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Minna H. Vesaluoma, Eeva–Marja Sankila, Juana Gallar, Linda J. Müller, W. Matthew Petroll, Jukka A. O. Moilanen, Henrik Forsius, Timo M. T. Tervo; Autosomal Recessive Cornea Plana: In Vivo Corneal Morphology and Corneal Sensitivity. Invest. Ophthalmol. Vis. Sci. 2000;41(8):2120-2126.
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purpose. Autosomal recessive corneal plana (RCP) is a rare corneal anomaly with
unknown pathogenesis and a high incidence in Finland. The aim was to
examine corneal sensitivity and the morphology of different corneal
layers and subbasal nerves in RCP patients.
methods. Three patients with a diagnosed autosomal recessive cornea plana were
examined. Corneal sensitivity to different modalities of stimulation
was tested in four corneas using noncontact esthesiometry. Tissue
morphology of three corneas was evaluated, and in two corneas thickness
of corneal layers was measured using in vivo confocal microscopy.
results. Corneas of RCP patients appear to have mechanosensory, polymodal, and
cold-sensitive nerve terminals. RCP patients had normal sensation
thresholds for chemical, heat, and cold stimulation but a high
threshold for mechanical stimulation. Their capacity to discriminate
increasing intensities of stimulus was reduced, except for cold
stimuli. Thickness of the epithelial layer was reduced, whereas total
corneal and stromal thicknesses were slightly reduced or close to
normal values. In all cases Bowman’s layer was absent. Subbasal nerves
had abnormal branching patterns. The arrangement of anterior
keratocytes was altered, showing clustered and irregularly shaped
nuclei. Increased backscattering of light in confocal microscopy
through focusing (CMTF) profiles was observed throughout the stroma.
Epithelial and endothelial cells appeared to be regular in shape.
conclusions. The present study revealed qualitative and quantitative alterations in
corneal sensitivity, cellular morphology, and the thickness of corneal
layers in RCP patients.
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