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Syn: Retrolental Fibroplasia

               

In some countries during and after World War II, the retinopathy of  prematurity was the leading cause of  blindness in infants.

This bilateral, iatrogenic ocular disorder is almost restricted to premature infants and is caused by the administration of high concentration of oxygen.

When a premature infant is exposed to excessive amounts of oxygen, as in an incubator, the developing retinal blood vessels become obliterated and the peripheral retina, which is normally avascular until the end of fetal life, does not vascularize.

The more mature the retina, the less the vaso-obliterative effect of hyperoxia.

When the infant eventually returns to ambient air, an intense proliferation of vascular endothelium and glial cells begins at the junction of the avascular and vascularized portions of the retina.

This becomes apparent 5  to 10 weeks after removal from the incubator, and, is thought to result from the liberation of an angiogenic factor produced by the avascular peripheral retina that is now ischemic.

This angiogenic factor is also thought to account for the neovascularization of the iris that sometimes accompanies the retinal angiogenesis in the retinopathy of prematurity.

In approximately 25% of cases the retinopathy progresses to a cicatricial phase, characterized by retinal detachment and a retrolental, fibrovascular mass.    Normal histology and diseases of the retina

           

Retinopathy of prematurity (retrolental fibroplasia): old and new facts.Helv Paediatr Acta. 1982;37(5):413-20

Retinopathy of prematurity (ROP) is found in 10% of patients in neonatological units. Most often, there is spontaneous restitution. Rarely, severe degrees develop (retrolental fibroplasia); occasionally, infants become blind even nowadays. The etiological role of oxygen is undisputed. Additional factors, however, are also found with significant frequency in the history of infants with ROP. These are elevated pCO2-values, an acidotic pH, and blood transfusions. These three parameters lead to increased availability of oxygen for the tissue. Data from animal experiments confirm this effect. The statistical significance of these factors is clear, whereas their clinical significance has not yet been proved. Probably, the most effective prophylactic measure for preventing severe degrees of ROP is the repeated examination of newborns presenting defined risks by especially trained ophthalmologists.

Retinopathy of prematurity and risk factors: a prospective cohort study.BMC Pediatr. 2005 Jun 28;5(1):18

BACKGROUND: Increased survival of extremely low birth infants due to advances in antenatal and neonatal care has resulted in a population of infants at high risk of developing retinopathy of prematurity (ROP). Therapeutic interventions include the use of antenatal and postnatal steroids however, their effects on the severity of ROP is in dispute. In addition, it has not been investigated whether severe ROP is due to therapeutic interventions or due to the severity of illness. The aim of the present study was to assess the association between the incidence of severe retinopathy of prematurity (greater than stage 2 - International classification of ROP) and mechanical ventilation, oxygen therapy, gestational age, antenatal and postnatal steroids in extremely low birth weight infants. METHODS: Neonates admitted to the neonatal intensive care unit in Lansing, Michigan, during 1993-2000 were followed to determine factors influencing the development of severe retinopathy of prematurity. Ophthalmologic examinations were started at 6 weeks and followed until resolution. We used logistic regression to estimate the relative risk (odds ratio) associated with risk factors of ROP. RESULTS: Of the neonates with <or= 1500 g birth weight, admitted to the neonatal intensive care unit, 85% (616/725) survived. Severe retinopathy of prematurity was detected in 7.8% of 576 neonates who had eye examinations. Neonates of lower gestational age (<or= 25 weeks and 26-28 weeks) had an increased odds ratio of 8.49 and 3.19 for the development of severe retinopathy of prematurity, respectively, compared to those 29 weeks and older. Late postnatal steroid treatment starting after 3 weeks of life showed 2.9-fold increased odds ratio, in particular administration for two weeks and more (OR: 4.09, 95% CI: 1.52-11.03). With increasing antenatal steroids courses the risk of severe retinopathy of prematurity decreased, however, it was not significant. Lower gestational age, dependence on ventilation, and use of postnatal steroids were intertwined. Simultaneous presence of these factors seems to indicate severe disease status. CONCLUSION: Prolonged and late postnatal steroids treatment in very low birth weight infants may pose an increased risk for the development of severe retinopathy of prematurity; however, use of postnatal steroids may also be a marker for severity of illness. Further studies need to focus on biologic markers in the pathogenesis of retinopathy of prematurity and to better understand the influence of therapies.

Risk factors of retinopathy of prematurity in infants 32 to 36 weeks gestational age.Z Geburtshilfe Neonatol. 2003 Jan-Feb;207(1):24-8.

INTRODUCTION: In our study we determined possible risk factors for retinopathy of prematurity (ROP) in infants 32 to 36 completed weeks of gestational age based on a regional German neonatal database. We examined especially whether or not oxygen therapy over more than 3 days is related to a higher risk of ROP. MATERIALS: We identified 7172 ophthalmologically examined infants, 32 to 36 completed weeks gestational age, born from 1990 to 1996. ROP was diagnosed in 195 (2.7 %). We examined the following variables as risk factors for ROP in infants receiving oxygen for less than 4 days: gestational age, sex, blood pH of 7.0 or less, body temperature of 36 degrees C or less, phototherapy, blood pO2 of 35 mm Hg or less, small-for-gestational age, sepsis, ventilation after birth, and blood transfusion. RESULTS: Sex, blood pH of 7.0 or less, blood pO2 of 35 mm Hg or less, sepsis, phototherapy, and small-for-gestational age were not associated with a significant risk of ROP. A gestational age of 32 weeks compared to a gestational age of 36 weeks was associated with an increased risk of ROP (odds ratio, 2.95; 95 % confidence interval, 2.18 to 4.01). Ventilation after birth (adjusted OR, 2.29; 95 % CI, 1.70 to 3.15) and blood transfusion (adjusted OR, 5.28; 95 % CI, 3.80 to 7.23) increased the risk of ROP regardless of gestational age. Oxygen therapy for more than 3 days was not associated with an increased risk of ROP (OR, 1.06; 95 % CI, 0.67 to 1.70). CONCLUSION: In neonates delivered between 32 and 36 weeks of gestation, the duration of oxygen supplementation should not necessitate an ophthalmological examination. A vigorous restriction of blood transfusions could reduce the incidence of ROP.

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