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Retinopathy of
prematurity
Dr. Tareq rahman ,
Resident Phase B , Neonatology,
Bangabandhu Sheikh Mujib Medical University( BSMMU).
Definition
Retinopathy of prematurity (ROP), formerly
known as retrolental fibroplasia because of its
end-stage appearance, is a developmental
vascular proliferative disorder that occurs in
the retina of preterm infants with incomplete
retinal vascularization.
Epidemiology
• Most studies report ROP incidences that are
about 60% for babies less than 1500 g (Zin and
Gole, 2013)
• The worldwide prevalence of blindness due to
ROP is approximately 50,000.
• In India the incidence of ROP is between 38
and 51.9% in low-birth-weight infants.
BSMMU experience :
- 1st case was reported in 2004
- Between 2013 to 2015 , 446 babies were
screened where 82 ( 19%) were diagnosed as
ROP and APROP 6% .
Embryology of retinal blood vessels
• Retinal vascularization begins
at 15 to 18 weeks’ gestation.
• Retinal blood vessels extend
out from the optic disc and
grow peripherally.
• Vascularization in the nasal
retina is complete at
approximately 32 – 34 weeks
and temporal retina by 40
weeks.
• Insulin-like growth factor-1
(IGF-1) supports normal retinal
vascular growth and interacts
with VEGF under influence of
hypoxic state in utero.
Pathogenesis
• Premature delivery interrupts normal vascular growth
• Phase 1 : delayed retinal vascularisation (birth-31/32
weeks)
• Developing retina exposed to hyperoxic environment
(ambient andsupplemental)
• Reduces angiogenic factors delaying retinal
vascularisation
• Phase 2 : neovascularisation
• Neuroretina continues to develop causing hypoxia
with overproduction of angiogenic factors especially
VEGF causes uncontrolled retinal blood vessel growth.
Risk factors for ROP
• Three crucial risk factors:
– Low Birth weight specially less than
1500 gm
– Prematurity specially 32 weeks of GA
– Unrestricted oxygen
supplementation
– Other risk factors:
– Anemia
– Blood transfusions
– Intra Uterine Growth Retardation
(IUGR)
– Failure of increase in weight
– Respiratory Distress Syndrome (RDS)
– Fluctuations in Sa O2
– Multiple apneic episodes
– Hypercarbia, Acidosis
– Sepsis
– Intra Ventricular Hemorrhage (IVH)
– Low level of the serum IGF-I in early
postnatal period
– Raised level of erythropoietin.
– Necrotising enterocolitis.
– Bronchopulmonary dysplasia
• Our baby is preterm (
30 weeks) and low
birth weight ( 1500
gm)
• He got MV , CPAP
support
• He had sepsis ,
acidosis and h/o
multiple apneic
episodes.
• Had h/o blood
transfusion due to
anaemia.
International Classification for
Retinopathy of Prematurity (ICROP)
• 5 parameters:
- Zone (1-3)
– Stage (1-5)
– Clock hours: The extent of the developing
vasculature that is involved quantified from 1-
12 clock hours.
– Plus disease (Plus disease is a primary factor in
treatment decision).
– Pre-plus disease
• Aggressive posterior
ROP (AP-ROP) is a
severe form of ROP that
rapidly causes total
retinal detachment
without going through
the normal stages of
ROP
Anterior-posterior location
Zone
• Zone I is the posterior
retina within 60 degrees
of the optic disc
• Zone II is a concentric
circle extending from
Zone 1 to the nasal ora
serrata
• Zone III is the remaining
temporal retina
Stage 1
Stage 2
Demarcation line with elevation between
vascular and avascular area
Stage 3
Stage 4
STAGE 5 : TOTAL RETINAL DETACHMENT
Stage 5
Stage 5. Total retinal detachment
• Plus disease:
Presence of dilated and tortuous vessels of
the posterior pole present in two or more
quadrants
• Preplus disease:
Abnormal vascular dilation and
tortuosity that is insufficient for
diagnosis of plus disease present in two
or more quadrants
Threshold ROP :
ROP is defined as five contiguous clock hours
or eight total clock hours of stage 3 and plus
disease in zone 1 or 2.
• Prethreshold ROP — Prethreshold ROP is
defined as one of the following –
 ROP at any stage less than threshold in zone I
 Stage 2 and plus disease in zone II
 Stage 3 without plus disease in zone II
 Stage 3 with plus disease in zone II but with
fewer clock hours of stage 3 than required to meet
threshold
• High-risk prethreshold ROP includes –
 Any stage ROP with plus disease in zone I
 Stage 3 ROP without plus disease in zone I
 Stage 2 or 3 ROP with plus disease in zone II
Long Term Complications of ROP
Retinal Dragging
and Folds
Others:
Acute angle closure glaucoma
Late onset Retinal Detachment( 22%)
Significant myopia (80%)
Anisometropia
Amblyopia
Strabismus
Criteria for screening
• United States: infants with a gestational age
of ≤30 weeks or birth weight of <1500 g and
selected infants with a gestational age of >30
weeks and an unstable clinical course.
• United Kingdom: infants with a gestational
age of ≤31 weeks or birth weight of ≤1500 g
• Canada: infants with a gestational age of ≤30
weeks, or birth weight of ≤1250 g
M. Elizabeth Hartnett et al , N Engl J Med 2012;367:2515-26.
ROP screening-current UK guideline
Which infant should be screened?
All infants born < 32 weeks of gestation or <1500g
When should the first screening examination be performed?
Gestational age at birth (weeks) Postnatal age at first ROP examination
(weeks)
23 7
24 6
25 5
26 4
27 4
28 4
29 4
30 4
31 4
ROP screening – in BSMMU
All neonates weighing <2000 g and/or with a gestation <35
weeks admitted to the NICU are being routinely screened for
ROP.
Neonates with birth weight ≥ 2000 g or gestational age more
than 35 weeks are being screened if they have an unstable
neonatal including those requiring cardiorespiratory support for
prolonged period.
ROP screening – in BSMMU
At a 4 weeks of postnatal age for infants born at
or more than 32 weeks of gestation or birth
weight ≥1200 g and at a 3 weeks of postnatal age
for infants less than 32 weeks of gestation or
birth weight <1200 g.
Diagnosis
• Ophthalmologic examination by an expert examiner usually
confirms the diagnosis.
• Binocular indirect ophthalmoscopy (BIO) is generally used.
• Newer digital camera technology demonstrated 100%
sensitivity in detecting ROP requiring treatment. But it does
not permit adequate assessment of retinal periphery.
Ophthalmoscopy
• Pupillary Dilation : 2.5% phenyl ephrine + 0.5%
Tropicamide – 3 times , 10 mins apart 30 mins
before exam
• Speculum
• INDIRECT ophthalmoscope
• PLUS DISEASE to be looked for before
speculum and scleral depression
Treatment
• Indications for treatment - Retinal ablative therapy is indicated
for eyes with threshold retinopathy of prematurity (ROP) and
eyes with high-risk prethreshold retinopathy of prematurity
(ROP) based on clinical findings.
• High-risk prethreshold ROP includes –
 Zone I: any stage ROP with plus disease
 Zone I: stage 3 ROP without plus disease
 Zone II: stage 2 or 3 ROP with plus disease
• When treatment should start?
Once a treatment decision has been made, treatment should be
performed within 48-72 hours.
Treatment
• Treatment modalities –
 Cryotherapy
 Laser photocoagulation – Standard treatment
 Antivascular endothelial growth factor (VEGF)
monoclonal antibody (Bevacizumab)
- stage 3 and plus disease in zone I.
 Surgery - vitrectomy, Scleral buckling.
- Results are best when done before the fovea
has detached.
Outcome of treatment
• Treatment of acute ROP generally results in normal or near
normal anatomy of macula and posterior retina.
• Treatment fails in small proportion of cases and retinal
detachment ensues.
• Prompt vitreoretinal surgery may be needed to preserve
vision.
• Severe visual impairment continue to occur in some infants.
Follow - Up
Care in NICU
• Maintain SpO2 88 – 93 %
• Avoid flactuation of SpO2 while on O2
supplementation.
• Keep Hb level not less than 10 gm/dl
• Ensure adequate weight gain.
Prognosis
Spontaneous regression:
• Stage I - and stage-II: 90%.
• Stage III+ disease : 50%.
• PREVENTION - Interventions to prevent or limit the
progression of retinopathy of prematurity (ROP) have been
unsuccessful, although further evaluation may be needed.
Because oxidant injury contributes to ROP, antioxidant
therapies, such as vitamin E, D-penicillamine, and limited
exposure to light, have been tested.
Concerning matter ?
• Retinopathy of prematurity has become a leading
but preventable cause of childhood blindness
worldwide.(M. Elizabeth Hartnett et al, 2012)
• ROP occurs primarily in infants of low birth
weight and low gestational age at birth.
• Infants with ROP having higher risk for developing
certain eye problems later in life
- retinal detachment, myopia, strabismus,
amblyopia, glaucoma.
Reference
• www.uptodate.com
• www.medscape.com
• American Academy of Ophthalmology
THANK YOU

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Retinopathy of prematurity by dr. tareq rahman

  • 1. Retinopathy of prematurity Dr. Tareq rahman , Resident Phase B , Neonatology, Bangabandhu Sheikh Mujib Medical University( BSMMU).
  • 2. Definition Retinopathy of prematurity (ROP), formerly known as retrolental fibroplasia because of its end-stage appearance, is a developmental vascular proliferative disorder that occurs in the retina of preterm infants with incomplete retinal vascularization.
  • 3. Epidemiology • Most studies report ROP incidences that are about 60% for babies less than 1500 g (Zin and Gole, 2013) • The worldwide prevalence of blindness due to ROP is approximately 50,000. • In India the incidence of ROP is between 38 and 51.9% in low-birth-weight infants.
  • 4. BSMMU experience : - 1st case was reported in 2004 - Between 2013 to 2015 , 446 babies were screened where 82 ( 19%) were diagnosed as ROP and APROP 6% .
  • 5. Embryology of retinal blood vessels • Retinal vascularization begins at 15 to 18 weeks’ gestation. • Retinal blood vessels extend out from the optic disc and grow peripherally. • Vascularization in the nasal retina is complete at approximately 32 – 34 weeks and temporal retina by 40 weeks. • Insulin-like growth factor-1 (IGF-1) supports normal retinal vascular growth and interacts with VEGF under influence of hypoxic state in utero.
  • 7. • Premature delivery interrupts normal vascular growth • Phase 1 : delayed retinal vascularisation (birth-31/32 weeks) • Developing retina exposed to hyperoxic environment (ambient andsupplemental) • Reduces angiogenic factors delaying retinal vascularisation • Phase 2 : neovascularisation • Neuroretina continues to develop causing hypoxia with overproduction of angiogenic factors especially VEGF causes uncontrolled retinal blood vessel growth.
  • 8.
  • 9. Risk factors for ROP • Three crucial risk factors: – Low Birth weight specially less than 1500 gm – Prematurity specially 32 weeks of GA – Unrestricted oxygen supplementation – Other risk factors: – Anemia – Blood transfusions – Intra Uterine Growth Retardation (IUGR) – Failure of increase in weight – Respiratory Distress Syndrome (RDS) – Fluctuations in Sa O2 – Multiple apneic episodes – Hypercarbia, Acidosis – Sepsis – Intra Ventricular Hemorrhage (IVH) – Low level of the serum IGF-I in early postnatal period – Raised level of erythropoietin. – Necrotising enterocolitis. – Bronchopulmonary dysplasia • Our baby is preterm ( 30 weeks) and low birth weight ( 1500 gm) • He got MV , CPAP support • He had sepsis , acidosis and h/o multiple apneic episodes. • Had h/o blood transfusion due to anaemia.
  • 10. International Classification for Retinopathy of Prematurity (ICROP) • 5 parameters: - Zone (1-3) – Stage (1-5) – Clock hours: The extent of the developing vasculature that is involved quantified from 1- 12 clock hours. – Plus disease (Plus disease is a primary factor in treatment decision). – Pre-plus disease
  • 11. • Aggressive posterior ROP (AP-ROP) is a severe form of ROP that rapidly causes total retinal detachment without going through the normal stages of ROP
  • 12. Anterior-posterior location Zone • Zone I is the posterior retina within 60 degrees of the optic disc • Zone II is a concentric circle extending from Zone 1 to the nasal ora serrata • Zone III is the remaining temporal retina
  • 14. Stage 2 Demarcation line with elevation between vascular and avascular area
  • 17.
  • 18. STAGE 5 : TOTAL RETINAL DETACHMENT
  • 19. Stage 5 Stage 5. Total retinal detachment
  • 20. • Plus disease: Presence of dilated and tortuous vessels of the posterior pole present in two or more quadrants
  • 21. • Preplus disease: Abnormal vascular dilation and tortuosity that is insufficient for diagnosis of plus disease present in two or more quadrants
  • 22. Threshold ROP : ROP is defined as five contiguous clock hours or eight total clock hours of stage 3 and plus disease in zone 1 or 2.
  • 23. • Prethreshold ROP — Prethreshold ROP is defined as one of the following –  ROP at any stage less than threshold in zone I  Stage 2 and plus disease in zone II  Stage 3 without plus disease in zone II  Stage 3 with plus disease in zone II but with fewer clock hours of stage 3 than required to meet threshold
  • 24. • High-risk prethreshold ROP includes –  Any stage ROP with plus disease in zone I  Stage 3 ROP without plus disease in zone I  Stage 2 or 3 ROP with plus disease in zone II
  • 25. Long Term Complications of ROP Retinal Dragging and Folds Others: Acute angle closure glaucoma Late onset Retinal Detachment( 22%) Significant myopia (80%) Anisometropia Amblyopia Strabismus
  • 26. Criteria for screening • United States: infants with a gestational age of ≤30 weeks or birth weight of <1500 g and selected infants with a gestational age of >30 weeks and an unstable clinical course. • United Kingdom: infants with a gestational age of ≤31 weeks or birth weight of ≤1500 g • Canada: infants with a gestational age of ≤30 weeks, or birth weight of ≤1250 g M. Elizabeth Hartnett et al , N Engl J Med 2012;367:2515-26.
  • 27. ROP screening-current UK guideline Which infant should be screened? All infants born < 32 weeks of gestation or <1500g When should the first screening examination be performed? Gestational age at birth (weeks) Postnatal age at first ROP examination (weeks) 23 7 24 6 25 5 26 4 27 4 28 4 29 4 30 4 31 4
  • 28. ROP screening – in BSMMU All neonates weighing <2000 g and/or with a gestation <35 weeks admitted to the NICU are being routinely screened for ROP. Neonates with birth weight ≥ 2000 g or gestational age more than 35 weeks are being screened if they have an unstable neonatal including those requiring cardiorespiratory support for prolonged period.
  • 29. ROP screening – in BSMMU At a 4 weeks of postnatal age for infants born at or more than 32 weeks of gestation or birth weight ≥1200 g and at a 3 weeks of postnatal age for infants less than 32 weeks of gestation or birth weight <1200 g.
  • 30. Diagnosis • Ophthalmologic examination by an expert examiner usually confirms the diagnosis. • Binocular indirect ophthalmoscopy (BIO) is generally used. • Newer digital camera technology demonstrated 100% sensitivity in detecting ROP requiring treatment. But it does not permit adequate assessment of retinal periphery.
  • 31. Ophthalmoscopy • Pupillary Dilation : 2.5% phenyl ephrine + 0.5% Tropicamide – 3 times , 10 mins apart 30 mins before exam • Speculum • INDIRECT ophthalmoscope • PLUS DISEASE to be looked for before speculum and scleral depression
  • 32. Treatment • Indications for treatment - Retinal ablative therapy is indicated for eyes with threshold retinopathy of prematurity (ROP) and eyes with high-risk prethreshold retinopathy of prematurity (ROP) based on clinical findings. • High-risk prethreshold ROP includes –  Zone I: any stage ROP with plus disease  Zone I: stage 3 ROP without plus disease  Zone II: stage 2 or 3 ROP with plus disease • When treatment should start? Once a treatment decision has been made, treatment should be performed within 48-72 hours.
  • 34. • Treatment modalities –  Cryotherapy  Laser photocoagulation – Standard treatment  Antivascular endothelial growth factor (VEGF) monoclonal antibody (Bevacizumab) - stage 3 and plus disease in zone I.  Surgery - vitrectomy, Scleral buckling. - Results are best when done before the fovea has detached.
  • 35. Outcome of treatment • Treatment of acute ROP generally results in normal or near normal anatomy of macula and posterior retina. • Treatment fails in small proportion of cases and retinal detachment ensues. • Prompt vitreoretinal surgery may be needed to preserve vision. • Severe visual impairment continue to occur in some infants.
  • 37. Care in NICU • Maintain SpO2 88 – 93 % • Avoid flactuation of SpO2 while on O2 supplementation. • Keep Hb level not less than 10 gm/dl • Ensure adequate weight gain.
  • 38. Prognosis Spontaneous regression: • Stage I - and stage-II: 90%. • Stage III+ disease : 50%.
  • 39. • PREVENTION - Interventions to prevent or limit the progression of retinopathy of prematurity (ROP) have been unsuccessful, although further evaluation may be needed. Because oxidant injury contributes to ROP, antioxidant therapies, such as vitamin E, D-penicillamine, and limited exposure to light, have been tested.
  • 40. Concerning matter ? • Retinopathy of prematurity has become a leading but preventable cause of childhood blindness worldwide.(M. Elizabeth Hartnett et al, 2012) • ROP occurs primarily in infants of low birth weight and low gestational age at birth. • Infants with ROP having higher risk for developing certain eye problems later in life - retinal detachment, myopia, strabismus, amblyopia, glaucoma.
  • 41. Reference • www.uptodate.com • www.medscape.com • American Academy of Ophthalmology