BAOP 2011

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

Case Report • A 61-year-old male • Transportation driver • Vague past medical history of nonsymptomatic dysproteinemia • Recent decrease in visual acuity

Slit Lamp Biomicroscopy

Case Report • • • •

VA: OD: 20/160 OS: 20/200 No evidence of conjunctivitis or uveitis IOP: WNL Funduscopy: Unremarkable

Corneal Confocal Scan

Corneal Confocal Scan

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

… 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

Kappa chain

Lambda chain

Histopathological Diagnosis

Paraproteinemic Crystalline Keratopathy

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

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

Non Infectious Crystalline Keratopathy 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

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

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

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

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

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.

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.

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

• 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.

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