning at 5 yr follow-up• Crescent-shaped lamellar or full-thickness
corneoscleral patch grafts • Annular lamellar grafts for 360° thinning
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Cataract surgery• Care must be taken during surgical planning to note
areas of peripheral corneal thinning• If thinning involves superior/temporal limbus, suture
placement or scleral wound are surgical options
• Our patient: s/p CE/IOL left eye 5 years ago with 10-0 nylon placed through inferotemporal paracentesis, stable post-op MRx and topography
• Now POD#2 s/p CE/IOL right eye via normal approach (main wound 10:00) as superior and temporal limbusunaffected
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Take home points
• Consider TMD in patients with painless peripheral corneal thinning, even if the superior limbus is spared
• TMD can present atypically as pseudopterygium, spontaneous corneal perforation, spontaneous filtering bleb, and acute hydrops
• If eye appears painful or inflamed, consider other causes of PUK including systemic inflammatory disease, Mooren’s ulcer, infectious keratitis, and Staph marginal disease
• Be careful to note areas of peripheral corneal thinning when planning cataract surgery
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References
• Ceresara G et al. In vivo confocal microscopy in Terrien marginal degeneration: a case report. Cornea. 2011 Jul;30(7):820-4.
• Dalton, M. Corneal diagnoses and systemic disease: PUK and systemic autoimmune disease. EyeWorld. April 2012. http://eyeworld.org/article-puk-and-systemic-autoimmune-disease
• External Disease and Cornea, Section 8. Basic and Clinical Science Course, American Academy of Ophthalmology, 2011-2012. 339-340
• Hafezi F et al. Corneal collagen cross-linking for Terrien marginal degeneration. J Refract Surg. 2014 Jul;30(7):498-500.
• Kursiah, MR. Iatrogenic corneal perforation in Terrien Marginal Degeneration. Med J Malaysia. 2013 Apr;68(2):173-4.
• Munro M et al. Two cases of spontaneous filtering blebs, one idiopathic and one associated with Terrien marginal degeneration. Cornea. 2014 Jul;33(7):752-4.
• Soong HK et al. Corneal hydrops in Terrien’s marginal degeneration. Ophthalmology. 1986 Mar;93(3):340-3.
• Yang R and Guo R. The Treatment of Terrien marginal degeneration using lamellar keratoplasty with dried corneosclera. Yan Ke Xue Bao. 2004 Sep;20(3):140-3.
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