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
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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.
Thank You for Your Attention