sclerocornea
MONDO:0019629
A corneal disease in which the cornea blends with sclera, resulting in clouding of the cornea.
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Directly mapped studies only · unique clinical trialsCurrent registry observations. Phase counts may overlap. Study start dates are not registry registration dates. Investigational interventions are not approved-treatment recommendations.
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Verified disease and drug mappings that co-occur within the same study; not evidence of effectiveness.
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Sclerocornea
Sclerocornea is an extremely rare congenital anomaly of the eye, it is considered a form of congenital corneal opacity (CCO) with no clear gender bias, in which the cornea blends with sclera, having no clear-cut boundary. The extent of the resulting opacity varies from peripheral to total (sclerocornea totalis). While the exact historical origins of its documentation are unclear, studies on sclerocornea has long been recognized in ophthalmology as a rare but significant anomaly going as far back as the 1960's. The severe form is thought to be inherited in an autosomal recessive manner, but there may be another, milder form that is expressed in a dominant fashion. In some cases the patients also have abnormalities beyond the eye (systemic), such as limb deformities and craniofacial and genitourinary defects. According to one tissue analysis performed after corneal transplantation, the sulfation pattern of keratan sulfate proteoglycans in the affected area is typical for corneal rather than scleral tissue. Sclerocornea may be concurrent with cornea plana.
Background only. Canonical identity remains MONDO; clinical-trial relationships come from ClinicalTrials.gov.Drugs being studied
Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.