Sclerocornea
Encyclopedia
Sclerocornea is a congenital anomaly of the eye
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). 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
proteoglycan
s in the affected area is typical for corneal rather than scleral tissue.
Sclerocornea may be concurrent with cornea plana
.
Human eye
The human eye is an organ which reacts to light for several purposes. As a conscious sense organ, the eye allows vision. Rod and cone cells in the retina allow conscious light perception and vision including color differentiation and the perception of depth...
in which the cornea
Cornea
The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Together with the lens, the cornea refracts light, with the cornea accounting for approximately two-thirds of the eye's total optical power. In humans, the refractive power of the cornea is...
blends with sclera
Sclera
The sclera , also known as the white or white of the eye, is the opaque , fibrous, protective, outer layer of the eye containing collagen and elastic fiber. In the development of the embryo, the sclera is derived from the neural crest...
, having no clear-cut boundary
Corneal limbus
The corneal limbus is the border of the cornea and the sclera . The limbus is a common site for the occurrence of corneal epithelial neoplasm....
. The extent of the resulting opacity varies from peripheral to total (sclerocornea totalis). 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
Systemic disease
Life-threatening disease redirects here.A systemic disease is one that affects a number of organs and tissues, or affects the body as a whole. Although most medical conditions will eventually involve multiple organs in advanced stage Life-threatening disease redirects here.A systemic disease is one...
), such as limb deformities and craniofacial and genitourinary defects.
According to one tissue analysis performed after corneal transplantation, the sulfation pattern of keratan sulfate
Keratan sulfate
Keratan sulfate , also called keratosulfate, is any of several sulfated glycosaminoglycans that have been found especially in the cornea, cartilage, and bone. It is also synthesized in the central nervous system where it participates both in development and in the glial scar formation following an...
proteoglycan
Proteoglycan
Proteoglycans are proteins that are heavily glycosylated. The basic proteoglycan unit consists of a "core protein" with one or more covalently attached glycosaminoglycan chain. The point of attachment is a Ser residue to which the glycosaminoglycan is joined through a tetrasaccharide bridge...
s in the affected area is typical for corneal rather than scleral tissue.
Sclerocornea may be concurrent with cornea plana
Cornea plana
Cornea plana may refer to:*Cornea plana 1, an eye condition*Cornea plana 2, an eye condition...
.
External links
- Congenital Clouding of the Cornea - eMedicine; by Noah S Scheinfeld, MD, JD, FAAD and Benjamin D Freilich, MD, FACS