Abstract
BackgroundPellucid marginal corneal degeneration is a non-inflammatory disorder complicated by severe inferior corneal thinning. The central portion of the cornea, consequently, appears to protrude outwards, decreasing vision by means of an irregular stigmatism. Additionally, acute hydrops can occur in case of Descemet’s membrane rupture.Case presentationA 41-year-old Japanese woman presenting with severe visual loss in the left eye was examined and observed to have had full thickness corneal perforation as well as a Descemet membrane rupture with massive edema in the corneal stroma. Anterior segment optical coherence tomography-based corneal topography revealed a distorted crab claw sign indicating pellucid marginal corneal degeneration. The Descemet membrane rupture allowed acute hydrops to occur which was especially noteworthy given the scale of edema present within the stroma, rotating 180 degrees along the limbus, causing a smiley-face like lesion. We visualized it via a gonioscopic three-dimensional optical coherence tomography to build a three-dimensional video. Patient history revealed a previous acute hydrops in the right eye as well, which was ultimately treated with anterior lamellar keratoplasty, suggesting the pellucid marginal corneal degeneration had a classic bilateral involvement, which was also characterized with bilateral acute hydrops.ConclusionThis appears to be a very rare and interesting presentation of bilateral pellucid marginal degeneration, wherein not only acute hydrops formed bilaterally, but the cavity within the cornea stroma was exceptionally large with an unusual shape. Using the gonioscopic three-dimensional optical coherence tomography imaging, we were able to easily visualize the massive intrastromal cavern, and appropriately planned the crescent-shaped anterior lamellar keratoplasty. The 3d video constructed using this data is particularly elucidative compared to 2d images. As such, we recommend utilizing 3d imaging in cases where more conventional topography is not as explanatory with respect to precise nature of deformation.
Highlights
Pellucid marginal corneal degeneration is a non-inflammatory disorder complicated by severe inferior corneal thinning
This appears to be a very rare and interesting presentation of bilateral pellucid marginal degeneration, wherein acute hydrops formed bilaterally, but the cavity within the cornea stroma was exceptionally large with an unusual shape
We describe a Pellucid marginal corneal degeneration (PMCD) patient with full thickness corneal perforation followed by acute hydrops with a massive edematous cavern, which we visualized with anteriorsegment optical coherence tomography (AS-OCT) gonioscopic view
Summary
We described a case with a very severe corneal perforation by acute hydrops with a massive edematous cavernous structure that was refractory to medical treatment. AS-OCT 3D imaging was useful for examining the detailed structure of the pathologic lesion, for purposes of planning surgery, and for monitoring corneal status in follow-up examinations. Additional file 1: 3d video of edema within the cornea. Video demonstrates in 3d the corneal edema with rotating image.
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