RETINOPATHY OF PREMATURITY

Retinopathy of prematurity (ROP) or Terry syndrome, previously known

as retrolental fibroplasia (RLF), is a disease of the eye affecting
prematurely-born babies generally having received intensive neonatal care,
in which oxygen therapy is used on them due to the premature development
of their lungs. It is thought to be caused by disorganized growth
of retinal blood vessels which may result in scarring and retinal detachment.
ROP can be mild and may resolve spontaneously, but it may lead
to blindness in serious cases. As such, all preterm babies are at risk for ROP,
and very low birth weight is an additional risk factor. Both oxygen
toxicity and relative hypoxia can contribute to the development of ROP.
CAUSES
ROP is a disease of premature infants. All babies less than 1500 g birth weight or younger than 32
weeks gestational age at birth are at risk of developing ROP.
Tolsma et al found that late neonatal bacteremia appears to be an independent risk factor for
prethreshold/threshold ROP and plus disease. The study also found that presumed late bacteremia
was associated with prethreshold/threshold ROP.

Risk factors

Birth before 32 weeks' gestation, especially before 30 weeks

Birth weight of less than 1500 g, especially less than 1250 g

Possible risk factors include supplemental oxygen, hypoxemia,

hypercarbia, concurrent illness

ROP is categorized by the severity of the disease in zones of the retina. It is

categorized by the lowest zone and the highest stage observed in each eye.
Zone 1

The center of zone 1 is the optic nerve

The zone extends twice the distance from the optic nerve to the
macula in a circle

Any portion of the optic nerve in the same view as the ridge of ROP is
considered zone 1

Zone 2

Zone 2 is a circle surrounding the zone 1 circle, with the nasal ora serrata as
its nasal border. Zone 2 disease may progress quickly, but the following
warning signs usually predate the threshold by 1-2 weeks:

The ridge shows signs of vascular arcading (increased branching); this

is usually a sign that the disease is starting to become aggressive

Increasing vascular dilation and tortuosity is present

A "hot dog" on the ridge (ie, a thickened, red, vascular area that may
not show the typical fronds of neovascularization); it usually is seen in
posterior zone 2 (borders zone 1); it indicates a poor prognosis

Threshold disease: 5 contiguous or 8 non-contiguous hours of

neovascularization (stage 3) with plus disease in zone 1 or 2

Zone 3

Zone 3 is the crescent that the circle of zone 2 did not encompass
temporally

Aggressive disease rarely is seen in zone 3

Slow vascularization is typical, requiring evaluations every few weeks

Many infants show inactive disease in zone 3, with a demarcation line

and nonvascularized retina; this has been noted in toddlers, can be
considered cicatricial peripheral disease, and has no known ill sequelae

Stage 0

The mildest form of ROP

Immature retinal vasculature

No clear demarcation of vascularized and nonvascularized retina is

present

In zone 1, stage 0 may appear as a vitreous haze, with the optic nerve
as the only landmark; weekly examinations should be performed

In zone 2, bimonthly examinations should be performed

In zone 3, examination every 3-4 weeks should be sufficient

Stage 1

A fine, thin demarcation line between the vascular and avascular

region is present

The demarcation line has no height and no thickness

In zone 1, the line should appear flat and thin (usually nasally first),
with no elevation from the avascular retina, and retinal vessels should
be smooth, thin, and supple

In zone 1, weekly examinations should be performed

In zone 2, bimonthly examinations should be performed

In zone 3, examination every 2-3 weeks should be sufficient

Stage 2

A broad, thick ridge clearly separates the vascular from the avascular
retina

In zone 1, any hint of pink or red in the ridge is an ominous sign

If there is any vessel engorgement in zone 1, the disease should be

considered threshold and treatment commenced within 72 hours

In zone 2, if there are no vascular changes and the ridge has no

engorgement, the eye should be examined within 2 weeks

In zone 2, prethreshold is defined as stage 2 with plus disease

In zone 3, examination every 2-3 weeks should be sufficient, unless

there is any vascular tortuosity or straightening of the vascular arcades

Stage 3
Extraretinal fibrovascular proliferation (neovascularization):

Sites include the ridge, the posterior surface of the ridge, and
anteriorly toward the vitreous cavity

The neovascularization gives the ridge a velvety appearance and a

ragged border

In zone 1, any neovascularization is serious and requires treatment

In zone 2, prethreshold is defined as stage 3 without plus disease, or

stage 3 with less than 5 contiguous or 8 non-contiguous hours;
threshold is stage 3 with at least 5 contiguous or 8 non-contiguous
hours and plus disease

In zone 3, examination every 2-3 weeks should be sufficient, unless

there is any vascular tortuosity or straightening of the vascular arcades

Stage 4

Subtotal retinal detachment beginning at the ridge

The retina is pulled anteriorly into the vitreous by the fibrovascular

ridge

Stage 4A does not involve the fovea

Stage 4B involves the fovea

Stage 5
This stage is a total retinal detachment in the shape of a funnel:

Stage 5A is an open funnel

Stage 5B is a closed funnel

Treatment

laser surgery, the most common type of ROP surgery, in which small
laser beams are used to scar the peripheral retina (also called laser
therapy or photocoagulation). This procedure lasts about 30-45 minutes
for each eye.
cryotherapy, where freezing temperatures are used to scar the
peripheral retina to stop abnormal blood vessel growth. For many years,
cryotherapy (also called cryosurgery) was the accepted method of ROP
surgery, but it has been all but replaced by laser therapy.
scleral buckling, which involves placing a flexible band, usually made
of silicone, around the circumference of the eye. The band is placed

around the sclera, or the white of the eye, causing it to push in, or
"buckle." This, in turn, causes the torn retina to push closer to and remain
against the outer wall of the eye. This surgery takes 1-2 hours.

Management of ROP is as follows:

Screening at-risk preterm infants at proper times and intervals, as

recommended by the American Academy of Pediatrics.

Although oxygen therapy has been blamed for ROP progression in the
past, maximizing the oxygen saturation (to 95%) may induce
regression in prethreshold disease

Cryotherapy was the original mode of treatment (since the 1970s)

Laser surgery (eg, xenon, argon, diode) is as effective as cryotherapy,

does not require general anaesthesia, and has a lower complication
rate

Scleral buckling surgery and/or vitrectomy is usually performed for

stages 4 and 5

Surgery for stage 4A and stage 5 is controversial

Prevention
The best way to prevent this condition is to take steps to avoid
premature birth. Preventing other problems of prematurity may also
help prevent ROP.