![]() Reduction of acuity predictably results when the visual axis is involved. Though there has been debate on whether it represents a truly unique corneal dystrophy and is not just a limited subtype of Meesmann microcystic corneal dystrophy, genetic analysis demonstrates abnormalities in different chromosomes of each dystrophy, indicating they are separate entities. Note the cystic appearance, which is most obvious at the margins. The lesion is connected to the 4 o’clock limbus. 3 Pedigree analysis suggests the dystrophy is inherited in an X-linked dominant fashion. On histology, light microscopy of the involved epithelium shows remarkable vacuolization of the cytoplasm of the cells, which are mostly optically empty. The involved epithelium in these other pathologies, however, tends to be coarse and gray rather than cystic. Given the whorled pattern sweeping from the limbus centrally, the condition looks somewhat like limbal stem cell dysfunction or advancing wavelike epitheliopathy. These cystic changes are most prominent on retroillumination. Upon high magnification slit lamp examination, the lesions appear to be clusters of microcystic epithelial tissue. The lesions are gray and feathery in appearance and frequently present in a whorled pattern. 1,2 It is characterized as a unilateral or bilateral condition that affects the corneal epithelial tissue sectorally. Lisch corneal dystrophy, sometimes referred to as band-shaped, whorled microcystic corneal dystrophy, is a relatively recently recognized corneal dystrophy, having first been described in 1992, and is currently recognized in the epithelial/subepithelial group by the International Classification of Corneal Dystrophies. As he was asymptomatic, I did not offer him treatment and asked him to follow up in six months. I asked the patient about his family history, which was unremarkable. My assessment was Lisch corneal dystrophy in the right eye on the basis of clinical evaluation alone. Interestingly, even though the lesion was in the far anterior cornea, there was no corresponding zone of anterior elevation abnormality. ![]() The lesion did not stain positively or negatively with sodium fluorescein.Ĭorneal topography showed a small area of steepening near the corneal irregularity, though it was mild and outside of the visual axis, and a negative posterior elevation close to the lesion. On close scrutinization, the lesion appeared to be a confluence of microcystic changes. A thin optic section localized the irregularity in the epithelial layer. The patient had a grayish feathery zone of anterior corneal tissue, which progressed from his 4 o’clock limbus to just below his visual axis in a whorled pattern. His right eye was normal with the exception of the cornea, which was remarkable. The slit lamp exam of the patient’s left eye was normal. The patient had a full range of motility with extraocular muscle function, a full confrontation field, normal pupils and intraocular pressures of 14mm Hg OD and 15mm Hg OS. Preliminary TestingĮntrance testing showed spectacle corrected vision of 20/20 OU. The patient had no concerns with his vision or comfort and was simply following up on his doctor’s orders. He had been seen recently for a routine evaluation by his primary OD who thought he should make an appointment at a cornea clinic. A 49-year-old male was referred for an evaluation of a corneal problem he had in his right eye.
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