Secondary Corneal Myxoma After DMEK (Descemet Membrane Endothelial Keratoplasty)

Abstract

BACKGROUND This is a clinical case of secondary corneal myxoma, which developed 18 months after Descemet membrane endothelial keratoplasty (DMEK). The DMEK was performed to treat viral endotheliitis and, postoperatively, a diagnosis of Crohn's disease was made. CASE REPORT A 52-year-old male, with no prior clinical history, presented with an endotheliitis in the left eye. The hypothesis of an undetected herpes infection was favored, and an antiviral treatment was prescribed using valacyclovir (1 g orally, 3 times daily). After 3 months of antiviral treatment, the endotheliitis was successfully controlled and a combined intervention of DMEK endothelial graft and phacoemulsification was performed. A corneal tumor was found 18 months after a successful DMEK procedure and was surgically removed. A pathological examination revealed a secondary corneal myxoma. CONCLUSIONS Corneal myxomas are rare lesions, often secondary to trauma in the Bowman's membrane, which is why it is often called a myxomatous corneal degeneration. In this instance, there is no link with Carney complex. However, myxomas under the eyelid or within the orbit are often associated with cardiac myxomas. Treatment is strictly surgical, either by simple excision or by surgical excision followed by graft. To the best of our knowledge, this is the first time that such an association between DMEK and secondary corneal myxoma has been described in the literature.

Figure 1.

Slit-lamp examination revealing a purely corneal white lesion, measuring about 9×5 mm, with visible vascularization, and covering almost all the upper-half of the cornea.

Figure 2.

Corneal involvement only, without associated limbus lesion, with chronic conjunctival hyperhemia.

Figure 3.

Anterior segment optical coherence tomography showing corneal myxoma: a round homogenous formation, mildly hyperreflective, which developed at the expense of the corneal epithelium and the Bowman’s membrane.

Figure 4.

Histological analysis showed a myxoid lesion projecting from the stroma, between the epithelium and Bowman’s membrane (HES ×2.5; A). The lesion was made of spindle or star-shaped fibroblastic elements, without cytonuclear atypia or mitosis (HES ×40; B). The lesion was colored with Alcian blue (×2.5; C). Immunohistochemistry analysis showed that tumoral cells were not targeted by anti-CD34 (×4; D), anti-PS100, or anti-actin smooth muscle antibodies. Proliferation index was estimated to under 1%, using Ki67 antibody.

Figure 5.

One month postoperatively bio-microscopical aspect.

Figure 6.

One month postoperatively anterior segment optical coherence tomography aspect.