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Mozhgan Rezaei Kanavi , MD. Ophthalmic Research Center

Appearance of Bilateral Corneal Opacities in a 61-year-old man. Mozhgan Rezaei Kanavi , MD. Ophthalmic Research Center Shahid Beheshti University of Medical Sciences, Tehran, Iran. BAOP 2011. Case Report. A 61-year-old male Transportation driver

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Mozhgan Rezaei Kanavi , MD. Ophthalmic Research Center

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  1. Appearance of Bilateral Corneal Opacities in a 61-year-old man MozhganRezaeiKanavi, MD. Ophthalmic Research Center ShahidBeheshti University of Medical Sciences, Tehran, Iran BAOP2011

  2. Case Report • A 61-year-old male • Transportation driver • Vague past medical history of non-symptomatic dysproteinemia • Recent decrease in visual acuity BAOP2011

  3. Slit Lamp Biomicroscopy BAOP2011

  4. Case Report • VA: OD: 20/160 OS: 20/200 • No evidence of conjunctivitis or uveitis • IOP: WNL • Funduscopy: Unremarkable BAOP2011

  5. Corneal Confocal Scan BAOP2011

  6. Corneal Confocal Scan BAOP2011

  7. … Case Report • Clinical diagnosis of a “Crystalline Keratopathy” • Systemic work-up • Diagnostic Anterior Lamellar Keratoplasty OS BAOP2011

  8. … Case Report • 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 BAOP2011

  9. Results BAOP2011

  10. Results BAOP2011

  11. Results BAOP2011 PAS

  12. Results BAOP2011

  13. Results Congo Red Alcian blue BAOP2011

  14. Results BAOP2011 Kappa chain

  15. Results BAOP2011 Lambda chain

  16. Histopathological Diagnosis ParaproteinemicCrystalline Keratopathy BAOP2011

  17. 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 BAOP2011

  18. Four Months after Keratoplasty • 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 BAOP2011

  19. Can be classified from the clinical and genetical point of view into 3 groups: Primary hereditary (Schnyder) Secondary hereditary (Cystinosis) Secondary non-hereditary in association with disorders of serum protein or lipid composition Non Infectious Crystalline Keratopathy BAOP2011

  20. Paraproteinemic Crystalline Keratopathy • 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 BAOP2011

  21. 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 BAOP2011

  22. Paraproteinemic Crystalline Keratopathy • Occurs as punctate or linear opacities with an irregular geographic or plaque-like configuration BAOP2011

  23. Paraproteinemic Crystalline Keratopathy • 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 BAOP2011

  24. Paraproteinemic Crystalline Keratopathy *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.

  25. 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. BAOP2011

  26. Management • 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 BAOP2011

  27. 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. BAOP2011

  28. Thank You for Your Attention

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