Congenital glaucoma and incomplete congenital glaucoma in two siblings. 1984

A Pollack, and M Oliver

The case histories and eye findings of 2 siblings of a non-sanguinous marriage are reported. The first child, a girl, had the typical findings of a unilateral congenital glaucoma. Her brother had an enlarged unilateral cornea (13 mm) without Descemet breaks, and normal intraocular pressure on repeated follow-up examination over 4 years. Gonioscopy showed numerous iris processes covering the iridocorneal angle. Fundus examination revealed a cup-disc ratio of 0.4 in the affected eye and 0.1 in the normal eye. Patients whose clinical picture resembles that of our second patient should be classified as having incomplete or abortive congenital glaucoma; such patients may develop raised intraocular pressure at a later stage. The term megalocornea should be reserved for eyes with enlarged cornea, normal intraocular pressure, normal iridocorneal angles, no cup-disc asymmetry and no family history of congenital glaucoma.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D009900 Optic Nerve The 2nd cranial nerve which conveys visual information from the RETINA to the brain. The nerve carries the axons of the RETINAL GANGLION CELLS which sort at the OPTIC CHIASM and continue via the OPTIC TRACTS to the brain. The largest projection is to the lateral geniculate nuclei; other targets include the SUPERIOR COLLICULI and the SUPRACHIASMATIC NUCLEI. Though known as the second cranial nerve, it is considered part of the CENTRAL NERVOUS SYSTEM. Cranial Nerve II,Second Cranial Nerve,Nervus Opticus,Cranial Nerve, Second,Cranial Nerves, Second,Nerve, Optic,Nerve, Second Cranial,Nerves, Optic,Nerves, Second Cranial,Optic Nerves,Second Cranial Nerves
D003315 Cornea The transparent anterior portion of the fibrous coat of the eye consisting of five layers: stratified squamous CORNEAL EPITHELIUM; BOWMAN MEMBRANE; CORNEAL STROMA; DESCEMET MEMBRANE; and mesenchymal CORNEAL ENDOTHELIUM. It serves as the first refracting medium of the eye. It is structurally continuous with the SCLERA, avascular, receiving its nourishment by permeation through spaces between the lamellae, and is innervated by the ophthalmic division of the TRIGEMINAL NERVE via the ciliary nerves and those of the surrounding conjunctiva which together form plexuses. (Cline et al., Dictionary of Visual Science, 4th ed) Corneas
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D005901 Glaucoma An ocular disease, occurring in many forms, having as its primary characteristics an unstable or a sustained increase in the intraocular pressure which the eye cannot withstand without damage to its structure or impairment of its function. The consequences of the increased pressure may be manifested in a variety of symptoms, depending upon type and severity, such as excavation of the optic disk, hardness of the eyeball, corneal anesthesia, reduced visual acuity, seeing of colored halos around lights, disturbed dark adaptation, visual field defects, and headaches. (Dictionary of Visual Science, 4th ed) Glaucomas
D005902 Glaucoma, Open-Angle Glaucoma in which the angle of the anterior chamber is open and the trabecular meshwork does not encroach on the base of the iris. Glaucoma Simplex,Glaucoma, Pigmentary,Glaucoma, Simple,Open-Angle Glaucoma,Chronic Primary Open Angle Glaucoma,Glaucoma, Compensated,Glaucoma, Compensative,Glaucoma, Open Angle,Glaucoma, Primary Open Angle,Glaucoma, Secondary Open Angle,Primary Open Angle Glaucoma,Secondary Open Angle Glaucoma,Compensated Glaucoma,Compensative Glaucoma,Open Angle Glaucoma,Open Angle Glaucomas,Open-Angle Glaucomas,Pigmentary Glaucoma,Simple Glaucoma,Simplex, Glaucoma,Simplices, Glaucoma
D006068 Gonioscopy Examination of the angle of the anterior chamber of the eye with a specialized optical instrument (gonioscope) or a contact prism lens. Gonioscopies
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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