Anophthalmia is a medical term used to describe
the absence of the globe and ocular tissue from the orbit.
This was first reported
more than 400 years ago, yet it is only recently that
significant reconstructive options became available.
There are many reasons why
one might lose an eye.
Surgeries which result in
anophthalmos
There are three
classifications for this condition:
Primary
anophthalmia is a complete absence of eye tissue
due to a failure of the part of the
brain that
forms the eye.
Secondary
anophthalmia the eye starts to develop and for
some reason stops, leaving the infant with only residual eye
tissue or extremely small eyes which can only be seen under
close examination.
Degenerative
anophthalmia the eye started to form and, for some
reason, degenerated. One reason for this occurring could be a
lack of blood supply to the eye.
Etiology
Anophthalmia ( A medical term used to describe the
absence of the globe and ocular tissue from the orbit ) can be
congenital (present at birth) or acquired later in life.
Congenital
anophthalmia can occur alone or along with other birth
defects. Cases of
Anophthalmia may result from inherited genetic mutations,
sporadic genetic mutations, chromosome abnormalities, prenatal
environmental insult or unknown.
True or
primary anophthalmos is very rare. Only when there is complete
absence of the ocular tissue within the orbit can the diagnosis
of true anophthalmos be made. Extreme microphthalmos is seen
more commonly. In this condition, a very small globe is present
within the orbital soft tissue, which is not visible on initial
examination.
Anophthalmia and microphthalmia may occur secondary to
the arrest of development of the eye at various stages of growth
of the optic vesicle. It is important to recognize
microphthalmia because the development of the orbital region, as
well as the lids and fornices, is dependent on the presence of a
normal-sized eye in utero.
Anophthalmia is sometimes a clinical characteristic of
Trisomy 13 which is a Gross Chromosomal Abnormality.
(Anophthalmia
is very rare but the exact incidence is unknown. One report from
a prospective study of 50,000 newborns found an incidence of
microphthalmia of 0.22 per 1,000 live births)
trauma
infection
tumor (such as
retinoblastoma, choroidal malignant melanoma)
Data from the U.S. National Center for Health Statistics
estimate that nearly 2.4 million eye injuries occur in the
United States annually. This report calculated that nearly one
million Americans have permanent significant visual impairment
due to injury, with more than 75% of these individuals being
blind in one eye. Eye injury is a leading cause of monocular
blindness in the United States, and is second only to cataract
as the most common cause of visual impairment. While no segment
of the population escapes the risk of eye injury, the victims
are more likely to be young. The majority of all eye injuries
occur in persons under thirty years of age. Trauma is considered
the most common cause of
enucleation in children over three years of age.
For the year 2000, Texas demographics for cancer of the eye
and orbit were fewer than five per 100,000. According to the
Nilt, there are about 2,200 cases of eye cancer diagnosed in the
United States each year
Removal of the eye by
enucleation or
evisceration can relieve pain and minimize further risk to
life and well-being of an individual with the above noted
conditions. Goals in Surgery