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![Page 1: Mozhgan Rezaei Kanavi, MD. Ophthalmic Research Center Shahid Beheshti University of Medical Sciences, Tehran, Iran Appearance of Bilateral Corneal Opacities.](https://reader038.fdocuments.in/reader038/viewer/2022110207/56649d195503460f949ee366/html5/thumbnails/1.jpg)
Mozhgan Rezaei Kanavi, MD.Ophthalmic Research Center
Shahid Beheshti University of Medical Sciences, Tehran, Iran
Appearance of Bilateral Corneal Opacities in a 61-year-old man
BAOP 2011
![Page 2: Mozhgan Rezaei Kanavi, MD. Ophthalmic Research Center Shahid Beheshti University of Medical Sciences, Tehran, Iran Appearance of Bilateral Corneal Opacities.](https://reader038.fdocuments.in/reader038/viewer/2022110207/56649d195503460f949ee366/html5/thumbnails/2.jpg)
Case Report
• A 61-year-old male• Transportation driver • Vague past medical history of non-
symptomatic dysproteinemia • Recent decrease in visual acuity
BAOP 2011
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Slit Lamp Biomicrosco
pyBAOP 2011
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Case Report
• VA: OD: 20/160 OS: 20/200• No evidence of conjunctivitis or uveitis• IOP: WNL• Funduscopy: Unremarkable
BAOP 2011
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Corneal Confocal Scan
BAOP 2011
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Corneal Confocal Scan
BAOP 2011
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• Clinical diagnosis of a “Crystalline Keratopathy” • Systemic work-up• Diagnostic Anterior Lamellar Keratoplasty OS
… Case Report
BAOP 2011
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• Anterior corneal lamella fixed in absolute alcohol weas sent to our ophthalmic pathology laboratory
• Gross examination: Two folded translucent pieces of tissue measuring 7x4x1mm and 5x3x1mm; which both were bisected and processed for histopathologic examination
… Case Report
BAOP 2011
![Page 9: Mozhgan Rezaei Kanavi, MD. Ophthalmic Research Center Shahid Beheshti University of Medical Sciences, Tehran, Iran Appearance of Bilateral Corneal Opacities.](https://reader038.fdocuments.in/reader038/viewer/2022110207/56649d195503460f949ee366/html5/thumbnails/9.jpg)
Results
BAOP 2011
![Page 10: Mozhgan Rezaei Kanavi, MD. Ophthalmic Research Center Shahid Beheshti University of Medical Sciences, Tehran, Iran Appearance of Bilateral Corneal Opacities.](https://reader038.fdocuments.in/reader038/viewer/2022110207/56649d195503460f949ee366/html5/thumbnails/10.jpg)
Results
BAOP 2011
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Results
PASBAOP 2011
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Results
BAOP 2011
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Results
Alcian blue
Congo Red
BAOP 2011
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Results
Kappa chainBAOP 2011
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Results
Lambda chainBAOP 2011
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Histopathological
Diagnosis
Paraproteinemic Crystalline Keratopathy
BAOP 2011
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Systemic Work-up
• IgG Monoclonal Gammopathy• Free kappa chains in urine • Lymphocytosis with 5% plasma cells and
a micro and macroblastic reaction on bone marrow biopsy
• Negative whole body scan• Refer to Haematologist• Chemotherapy was not decided
BAOP 2011
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• Corneal deposits recurred at the deep layers of the donor cornea
• Repeat confocal scan confirmed recurrence of the crystalline deposits in the graft
• Refer to haematologist for chemotherapy
Four Months after Keratoplasty
BAOP 2011
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Can be classified from the clinical and genetical point of view into
3 groups: 1) Primary hereditary (Schnyder)
2) Secondary hereditary (Cystinosis)
3) Secondary non-hereditary in association with disorders of serum protein or lipid composition
Non Infectious Crystalline Keratopathy
BAOP 2011
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• In association with disorders of serum protein composition
• In cases above 50 years of age • Bilateral corneal opacities may be the first
clinical evidence of systemic disease
Paraproteinemic Crystalline Keratopathy
BAOP 2011
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Mechanism of Corneal Crystallization
• Unknown • May be spontaneous• Plasma cells containing crystals
infiltrate the cornea during episodes of conjunctivitis or anterior uveitis that sometimes precede the occurrence of corneal crystals
BAOP 2011
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• Occurs as punctate or linear opacities with an irregular geographic or plaque-like configuration
Paraproteinemic Crystalline Keratopathy
BAOP 2011
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• Affects the epithelium and the anterior or posterior portion of the stroma
• Limbal area in most cases is spared • Corneal vascularization or loss of corneal
sensation has not been reported• Recurrence of the immune crystalline deposits in
the grafted cornea is common in cases with uncontrolled systemic disorder
Paraproteinemic Crystalline Keratopathy
BAOP 2011
![Page 24: Mozhgan Rezaei Kanavi, MD. Ophthalmic Research Center Shahid Beheshti University of Medical Sciences, Tehran, Iran Appearance of Bilateral Corneal Opacities.](https://reader038.fdocuments.in/reader038/viewer/2022110207/56649d195503460f949ee366/html5/thumbnails/24.jpg)
*Confocal Microscopy*
As a non invasive diagnostic method is a useful technique for diagnosing and following
up such cases before and after systemic therapy and/or corneal transplantation.
Paraproteinemic Crystalline Keratopathy
![Page 25: Mozhgan Rezaei Kanavi, MD. Ophthalmic Research Center Shahid Beheshti University of Medical Sciences, Tehran, Iran Appearance of Bilateral Corneal Opacities.](https://reader038.fdocuments.in/reader038/viewer/2022110207/56649d195503460f949ee366/html5/thumbnails/25.jpg)
Histopathology• Homogeneous eosinophilic and PAS-positive
deposits in the epithelium and stroma that stain brilliant red with Masson's trichrome
• The deposits are strongly immune reactive for IgG-kappa chain and occasionally mildly for lambda chain.
BAOP 2011
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• Haematology consultation and proper systemic chemotherapy could be of help
• The opacities gradually diminish in patients receiving appropriate therapy
• Occasionally keratoplasty is necessitated in addition to the systemic therapy
Management
BAOP 2011
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CONCLUSION
• A rare case of “Paraproteinemic Crystalline Keratopathy” as a first clinical evidence of a non-symptomatic hypergammaglobulinemia
• Recurred after lamellar corneal graft• Haematology control would be necessary to
control the systemic as well as the ocular disorder.
BAOP 2011
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Thank You for Your Attention