Buphthalmos

Buphthalmos
Classification and external resources
Specialty medical genetics
ICD-10 Q15.0
ICD-9-CM 743.2
DiseasesDB 1778
MeSH D006871

Buphthalmos (plural: buphthalmoses) is enlargement of the eyeball and is most commonly seen in infants and young children. It is sometimes referred to as buphthalmia (plural buphthalmias).[1] It usually appears in the newborn period or the first 3 months of life.[2] and in most cases indicates the presence of congenital (infantile) glaucoma, which is a disorder in which elevated pressures within the eye lead to structural eye damage and vision loss.

Etiology

Infantile glaucoma, which often produces the clinical sign of buphthalmos, can be caused when an abnormally narrow angle between the cornea and iris blocks the outflow of aqueous humor;[3] this causes increased intraocular pressure and eventual enlargement of the globe (eyeball). Angle closure can be caused by developmental abnormalities of the eye as well as the presence of abnormal structures within the vitreous.[4]

Symptoms

Buphthalmos in itself is merely a clinical sign and does not generate symptoms. Patients with glaucoma often initially have no symptoms; later, they can exhibit excessive tearing (lacrimation) and extreme sensitivity to light (photophobia). On ophthalmologic exam, one can detect increased intraocular pressure, distortion of the optic disc, and corneal edema, which manifests as haziness.

Signs

Prominent eyeball, haab's striae in descemets membrane of cornea, enlarged cornea, myopia.

Prognosis and treatment

Untreated glaucoma leads to total blindness. Surgical treatment is required. Presently-utilized surgical procedures include goniotomy, trabeculotomy, or trabeculectomy.

Etymology

The name of the condition derives from the Greek βοῦς bous (ox or cow), referring to the bulging eyes common to bovines.

Cases of buphthalmos in popular culture

US musical legend Ray Charles, who was totally blind by age 7, had probably been afflicted with glaucoma and may have had buphthalmos early in life.[5]

References

  1. http://www.merriam-webster.com/medical/Buphthalmos Merriam-Webster online medical dictionary
  2. Merriam-Webster: Over 80% of cases are evident by 3 months of age.
  3. The Schlemm's canal is usually collapsed (Merriam-Webster)
  4. Yanoff & Duker: Ophthalmology, 3rd ed. 2008
  5. http://www.swingmusic.net/Ray_Charles_Biography.html Swing Music website, page for Ray Charles
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