Korean J Ophthalmol.  2011 Jun;25(3):202-205. 10.3341/kjo.2011.25.3.202.

A Case of Crystalline Keratopathy in Monoclonal Gammopathy of Undetermined Significance (MGUS)

• Koo H
• Oh DH
• Chun YS
• Kim JC

• KMID: 1010029
• DOI: http://doi.org/10.3341/kjo.2011.25.3.202

Abstract

A 62-year-old female visited our clinic with progressively decreased vision in both eyes beginning 12 years prior. Idiopathic corneal opacity in all layers of the cornea was found in both eyes. One year later, we performed penetrating keratoplasty on the undiagnosed right eye. During post-surgical follow-up, corneal edema and stromal opacity recurred, and penetrating keratoplasty was performed two more times. The patient's total serum protein level, which had previously been normal, was elevated prior to the final surgery. She was diagnosed with monoclonal gammopathy of undetermined significance. We made a final diagnosis of monoclonal gammopathy-associated crystalline keratopathy after corneal biopsy. Monoclonal gammopathy-associated crystalline keratopathy is difficult to diagnose and may lead to severe visual loss. A systemic work-up, including serologic tests like serum protein or cholesterol levels, is needed in patients with unexplainable corneal opacity.

Keyword

Corneal opacity; Crystallins; Paraproteinemias

MeSH Terms

Corneal Edema/etiology/*metabolism/physiopathology/surgery
Corneal Neovascularization/etiology/*metabolism/physiopathology/surgery
Corneal Opacity/etiology/*metabolism/physiopathology/surgery
Crystallins/*metabolism
Female
Humans
Keratoplasty, Penetrating
Microscopy, Electron
Middle Aged
Monoclonal Gammopathy of Undetermined Significance/*complications/pathology
Reoperation
Visual Acuity

Figure

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  Fig. 1 Slit-lamp photographs of recurring corneal opacity in a crystalline keratopathy patient with monoclonal gammopathy of undetermined significance despite keratoplasty surgeries. (A) Before the penetrating keratoplasty, corneal stromal edema, opacity and Descemet's folds presented in both eyes. (B) Four years after the first surgery, corneal opacity recurred in the right eye. (C) Photograph showing corneal opacity two years after the second keratoplasty.

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  Fig. 2 Corneal biopsy photographs from a crystalline keratopathy patient. (A) Hematoxylin and eosin staining showing loss of polarity, with clusters in the epithelium (×100). (B) Hematoxylin and eosin staining showing a decreased number of endothelial cells with some vacuoles in Descemet's membrane (×40). (C) TUNEL staining showing increased keratocyte apoptosis with condensed nuclei (×100). (D) Masson trichrome staining showing deposits in the corneal stroma (×40).

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  Fig. 3 Electron microscopic photographs of crystalline keratopathy. (A) Microtubular crystal deposits were found around a keratocyte whose chromatin was condensed (×28,000). (B) Crystal deposits phagocytosed by a macrophage (white arrow) (×28,000). (C) Vacuoles in the endothelium (black arrow) and loss of microvilli (arrow head) (×10,000). (D) Abnormal banded collagen fibrils were seen with a background of amorphous material in the posterior portion of Descemet's membrane (×28,000).

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