RETINOPATHY OF PREMATURITYChikita Artia Sari / I 11109014

Definition Retinopathy of prematurity (ROP) is a

disease that primarily occurs in premature babies.

It causes abnormal blood vessels to grow in the retina, the layer of nerve tissue in the eye that enables us to see. This growth can cause the retina to detach from the back of the eye, leading to blindness.

Definition ROP originally called retrolental fibroplasia,

was the leading cause of blindness in children in the 1940s and 1950s.

ROP occurs when abnormal blood vessels grow and spread throughout the retina, the tissue that lines the back of the eye. These abnormal blood vessels are fragile and can leak, scarring the retina and pulling it out of position. This causes a retinal detachment. Retinal detachment is the main cause of visual impairment and blindness in ROP. 

Epidemiology Related to gestational age (GA) and birth

weight (bw). ROP rare in bw > 2000 grams. 70% ROP in bw < 1250g and 7% develop

threshold ROP. Thereshold ROP very rare in bw > 1250g. 95% ROP begins at 32-34 weeks GA. Threshold disease at 36 weeks. Regression with spontaneous healing at 45-48

weeks GA. Long term ophthalmic follow up of formerly

premature neonates who suffered severe ROP.

Risk Factors Prematurity (most significant) LBW Assisted ventilation longer than one week Surfactant therapy High blood transfusion volume Sepsis IVH Bronchopulmonary dysplasia Elevated arterial oxygen tension

PathophysiologyStage I: an initial injury (such as hypotension, hypoxia, or hyperoxia) causes vasoconstriction and reduced blood flow to the retina, disrupting the normal process of vascularization.

Stage II: Vessels then either resume normal growth or new vessels grow abnormally out from the retina into the vitreous. The abnormal vessels have increased permeability which can result in edema and hemorrhage. Inflammation → fibrous tissue → traction on the retina and detachment. Alternatively, the abnormal vascularization may regress with little residual effect.

Pathophysiology Recently, the interaction between insulin-

like growth factor-1 (IGF-1) and VEGF has been studied and proposed to play a role in the pathogenesis of ROP

Pathophysiology

DiagnosisFour features are evaluated:

Zone (1-3) Stage (1-5) Extent Presence or absence of plus disease

Zone 1, any stage ROP with plus disease. Zone 2, stage 3 with or without plus

disease. Zone 2, stage 2/3 with plus disease.

Stage IMildly abnormal blood vessel growth. Many children who develop stage I improve with no treatment and eventually develop normal vision. The disease resolves on its own without further progression.

Stage IIModerately abnormal blood vessel growth. Many children who develop stage II improve with no treatment and eventually develop normal vision. The disease resolves on its own without further progression.

Stage IIISeverely abnormal blood vessel growth. The abnormal blood vessels grow toward the center of the eye instead of following their normal growth pattern along the surface of the retina. Some infants who develop stage III improve with no treatment and eventually develop normal vision.

Stage IVPartially detached retina. Traction from the scar produced by bleeding, abnormal vessels pulls the retina away from the wall of the eye.o Stage 4A: excludes

the maculao Stage 4B: includes

the macula

Stage VCompletely detached retina and the end stage of the disease. If the eye is left alone at this stage, the baby can have severe visual impairment and even blindness.

"Plus disease“ Presence indicates

severe ROP and is often followed by rapid progression

to retinal detachment. May be accompanied by vitreous haze, engorgement

of the iris vessels, and poor dilation of the pupil.

Management ETROP & Cryo-ROP Treat threshold disease as it decreases

unfavourable outcome from 15 to 9%. Ablation is beneficial for prethreshold ROP.

Photograph screening for ROP Telemedicine with on-site treatment.

Management Evidence based screening criteria Screen at 31 weeks GA or 4 weeks

chronologic age if born before 27 weeks. Continue screening till 45 weeks GA or

vascularisation in zone 3. Screening of 1250g-1800g infants is cost

effective. ETROP : Recognize prethreshold disease

and follow up 2 weekly. Follow up weekly if zone 2, stage

2 ROP or if vascularisation ends in zone 1.

Management Treatment of ROP Portable indirect laser units. Laser superior to

cryotherapy. ROP related retinal detachment * misconceptions 1. Stage 4A = benign and can wait till stage 4B

before treating. 2. Poor prognosis with total detachment. Surgery reserved for stages 4A, 4B and 5. Intravitreal triamcinolone in Stage 5

reattachment success rate. Vision restoration technology – future.

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