Most infants with retinopathy of prematurity
undergo regression, which means that the abnormal proliferating blood
vessel disappear and are replaced with more normal-appearing vessels.
The further growth and development of the eye in the majority of
patients with regressed ROP is normal. Even infants with severe stage
3 ROP can usually be expected to have good vision assuming that
regression occurs without distortion or detachment of the retina. This
is true for patients who have undergone laser or cryotherapy as well
as for patients who have spontaneous regression. However, some long
term complications of ROP may occur.
REGRESSION OF ROP
The abnormal vascular shunt of ROP usually disappears in one of two
ways. In one type of regression, the shunt shrinks in size and normal
blood vessels cross the shunt and extend out to the retinal periphery.
REGRESSION OF ROP
This photograph shows some faint white lines
in the retina (arrowhead) where the neovascular ridge used to be.
Normal branching retinal vessels are growing over and beyond the white
"regression line" toward the edge of the retina.
In the other type of regression, the neovascular shunt itself moves
gradually out to the retinal periphery, leaving normal vessels in its
wake. When the shunt reaches the ora serrata (the anterior edge of the
retina), it disappears, usually leaving no trace. My experience has
been that most ROP regression is of this latter type.
RETINAL DRAGGING AND FOLDS
If the ROP produces significant fibrovascular
tissue, the retina may be distorted when the scar tissue contracts.
Instead of producing a retinal detachment, the traction may simply
drag the retina over the inside wall of the eye. This dragging is
associated with markedly decreased vision, but usually not with
complete blindness.
RETINAL DRAGGING
In this photograph, you can see the
yellow/white optic nerve head in the center of the picture. The
retinal vessels coming from the optic nerve do not have a normal
branching pattern, but instead are all dragged in the direction of the
arrow by scar tissue (out of picture on the right side). The macula,
which should be off the left edge of this photograph, is visible as a
subtle dark area about one disc diameter to the left of the disc. This
patient's vision would probably be 20/200 or worse, primarily because
of the dragging of the macula and subsequent amblyopia.
With more severe dragging or partial traction elevation of the retina
(stage 4A or 4B), folds in the retina may occur when the retina
reattaches. If one of these folds extend through the macular region,
markedly decreased vision (20/200 or worse) occurs, usually without
complete blindness.
GLAUCOMA
Infants with stage 5 ROP develop angle
closure glaucoma in as many as 30% of cases, probably due to the
contraction of fibrous membranes behind the lens. Multiple treatment
modalities have been tried for this problem, with limited success. A
late-onset form of angle closure glaucoma has been described in ROP
patients 12 to 45 years of age, although this is much less common.
LATE-ONSET RETINAL DETACHMENT
As the eye develops during childhood and
adolescence, it increases significantly in size. Retinal detachments
may occur in patients with regressed ROP in the mid-teens or early
adulthood as a result of this growth. If a portion of the retina is
firmly adherent to the wall of the eye due to fibrovascular scar or
laser/cryotherapy scar, tears in the retina can develop at the edges
of the scar because of increased traction. These tears then allow
fluid under the retina, and the retina detaches. Late-onset retinal
detachments are much easier to repair and carry a much better
prognosis than the traction detachments of stage 4 or 5 ROP,
especially if they are repaired quickly. Any person with a history of
ROP should have yearly retinal examinations during adolescence and
early adulthood.
MYOPIA, STRABISMUS AND AMBLYOPIA
Myopia, or nearsightedness, is much more
common in premature infants (6%) than in full-term infants (2%). It is
also more common in infants with ROP (24%) than in premature infants
without ROP, especially in patients with threshold ROP (70-80%). Lower
birth weight and increasing severity of ROP are strong predictors of
myopia. Fortunately, even high myopia can usually be corrected
satisfactorily with glasses. Stabismus (crossed eyes) and amblyopia
(lazy eye) are also more common in premature infants than in full term
infants, and are even more likely in premature children who had ROP.
Amblyopia can be especially severe in infants with stage 4 or 5 ROP,
and makes visual rehabilitation of these patients particularly
difficult. Eye patching for amblyopia and muscle surgery for
strabismus are often helpful. Because of the increased risk of these
two potentially treatable problems, premature infants need to be seen
more frequently during their first few years of life than term
infants.
RISK OF EYE PROBLEMS IN
PREMATURE VS. TERM INFANTS:
Myopia 6% vs. 2%
Amblyopia 4% vs. 0.1%
Strabismus 10% vs. 2%
Anisometropia (markedly different amount of nearsightedness or
farsightedness in the right and left eyes) 6% vs. 1.5%
Nystagmus (rapid "shaking" of the eyes) 2.5% vs. 0.1%
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