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). It usually appears in the newborn period or the first 3 months of life. and in most cases indicates the presence of congenital (infantile)

glaucoma Glaucoma is a group of eye diseases that result in damage to the optic nerve (or retina) and cause vision loss. The most common type is open-angle (wide angle, chronic simple) glaucoma, in which the drainage angle for fluid within the eye rem ...

, which is a disorder in which elevated pressures within the eye lead to structural eye damage and vision loss.

Signs and 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 Tearing is the act of breaking apart a material by force, without the aid of a cutting tool. A tear in a piece of paper, fabric, or some other similar object may be the result of the intentional effort with one's bare hands, or be accidental. Un ...

(lacrimation) and extreme sensitivity to light (

photophobia Photophobia is a medical symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of ...

). On ophthalmologic exam, a doctor can detect increased intraocular pressure, distortion of the

optic disc The optic disc or optic nerve head is the point of exit for ganglion cell axons leaving the eye. Because there are no rods or cones overlying the optic disc, it corresponds to a small blind spot in each eye. The ganglion cell axons form the ...

, and corneal

edema Edema, also spelled oedema, and also known as fluid retention, dropsy, hydropsy and swelling, is the build-up of fluid in the body's Tissue (biology), tissue. Most commonly, the legs or arms are affected. Symptoms may include skin which feels t ...

, which manifests as haziness. Other symptoms include a prominent eyeball,

Haab's striae Haab's striae, or Descemet's tears, are horizontal breaks in the Descemet membrane associated with congenital glaucoma Primary juvenile glaucoma is glaucoma that develops due to ocular hypertension and is evident either at birth or within the fir ...

tear in the

Descemet's membrane Descemet's membrane ( or the Descemet membrane) is the basement membrane that lies between the corneal proper substance, also called stroma, and the endothelial layer of the cornea. It is composed of different kinds of collagen (Type IV and VIII) ...

of the cornea, an enlarged cornea, and myopia.

Cause

Infantile glaucoma Primary juvenile glaucoma is glaucoma that develops due to ocular hypertension and is evident either at birth or within the first few years of life. It is caused due to abnormalities in the anterior chamber angle development that obstruct aqueous o ...

, which often produces the clinical sign of buphthalmos, can be caused when an abnormally narrow angle between the

cornea The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Along with the anterior chamber and lens, the cornea refracts light, accounting for approximately two-thirds of the eye's total optical power ...

and

iris Iris most often refers to: *Iris (anatomy), part of the eye *Iris (mythology), a Greek goddess * ''Iris'' (plant), a genus of flowering plants * Iris (color), an ambiguous color term Iris or IRIS may also refer to: Arts and media Fictional ent ...

blocks the outflow of

aqueous humor The aqueous humour is a transparent water-like fluid similar to plasma, but containing low protein concentrations. It is secreted from the ciliary body, a structure supporting the lens of the eyeball. It fills both the anterior and the posterior ...

; this causes increased

intraocular pressure Intraocular pressure (IOP) is the fluid pressure inside the eye. Tonometry is the method eye care professionals use to determine this. IOP is an important aspect in the evaluation of patients at risk of glaucoma. Most tonometers are calibrated to ...

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. Corneal diameter of greater than 11mm before the age of one year or corneal diameter greater than 13mm at any age are diagnostic criteria for buphthalmos.

Treatment

Untreated glaucoma leads to total blindness. Surgical treatment is required. Presently-utilized surgical procedures include goniotomy, trabeculotomy, or trabeculectomy. Goniotomy (ab interno) is done when the cornea is clear while in the case of a hazy or opaque cornea, trabeculotomy (ab externo) can be done. Some hyperosmotic agents such as glycerine can be applied over the cornea if cornea haziness is due to increase in intraocular pressure(with cornea epithelial/stromal edema). This is useful in enabling immediate visualization of the anterior chamber angle. Trabeculotomy-trabeculectomy can also be done as a combined procedure or trabeculectomy alone when the other previously mentioned procedures are either contraindicated or fails.

Etymology

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

Culture

US musical legend

Ray Charles Ray Charles Robinson Sr. (September 23, 1930 – June 10, 2004) was an American singer, songwriter, and pianist. He is regarded as one of the most iconic and influential singers in history, and was often referred to by contemporaries as "The Ge ...

, who was totally blind by age 7, had probably been afflicted with glaucoma and may have had buphthalmos early in life.http://www.swingmusic.net/Ray_Charles_Biography.html Swing Music website, page for Ray Charles

References

External links

{{Congenital malformations and deformations of eye Eye diseases Blindness Medical signs Congenital disorders of eyes