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Retrolental fibroplasia and blood transfusion in very low-birth-weight infants.

The relative contribution of transfusions of adult blood to the development of retrolental fibroplasia (RLF) in very low-birth-weight infants was examined. Five years of experience with the expanded use of replacement and exchange transfusions in 90 infants with birth weight less than or equal to 1,250 gm was reviewed. Twenty percent of the infants developed cicatricial RLF. Exchange transfusion was not related to development of cicatricial RLF. The incidence of RLF in infants receiving greater or equal to 130 ml of packed red blood cells per kilogram of birth weight as replacement blood transfusion (RBT) was significantly higher (42.9%) than that in infants receiving 61 to 131 ml of packed red blood cells per kilogram (15.4%) and infants receiving less than or equal to 60 ml of packed red blood cells per kilogram (0%), P less than .001. The need for RBT, however, was strongly correlated (r = .85, P less than .001) with increasing duration of O2 therapy. When O2 therapy was controlled for, the association between RBT and RLF did not achieve statistical significance (P = .07). The association between RBT and RLF remained significant when adjusted for duration of therapy in fractional inspired oxygen (FIO2) greater than 0.4. Further detailed studies of large numbers of susceptible infants are warranted to assess the magnitude of the contribution of transfusions of adult blood to development of RLF.

Exchange Transfusion, Whole Blood↗

Scleral buckling in 2 1/2- to 11-month-old premature infants with retinal detachment associated with acute retrolental fibroplasia.

The authors' experience over the past five years in the cryosurgical management of retinal detachment in ten eyes of eight premature infants with retrolental fibroplasia, whose ages at the time of detachment surgery ranged from 2 1/2 months to 11 months with an average of 5 1/2 months, is reported. Postoperative follow-up, ranging from eight to 61 months, has revealed that scleral buckling was successful in six eyes (60%). The ERG responses are slow to become established postoperatively, and VER testing is of questionable value in these cases.

Age Factors↗

Retinal detachment in young premature infants with acute retrolental fibroplasia. Thirty-two new cases.

Experience with scleral buckling for exudative and tractional retinal detachments in very young premature infants who have acute retrolental fibroplasia (RLF) is reported. In this series, surgery was successful in 24 of 32 eyes (75%) of 23 infants, (age at surgery ranged from 1.75 months to 6 months, average age of 3 months). We attribute the 15% improvement in the success rate over our 1979 series in part to closer screening with emphasis on the examination at age 8 weeks, to earlier referral for treatment, and to our observation that these fragile eyes can withstand reoperation if buckle revision is necessary to combat persistent retinal traction. We encountered no serious complications either at surgery or immediately after surgery. We stress that (1) because of the high incidence of spontaneous remission, buckling surgery should not be performed until the detachment has progressed well posterior to the equator and remission is obviously unlikely (Grade IV or V); (2) excessive cryotherapy and undue surgical trauma should be avoided; and (3) because scleral erosion is certain as the eyes grow, the encircling band should be transected six months to one year after surgery.

Female↗

Retrolental fibroplasia. Experience over two decades in one institution.

We examined 1,849 consecutively admitted premature infants weighing 2000 gm or less at birth for retrolental fibroplasia (RLF). Proliferative RLF was diagnosed frequently in low birth weight infants who were more likely to survive with modern neonatal intensive care. Cicatricial RLF was more likely to result from proliferative RLF and to be more severe in lower birth weight infants, but most affected eyes retained useful vision. Comparison of cases diagnosed over the 20 years of this study suggest that cicatricial RLF in recent years is less likely to result in severe visual disability. Improving survival rates for lower birth weight infants mandate continued surveillance for RLF and study of improved oxygen monitoring techniques.

Female↗

Scleral buckling in 2 1/2 to 11-month-old premature infants with retinal detachment associated with acute retrolental fibroplasia.

The authors report their experience over the past 5 years in the cryosurgical management of retinal detachment in 10 eyes of 8 premature infants with retrolental fibroplasia, whose ages at the time of detachment surgery ranged from 2 1/2 months to 11 months, with an average of 5 1/2 months. Postoperative follow-up, ranging from 8 to 61 months, has revealed that scleral buckling was successful in 6 eyes (60%). The authors have found that ERG responses are slow to become established postoperatively, and have concluded that VER testing is of questionable value in these cases.

Age Factors↗

Retrolental fibroplasia or congenital encephalo-ophthalmic dysplasia?

Congenital encephalo-ophthalmic dysplasia (CEOD) is described in 2 cases. The eye signs, clinically identical to the cicatricial stage of retrolental fibroplasia (RLF), are combined with brain maldevelopment and occurred without oxygen treatment. RLF in a strict sense could be reserved for oxygen-induced retinopathy in prematures.

Abnormalities, Multiple↗

[Retinopathy of prematurity (retrolental fibroplasia): old and new facts].

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.

Animals↗

Acute retrolental fibroplasia.

Three hundred eighty-four premature infants were examined by indirect ophthalmoscopy in a period of 38 months. Sixty-eight were found to have some degree of retrolental fibroplasia. Most pathologic changes resolved spontaneously to normal or near normal. A classification was formulated, based on peripheral vascular changes, to enable observers to interpret and quantitate the amount of disease present. The role of the ophtalmologist in the perinatal intensive care nursery is not to dictate the amount of oxygen administration during the acute phase of respiratory distress syndrome but to monitor peripheral fundus and posterior vascular changes of infants of low birth weight, or who have received oxygen, or both, at a time that is not detrimental to the health of the infant. To evaluate the possible indications and efficacy of surgical intervention, an understanding of the natural course of the disease process is mandatory.

Acute Disease↗

Optimal timing of examination for acute retrolental fibroplasia.

The records of low birth weight infants who had ophthalmic examination during the first year of life were studied to determine the optimal age at which to screen for retrolental fibroplasia. Fifty-nine cases of RLF were detected in a total of approximately 175 examinations for the birth weight category under 1700 gm during a five-year period. Although about 25% of the initial examinations were performed prior to age 5 weeks, only two cases of RLF were found in that age range. Nine patients found to have a normal peripheral fundus at initial examination, later developed RLF. Since examinations performed during the fourth fortnight of life were 85% positive for RLF, and since retinal detachment in this condition rarely occurs before age 8 weeks, the present series of cases suggests that the optimal time for a single screening examination for RLF is between 7 and 9 weeks of age.

Age Factors↗

Risk factors associated with retrolental fibroplasia.

The eyes of infants who were premature or had exposure to increased ambient oxygen from 1979 to 1981 were examined. Of 1012 neonates, 19 were found to have acute retrolental fibroplasia (RLF) grade III or worse in at least one eye. Sixteen also had chronic lung disease (CLD), and when compared to 25 control patients who had CLD but not grade III or more RLF, they were found to consistently have lower blood CO2 tensions (PCO2), lower pH values, higher inspired oxygen concentrations (FIO2S) and a higher incidence of seizure disorders (100% vs. 48%). We could not show that an elevated PCO2 increased the risk for developing RLF. Infants with either chronic lung disease or chronic lung disease and seizures had a high risk for developing RLF grade III (39% and 57%, respectively).

Carbon Dioxide↗

Retrolental fibroplasia: further clinical evidence and ultrastructural support for efficacy of vitamin E in the preterm infant.

To further evaluate the efficacy of oral vitamin E in preventing the development of severe retrolental fibroplasia (RLF) in very low-birth-weight infants, 100 infants treated with 100 mg/kg/d of vitamin E (dl-alpha-tocopheryl acetate) were compared with 75 infants treated with 5 mg/kg/d of vitamin E (dl-alpha-tocopherol) in the same nursery during the previous year. All 175 infants weighed less than or equal to 1,500 g at birth and required supplemental oxygen. A total of 120 infants (69 treatment; 51 control) survived greater than or equal to 10 weeks. Multivariate analysis of the control population identified five risk factors (P less than or equal to .10): gestational age, level and duration of oxygen administration, intraventricular hemorrhage, sepsis, and birth weight. When multivariate analysis was applied to both control and treatment groups, the severity of RLF was found to be significantly reduced in infants given the treatment dose of vitamin E (P = .003). Ultrastructural analyses of 58 pairs of whole-eye donations from high-risk infants surviving less than 10 weeks suggest that the initial morphologic event is gap junction increases between the plasma membranes of adjacent spindle cells of the van-guard retina. Such extensively gap junction-linked spindle cells are apparently removed from the vasoformative process as early as 4 days of life, forming a barrier to further normal vascular development and triggering retinal and vitreal neovascularizations approximately 8 weeks later. These events are maximally suppressed by elevated plasma vitamin E levels in infants greater than or equal to 27 weeks gestational age.

Cell Membrane↗

Increased morbidity in low-birth-weight survivors with severe retrolental fibroplasia.

Fourteen (2.2%) of 645 survivors with birth weight less than or equal to 1500 gm cared for in the Special Care Nursery between 1975 and 1981 were identified as having stage III-IV retrolental fibroplasia (RLF) in one or both eyes. These 14 survivors and 14 controls matched for birth weight have been observed for 2 to 7 years. There were no differences between survivors with RLF and controls in birth weight, gestation, multiple neonatal risk factors, or Hollingshead socioeconomic status. The only significant difference was in prolonged O2 requirements (greater than 2 weeks) (13 of 14 patients vs 7 of 14 control infants (P less than 0.025) and prolonged assisted ventilation requirements (21 +/- 15 for RLF vs 6 +/- 10 days for control infants, P less than 0.05). Follow-up assessments showed that the RLF survivors had a significantly higher incidence of neurologic abnormality, lower developmental quotients, increased requirements for special education, increased number of hospitalizations for illness, and more maternal stress as observed in the mother's adapted Life Experience Survey. We conclude that low-birth-weight infants with stage III-IV RLF are at a higher risk for neurodevelopmental problems and that their families are in need of comprehensive support services.

Child Development↗

[Early ERG-signs of retrolental fibroplasia (author's transl)].

Examinations of retinograms of premature infants by the authors showed a time lag, changes in the wave form and reduction of the bioelectric activity of the retina (which shows itself very early) in association with the first signs of retrolental fibroplasia. In their experience the ERG becomes normal when the fibroplasia stabilizes itself early or when the retinal changes regress. In conclusion it must be stressed that with progression of the fibroplasia and retinal changes the biological activity of the retina also suffers.

Bioelectric Energy Sources↗

Effect of intramuscular vitamin E on frequency and severity of retrolental fibroplasia. A controlled trial.

126 appropriate-for-gestational age neonates born in hospital with birth-weights of 750 g-1500 g were prospectively studied in a randomised trial to determine the effect of intramuscular vitamin E on the frequency and severity of retrolental fibroplasia (RLF). In the 99 who lived for more than one month the frequency of active RLF in the vitamin E and control groups in the nursery (18.8% vs 23.5%) and at discharge (8.3% vs 19.6% was not significantly different. Vitamin E had significantly reduced the severity of RLF at follow-up eye examination 6-33 months after discharge (p less than 0.01). 3 control infants (760 g, 780 g, 1020 g) were blind in both eyes (grade V cicatricial RLF), whereas the most severe grade of disease in the vitamin E infants was grade II cicatrix. Infants in whom RLF developed were gestationally younger (p = 0.005), required a longer period of mechanical ventilation (p = 0.035), had a greater exposure to oxygen (p = 0.03), and had a greater number of arterial PO2 values between 75 and 99 mm Hg (p = 0.004). Early administration of vitamin E, while not affecting the frequency of RLF, will significantly reduce its severity and subsequent eye damage, and such treatment is recommended for all low-birth-weight infants of less than 1250 g who require supplementary oxygen.

Acute Disease↗

Retrolental Fibroplasia. II. Pathologic correlation.

Retinas of 142 eyes from 71 premature infants on whom autopsies had been done were studied by gross observation and by using PAS-stained whole mount, trypsin-digest, and conventional histological techniques. With these techniques, vascular pathologic condition was correlated with fluorescein angiograms in living infants with acute retrolental fibroplasia. The specific lesions seen in acute phase were a major arteriovenous shunt in the eye, microvascular changes including tufting, and obliteration of capillaries around arteries and veins. Regression occurred by vascular budding from the anterior edge of the shunt. Observations on vascularization in the normal indicated a variability of the level of maturation of the retinal vasculature, only roughly correlated with gestational age.

Arteriovenous Malformations↗

A classification of retrolental fibroplasia to evaluate vitamin E therapy.

A refined classification of the stages of the retinopathy of prematurity (RLF) based on the experience of over 7500 examinations during the past decade is presented. We have been using the basic elements of this classification since 1972 in order to evaluate the influence of vitamin E on retrolental fibroplasia (RLF). It is our impression that it provides a more accurate clinical method of following the course of the retinopathy and a tool for assessing the factors other than prematurity and hyperoxia that may play a subtle role in the development of RLF.

Humans↗

[Epidemiological study of severe vision impairment in Swiss children with special reference to retrolental fibroplasia].

On December 31 1984, 262 children between 7 and 16 years of age were being trained in the major institutions for the visually handicapped in Switzerland. Of these, 173 (66%) were visually impaired and 89 (34%) were blind (i.e. some or no light perception). The causes of the visual impairment were malformations (65%), infectious diseases (14%), retrolental fibroplasia (12%), metabolic disorders (4%), tumors (3%) and accidents (2%). Among the blind children these diagnostic groups were distributed as follows: malformations (53%), RLF (25%), infections (10%), tumors (10%). The rates of blindness within The single diagnostic groups were as follows: accidents: 4 out of 5 children; tumors: 78%; RLF: 69%. This risk of developing blindness in RLF children, as compared to the risk in the overall population studied, had the highest statistical significance (p less than 0.0001). Malformations (28%) and infectious diseases (22%) represented a lower than average risk of developing blindness, the average figure for the whole population being 34%. The risk of developing blindness as a consequence of infections was statistically not different from that in the total population investigated. All RLF children had a birthweight below 2000 g. By extrapolation an incidence of 0.24% RLF cases was estimated for prematures below 2000 g. During the 10 years under investigation (1968-1977), 3.6 new cases of RLF/100,000 live births were diagnosed per year, with a peak in 1975 and 1976. This figure is probably an underestimate, since severely brain damaged blind children with multiple handicaps were not considered.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Blood transfusion: a possible risk factor in retrolental fibroplasia.

The effect of blood transfusion on the occurrence of RLF was evaluated in 58 infants who weighed less than 1001 g at birth (Group I) and 70 oxygen treated infants of various birth weights (Group II). Although there was no significant difference between Group I infants with or without exchange transfusion as to birth weight, gestational age, duration of oxygen therapy, peak Pa02's, or multiple births, there was a significantly increased incidence of pre-retrolental fibroplasia in transfused over non-transfused Group II infants. When Group II infants were stratified for prematurity and oxygen duration, this difference persisted in those not already at risk for RLF. This increased incidence of retinopathy in transfused infants suggests that blood transfusion may be a risk factor in the pathogenesis of RLF.

Apnea↗