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A 67-year-old man presented with declining bilateral vision over several months. His medical history was notable for IgG lambda multiple myeloma. Best corrected visual acuity was 20/70 in each eye. Slit-lamp examination revealed an abnormal gray-white deposition of material at the level of the Descemet membrane centrally in both corneas (Figure 1). Visual acuity progressively declined during chemotherapy, with worsening corneal deposits. The patient underwent Descemet stripping endothelial keratoplasty in the right eye to replace the host Descemet membrane, endothelium, and corneal deposits with healthy donor tissue, resulting in substantial clearance of the corneal opacity. Histopathologic examination revealed deposition of acellular granular material anterior to the Descemet membrane (Figure 2). Liquid chromatography–tandem mass spectrometry revealed IgG heavy chain and lambda light chains. Uncorrected visual acuity at 2 weeks was 20/80 with further improvement expected, although the patient could not return for further assessment because of adverse effects of chemotherapy and subsequently death.
Immunoglobulin deposition in the cornea is uncommon, occurs through unknown mechanisms, usually manifests as crystals in the epithelium or anterior stroma, and typically does not impair vision.
Posterior corneal paraprotein deposition, as in this case, is a rarer manifestation of myeloma that has a more substantial impact on vision and has previously been managed by penetrating (full-thickness) keratoplasty.
This case was successfully managed by partial-thickness Descemet stripping endothelial keratoplasty. New vision impairment in patients with monoclonal gammopathies should be evaluated for crystal or paraprotein deposition in the cornea. Similarly, corneal deposits could be the presenting sign of a malignant process associated with a monoclonal gammopathy.
Incidence of corneal crystals in the monoclonal gammopathies.