Retinopathy of Prematurity Geoffrey T. Tufty, MD Sanford Clinic Ophthalmology

 Failure of the peripheral retina to vascularize Definition

ROP

 To understand the etiology of ROP  To understand the anatomy of the eye  To understand the stages/severity of ROP  To understand the zones/location of ROP  To understand the screening guidelines for ROP  To understand the treatment options for ROP  To understand the long term affects of ROP Objectives

ROP-historical  Terry  Leading cause of childhood blindness  Retro lental fibroplasia  4 million premature babies born each year  14,000 have ROP  90% of those are mild cases and resolve

Retro lental fibroplasia  Fibrous mass behind the lens is detached retina

Risk factors  Low birth weight  Early gestational age  Anemia  Respiratory distress  Poor weight gain  Blood transfusion  Multiples  Intraventricular hemorrhage  Low vitamin E  ? light exposure

When to Screen??  BW <1500 grams  <32 weeks  >32 weeks with a difficult clinical course  4 weeks of age or 31 weeks which ever is first

Normal Retinal Development  Nasal(32 weeks) then temporal (40 weeks)  VEGF  Stimulates normal development-reduced by hyperoxia-cessation of growth  Hypoxic retina upregulates VEGF and thus ROP  Immature retina-hyperoxia- produces VEGF and neo grows- ROP

Anatomy of the Eye  Tear film  Cornea  Iris  Lens  Vitreous  Retina-macula  Optic nerve

Retina  Normal retina fully developed

Tools of the trade  Dilating drops-cyclomydril  Anesthetic drops

Tools of the trade

Indirect Ophthalmoscope

Good help  You need to swaddle the baby and take your time but be efficient.  Get a good look.  Using the “force” does not work

Ret-Cam  Used for retinal photos to detect ROP and other forms of retinal pathology

Let’s set the Stage  Stages are the severity of the ROP  The higher the stage the worse the disease

Stage 1 ROP  A demarcation line between normal retinal vasculature and avascular retina

Stage 1

Stage 1 ROP

Stage 2 ROP  The demarcation line between vascular retina has increased volume

Stage 2 ROP  The demarcation line has volume to it.

Stage 3 ROP  Neovascularization on the dividing line between vascular and avascular retina

Stage 3 ROP  Continuous neovascularization  Popcorn  Sausage

Stage 3 ROP

Stage 4A ROP  Extra foveal retinal detachment

Stage 4A ROP  Peripheral retina is being pulled off of the attachments-traction

Stage 4B ROP  Traction and detachment of the fovea-center vision

Stage 5 ROP  Bad- total tractional retinal detachment

Stage 5 ROP  Poor overall visual outcomes despite aggressive treatment

Plus Disease  Increase in venous and arteriolar dilation  Due to blood shunting  Indicates severity

No Zoning Out

Get into the Zone  Location of ROP in the eye  Higher the zone-the better

Zone I A circle with the optic nerve as the center and extends twice the distance from the optic disc to the macula

Zone II  From zone I to the nasal peripheral retina

Zone III  Temporal retinal crescent

Follow up Exams  1 week  Early zone II  Zone I stage 1 or 2  progression  2 weeks  Zone II immature  Zone II stage 1 or 2  Zone II no ROP  3 weeks  Zone III

When to treat??  Zone 1 ROP any stage with plus disease  Zone I stage 3 no plus  Zone II stage 2 or 3 with plus  Should be treated within 72 hours

Treatment  In the NICU?  In the OR?  Sit down with family and cover everything- layman’s terms

Treatment  Cryotherapy

Cryotherapy  Cryo ROP study in 1986 reduced unfavorable outcomes from  Eyes are red and sore

Cryotherapy

Laser  Portable  Learning curve  Less painful

Laser

 ROP may get worse before it gets better

Avastin  Anti VEGF therapy

Injection  Dosage- half the adult dose  30 g needle  Betadine  Pars plana

Avastin  Risks of injection  Infection  Cataract  Normal vascularization  What is the correct dosage  Frequency  Developing brain and organs  Long term follow up

Vitrectomy  Surgical reattachment of the retina  40% of stage 5  2/3rds of stage 4  Visual outcomes are generally poor

Risk Factors of Treatment  Blindness  Retinal detachment-traction  Strabismus  Myopia  Anisometropia  Cataract  Amblyopia  Low vision

Aids  Glasses  Low vision aids  Magnifiers  Computers/tablets  CCTV  Large print books  Good lighting  Low vision teachers  School for the blind  Good communication with parents and teachers

Questions??? When it comes to ROP there is no zoning out. If you do, you will set the stage for disaster Thank you!!!