In Vivo Confocal Microscopy of Combined Pre-Descemet Membrane Corneal Dystrophy and Fuchs Endothelial Dystrophy

Yeh, Shu‐I; Liu, Tzong-Shyne; Ho, Chia-Ching; Cheng, Huey-Chuan

doi:10.1097/ico.0b013e3181e2cf3f

Cited by 17 publications

(11 citation statements)

References 20 publications

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“…We found that the activated keratocytes in the posterior stroma had enlarged cell bodies with a regular arrangement of hyperreflective particles inside. These IVCM findings were similar to the previous reports of isolated PDCD in the literatures [2, 11, 12]. In some other previous studies, the size and morphology of keratocytes in the posterior stroma were reported to be normal in isolated PDCD, although hyperreflective particles were also found in the intracellular and extracellular compartments [9, 10, 13].…”

Section: Discussionsupporting

confidence: 91%

“…However, other IVCM studies have found that the lesion of PDCD was involved in the whole thickness of the corneal stroma [9, 10, 13]. The involvement of corneal endothelial layer was also reported in some PDCD patients [2, 12]. In our present case, using IVCM, we found that the enlarged keratocytes with hyperreflective particles in the posterior stroma were consistent with the histopathological study.…”

Section: Discussionsupporting

confidence: 90%

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In vivo confocal microscopy of pre-Descemet corneal dystrophy associated with X-linked ichthyosis: a case report

Shi

Liu

et al. 2017

BMC Ophthalmol

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BackgroundPre-Descemet corneal dystrophy (PDCD) is characterized by the presence of numerous, tiny, polymorphic opacities immediately anterior to Descemet membrane, which is a rare form of corneal stromal dystrophy and hard to be diagnosed. In vivo confocal microscopy (IVCM) is a useful tool to examine the minimal lesions of the cornea at the cellular level. In this article, we report a rare case of PDCD associated with X-linked ichthyosis and evaluate IVCM findings.Case presentationWe present a 34-year-old male Chinese patient with PDCD associated with X-linked ichthyosis. Slit-lamp biomicroscopy showed the presence of tiny and pleomorphic opacities in the posterior stroma immediately anterior to Descemet membrane bilaterally. IVCM revealed regular distributed hyperreflective particles inside the enlarged and activated keratocytes in the posterior stroma. Hyperreflective particles were also observed dispersedly outside the keratocytes in the anterior stroma. Dermatological examination revealed that the skin over the patient’s entire body was dry and coarse, with thickening and scaling of the skin in the extensor side of the extremities. PCR results demonstrated that all ten exons and part flanking sequences of STS gene failed to produce any amplicons in the patient.ConclusionsIVCM is useful for analyzing the living corneal structural changes in rare corneal dystrophies. We first reported the IVCM characteristics of PDCD associated with X-linked ichthyosis, which was caused by a deletion of the steroid sulfatase (STS) gene, confirmed by gene analysis.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 90%

In vivo confocal microscopy of pre-Descemet corneal dystrophy associated with X-linked ichthyosis: a case report

Shi

Liu

et al. 2017

BMC Ophthalmol

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“…32,40–43 Between them, pleomorphic and polymegathic endothelial cells appeared hyperreflective and could not be identified individually. In the late stage, diffuse hyporeflective areas surrounded by hyperreflective endothelial cells were noted.…”

Section: Resultsmentioning

confidence: 96%

“…On slit-lamp examination, larger and more polymorphous opacities than those of Cornea Farinata or Fleck’s dystrophy are seen, distributed throughout the cornea but with a predilection for the deep stroma. 32 IVCM demonstrates intra- and extra-cellular hyper-reflective inclusions, pleomorphic in shape and size, measuring from 30 μm to 80 μm immediately anterior to Descemet’s membrane and prominent subbasal nerves (Figure 4). 30 The intracellular particles have been noted inside enlarged keratocytes with abnormally visible intercellular processes.…”

Section: Resultsmentioning

confidence: 99%

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Confocal Microscopy of Corneal Dystrophies

Shukla

Cruzat

Hamrah

2012

Seminars in Ophthalmology

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In vivo confocal microscopy (IVCM) of the cornea is becoming an indispensable tool in the cellular study of corneal physiology and disease. This technique offers non-invasive imaging of the living cornea with images comparable to that of ex vivo histology. The ability to provide high-resolution images of all layers in the living cornea has resulted in new discoveries of corneal pathology at the cellular level. The IVCM analysis of corneal dystrophies is of importance to clinicians, as current methods of diagnosis involve slit-lamp characteristics, genetic analysis, and invasive biopsy. IVCM is helpful in evaluating the morphological characteristics of corneal dystrophies at the histological level and may be helpful in diagnosis, determination of progression, and understanding the pathophysiology of disease. The purpose of this review is to describe the principles, applications, and clinical correlation of IVCM in the study of corneal dystrophies.

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