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RETINOPATHY OF PREMATURITY
DR. JASIR.K
REFERENCES
 Nelson text book of pediatrics 20th e
 Cloherty Manual of neonatal care 8th e
 NNF guidelines
 Rashtriya bal swasthya karyakram , guidelines for universal eye screening in
newborns inclu ding retinopathy of prematurity 2016
DEFINITION
 It is a multifactorial vasoproliferative retinal disorder that increase in incidence with
decreasing gestational age
 Approximately 65% of infants with birth weight <1250 gms and 80% of those with a
birth weight <1000 gms will develop some degree of ROP.
NORMAL DEVELOPMENT
 Retinal Vascularization begins – 16 weeks
 Angiogenesis :normally proceeds from the optic disc to periphery
 Reaching the outer rim of the retina [ora serrata] nasally by about 36 weeks and
extending temporally by 40 weeks
PATHOGENESIS
 Various theories have been proposed for the pathogenesis
1.Classical theory
2.Gap junction theory
Classical theory:
1.Hyperoxic phase:
 It occurs immediately after birth due to exposure to hyperoxic environment
 The maximum Paco2 is 35mmHg.After birth this increase to 60-80 mmHh
 Increased 02 delivery to retina results in vasoconstriction,irreversible
vasoobliteration and dissolution of the retinal capillary endothelial cells
 Hypoxic phase:
On withdrawal of the hyperoxic environment,an ischemia induced
vasoproliferative response is seen,resulting in development of ROP
RISK FACTORS
Three crucial risk factors:
 Birth weight
 Gestational age
 Prolonged oxygen exposure
Other risk factors:
 Blood transfusions
 Sepsis
 Intra Uterine Growth Retardation (IUGR)
 Failure of increase in weight
 Respiratory Distress Syndrome (RDS)
 Multiple apneic episodes
 Hypercarbia, Acidosis
 Intra Ventricular Hemorrhage (IVH)
 Anemia
 Seizures.
CLASSIFICATION
 ZONE
 EXTENT
 STAGE
 PLUS
ZONES
An i,maginary circle with
optic nerve at the center
and a radius of twice the
distance from the optic
nerve to the macula
Extends from zone 1 to the ora
serrata on the nasal side of
theeye and approximately half
the distance to the ora serrata
on the temporal side
Outer crescent shaped area
extending from zone 2 out to the
ora serrata temporally
EXTENT
Circumferential location of disease and is reported as clock hours in the appropriate zone
STAGE
STAGE I : DEMARCATION LINE
White in color
STAGE III : EXTRA RETINAL
NEOVASCULARIZATION
STAGE IV a
Macula Spared
STAGE IV b
Macula involved
STAGE IV : PARTIAL RETINAL DETACHMENT
PLUS DISEASE
 posterior venous dilation and arteriolar tortuosity of at least 2 quadrants
 Arises gradually or very rapidly.
 Due to AV shunting mainly in ridge tissue
 Severity indicator Often associated
• Iris vessel
engorgement
• Miosis
• Resistance to dilating
medications
• Vitreous haze
 Preplus disease: vascular abnormalities of the posterior pole more than
normal, less than PLUS
CLINICALLY SIGNIFICANT TERMS
Threshold ROP: CRYO ROP study
Zone I stage III with Plus
Zone II Stage III with Plus
( 5 contiguous or total 8 clock hours)
Prethreshold ROP:
Zone I Stage I, II, III with plus
Stage III without plus
Zone II Stage II and III with plus
 Plus disease has increased in importance while the extent (clock hours) of disease has
diminished
Aggressive posterior ROP [AP-ROP]
 Earlier known as ‘RUSH Disease’
 posterior location
 Rapidly evolving preplus and plus disease , neovascularization that may be
subtle or even intraretinal in nature.
 Progress to stage IV & V in 2-3 weeks without passing through characteristic
stages II and III
 Requires laser treatment more than once
TREATMENT
 Therapy for ROP is directed at treating the underlying pathogenesis by
decreasing VEGF levels
 Either by completely ablating the peripheral avascular retina that produces
the VEGF [LASER therapy/Cryotherapy] or by iactivating VEGF b binding it
after its production[anti-VEGF therapy]
 LASER[Light amplification by stimulated emission of radiation]
 Treatment modality of choice
 This prevents the progression and cause the regression of an established
ROP
CRYOTHERAPY LASER
ANAESTHESIA GA LOCAL/SEDATION
PAIN +++ +
EFFICACY DIFFUSE MORE PRECISE
RETINAL DETACHMENT POSSIBLE RARE
POST PROCEDURE
CHEMOSIS/LID EDEMA
+++ NIL
ANTIVASCULAR ENDOTHELIAL GROWTH FACTOR DRUGS
 BEVACIZUMAB[AVASTIN]
 RANIBIZUMAB
SURGICAL MANAGEMENT
Done for the treatment of retinal detachment
1. Scleral buckling
2. Vitrectomy
 Lens sparing
 With lensectomy
SCREENING
INTERNATIONAL
Birth Weight<1500gGA<32weeks
HigherBW/GAwith risks(unstablebabies)
31weeksPCAor4weeksCA,whichislater
GOVT OF INDIA 2016
BW<2000g
GA<35weeks
HigherBW/GAwith risks(unstable
babies)
31weeksPCAor4weeksCA,whichisearlier
[Infantsweighinglessthan1200gramsandperiod
ofgestation24-30weeksfirstscreeningshouldbe
doneat2-3weeksafterdelivery,andnotlaterthan
3weeks]
 NNF
BW<1750G
GA<34W
34-36 W/1750-2000G IF RISK
FACTOR
FIRST SCREENING WITHIN 4
WEEKS OF POST NATAL AGE[2-3
WEEKS IF <28W,GA<1200G
END OF SCREENING
 COMPLETE VASCULARIZATION
 VASCULARIZATION in ZONE III (till 1 DD of temporal ora) – if no previous ROP in zone I
& II
 REGRESSED ROP ( b/w 40 -44 weeks PCA)– no active disease left
 45 weeks PCA with less than pre threshold disease
THANKS

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Retinopathy of prematurity

  • 2. REFERENCES  Nelson text book of pediatrics 20th e  Cloherty Manual of neonatal care 8th e  NNF guidelines  Rashtriya bal swasthya karyakram , guidelines for universal eye screening in newborns inclu ding retinopathy of prematurity 2016
  • 3. DEFINITION  It is a multifactorial vasoproliferative retinal disorder that increase in incidence with decreasing gestational age  Approximately 65% of infants with birth weight <1250 gms and 80% of those with a birth weight <1000 gms will develop some degree of ROP.
  • 4. NORMAL DEVELOPMENT  Retinal Vascularization begins – 16 weeks  Angiogenesis :normally proceeds from the optic disc to periphery  Reaching the outer rim of the retina [ora serrata] nasally by about 36 weeks and extending temporally by 40 weeks
  • 5. PATHOGENESIS  Various theories have been proposed for the pathogenesis 1.Classical theory 2.Gap junction theory Classical theory: 1.Hyperoxic phase:  It occurs immediately after birth due to exposure to hyperoxic environment  The maximum Paco2 is 35mmHg.After birth this increase to 60-80 mmHh  Increased 02 delivery to retina results in vasoconstriction,irreversible vasoobliteration and dissolution of the retinal capillary endothelial cells
  • 6.  Hypoxic phase: On withdrawal of the hyperoxic environment,an ischemia induced vasoproliferative response is seen,resulting in development of ROP
  • 7. RISK FACTORS Three crucial risk factors:  Birth weight  Gestational age  Prolonged oxygen exposure Other risk factors:  Blood transfusions  Sepsis  Intra Uterine Growth Retardation (IUGR)  Failure of increase in weight  Respiratory Distress Syndrome (RDS)  Multiple apneic episodes  Hypercarbia, Acidosis  Intra Ventricular Hemorrhage (IVH)  Anemia  Seizures.
  • 9. ZONES An i,maginary circle with optic nerve at the center and a radius of twice the distance from the optic nerve to the macula Extends from zone 1 to the ora serrata on the nasal side of theeye and approximately half the distance to the ora serrata on the temporal side Outer crescent shaped area extending from zone 2 out to the ora serrata temporally
  • 10. EXTENT Circumferential location of disease and is reported as clock hours in the appropriate zone
  • 11. STAGE
  • 12. STAGE I : DEMARCATION LINE White in color
  • 13.
  • 14. STAGE III : EXTRA RETINAL NEOVASCULARIZATION
  • 15. STAGE IV a Macula Spared STAGE IV b Macula involved STAGE IV : PARTIAL RETINAL DETACHMENT
  • 16.
  • 17. PLUS DISEASE  posterior venous dilation and arteriolar tortuosity of at least 2 quadrants  Arises gradually or very rapidly.  Due to AV shunting mainly in ridge tissue  Severity indicator Often associated • Iris vessel engorgement • Miosis • Resistance to dilating medications • Vitreous haze
  • 18.  Preplus disease: vascular abnormalities of the posterior pole more than normal, less than PLUS
  • 19. CLINICALLY SIGNIFICANT TERMS Threshold ROP: CRYO ROP study Zone I stage III with Plus Zone II Stage III with Plus ( 5 contiguous or total 8 clock hours) Prethreshold ROP: Zone I Stage I, II, III with plus Stage III without plus Zone II Stage II and III with plus  Plus disease has increased in importance while the extent (clock hours) of disease has diminished
  • 20. Aggressive posterior ROP [AP-ROP]  Earlier known as ‘RUSH Disease’  posterior location  Rapidly evolving preplus and plus disease , neovascularization that may be subtle or even intraretinal in nature.  Progress to stage IV & V in 2-3 weeks without passing through characteristic stages II and III  Requires laser treatment more than once
  • 21. TREATMENT  Therapy for ROP is directed at treating the underlying pathogenesis by decreasing VEGF levels  Either by completely ablating the peripheral avascular retina that produces the VEGF [LASER therapy/Cryotherapy] or by iactivating VEGF b binding it after its production[anti-VEGF therapy]
  • 22.  LASER[Light amplification by stimulated emission of radiation]  Treatment modality of choice  This prevents the progression and cause the regression of an established ROP CRYOTHERAPY LASER ANAESTHESIA GA LOCAL/SEDATION PAIN +++ + EFFICACY DIFFUSE MORE PRECISE RETINAL DETACHMENT POSSIBLE RARE POST PROCEDURE CHEMOSIS/LID EDEMA +++ NIL
  • 23. ANTIVASCULAR ENDOTHELIAL GROWTH FACTOR DRUGS  BEVACIZUMAB[AVASTIN]  RANIBIZUMAB
  • 24. SURGICAL MANAGEMENT Done for the treatment of retinal detachment 1. Scleral buckling 2. Vitrectomy  Lens sparing  With lensectomy
  • 26. INTERNATIONAL Birth Weight<1500gGA<32weeks HigherBW/GAwith risks(unstablebabies) 31weeksPCAor4weeksCA,whichislater GOVT OF INDIA 2016 BW<2000g GA<35weeks HigherBW/GAwith risks(unstable babies) 31weeksPCAor4weeksCA,whichisearlier [Infantsweighinglessthan1200gramsandperiod ofgestation24-30weeksfirstscreeningshouldbe doneat2-3weeksafterdelivery,andnotlaterthan 3weeks]  NNF BW<1750G GA<34W 34-36 W/1750-2000G IF RISK FACTOR FIRST SCREENING WITHIN 4 WEEKS OF POST NATAL AGE[2-3 WEEKS IF <28W,GA<1200G
  • 27. END OF SCREENING  COMPLETE VASCULARIZATION  VASCULARIZATION in ZONE III (till 1 DD of temporal ora) – if no previous ROP in zone I & II  REGRESSED ROP ( b/w 40 -44 weeks PCA)– no active disease left  45 weeks PCA with less than pre threshold disease