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PubMed1984

Retrolental fibroplasia and vitamin E in the preterm infant--comparison of oral versus intramuscular:oral administration.

To evaluate the efficacy of four early intramuscular injections of vitamin E given in addition to continuous minimal oral vitamin E supplementation, 168 very low-birth-weight infants (less than or equal to 1,500 g) have enrolled in a randomized, double-masked, clinical study. All infants received vitamin E orally, 100 mg/kg/d. In addition, on days 1, 2, 4, and 6, seventy-nine infants received vitamin E intramuscularly, 15, 10, 10, and 10 mg/kg, respectively. On the same days, 89 control infants received placebo intramuscular injections. Multivariate analysis of the 135 infants who survived greater than or equal to 10 weeks showed no significant difference in the development of severe retrolental fibroplasia between these two supplementation schedules (P = .86). Plasma vitamin E levels never exceeded a mean of 3.3 mg/100 mL, and no toxicity was observed. Ultrastructural analyses of seven pairs of whole eye donations from infants receiving IM vitamin E demonstrated identical kinetics of gap junction formation between adjacent spindle cells as compared with 13 pairs of whole eye donations from control infants (P greater than .3). Therefore, oral vitamin E supplementation affords retinal protection against the development of severe retrolental fibroplasia when initiated on the first day of life and maintained continuously until retinal vascularization is complete.

Administration, Oral↗

PubMed1978

Acute retrolental fibroplasia. II. Treatment by cryosurgery.

Fourteen eyes of 12 infants with acute retrolental fibroplasia were treated with cryotherapy. In only one or possibly two cases was treatment considered to be of positive value. Treatment in the remaining 12 cases was either ineffective or deleterlous. Surgical treatment is rarely, if ever, indicated and spontaneous resolution of retinopathic changes is still the most common course of the natural history of the disease. The role of the ophthalmologist in the perinatal intensive care nursery remains one of advisor to pediatricians and informant to parents of affected children.

Acute Disease↗

PubMed1977

PaO2 levels and retrolental fibroplasia: a report of the cooperative study.

The relation between PaO2 and retrolental fibroplasia (RLF) was studied prospectively in 719 premature infants born in or treated in the intensive care units of a group of university hospitals. Blood gas studies were performed on 589 of these infants, 66 of whom had a diagnosis of RLF; in 27 of these 66, some grade of mostly nonblinding cicatricial disease developed. The frequency of RLF was highest among infants of lowest birth weight. A multivariate statistical method was used to analyze simultaneously the effect of possible etiologic factors associated with RLF. The occurrence of RLF was found to be unrelated to PaO2, as determined by the limited information available from intermittent sampling. RLF is associated with concentration of oxygen administered in the lightest birth weight group, but the strongest association, aside from birth weight, was with time in oxygen. None of the other variables involving blood chemical values appeared to be associated with RLF. The severity of cicatricial RLF is clearly greater in infants weighing less than 1,200 g at birth. Conservative administration of oxygen may have been responsible for failure to demonstrate quantitative association between PaO2 levels and disease. Agreement between the observed and predicted numbers of infants with RLF demonstrate the strength of the multivariate technique employed in making the statistical analyses.

Birth Weight↗

PubMed1983

Development of blind infants and children with retrolental fibroplasia: implications for physicians.

Despite an apparent increase in the incidence of retrolental fibroplasia (RLF) and renewed interest in its pathophysiology and prevention, clinicians are often ill prepared to help the family promote the optimal development of its blind or severely visually impaired child. The physician's management approach is molded by both knowledge about the effects of blindness and prematurity on subsequent development and attitudes about blindness. Both issues are reviewed, with particular focus on the developmental course of the infant or child who is blind due to RLF. Although the literature regarding developmental outcome of premature infants with RLF has failed to reach definitive conclusions, studies reveal a tendency toward an IQ distribution similar to or slightly lower than that of various control samples. Blind children with RLF may also be at increased risk for emotional problems, largely as a function of early maladaptive family interaction patterns. Implications of these and other developmental patterns are translated into general and specific interventions that neonatologists, ophthalmologists, and primary care physicians can facilitate. Such physician guidance can help assure the blind or severely visually impaired child's optimal cognitive and emotional development.

Attitude of Health Personnel↗

PubMed1980

Risk factors in retrolental fibroplasia.

Possible determinant factors that may increase the risk of the occurrence of retrolental fibroplasia (RLF) were analyzed in 80 infants born in 1975 and 1976 with birth weights between 501 and 1,500 gm and who survived. Active and/or cicatricial RLF occurred in 27 (33.8%) infants and the factors significantly associated with RLF were: gestational age (P less than .001); apnea requiring bag and mask resuscitation with oxygen (P less than .001); septicemia (P less than .005); degree of illness; blood transfusion; and mechanical ventilation. Severe cicatricial RLF developed in eight (10%) infants (grades 2 to 5). In the group of infants all of whom had apnea which required resuscitation, septicemia was also significantly associated with RLF (P less than .01). A highly significant association between RLF and severe myopia (P less than .001) was found in follow-up in all infants. Data show a resurgence and high incidence of RLF in low birth weight infants who survived. Infants with these factors should be considered to have greater risk for the occurrence of RLF and ophthalmologic examination prior to, and within three months following discharge is recommended.

Apnea↗

PubMed1983

Closed vitrectomy for severe retrolental fibroplasia in the infant.

Fifteen infants between the ages of 5 months and 2 years suffering from severe retrolental fibroplasia with near total and total retinal detachment and retrolental scar tissue formation were operated upon with closed vitrectomy. The surgical technique and the problems related to the smallness of the eye and the changes caused by RLF are discussed in detail. Seven retinas were reattached completely and one retina partially reattached. Vision was definitely improved, although the visual acuity was probably low.

Cataract Extraction↗

PubMed1984

Lensectomy for secondary angle-closure glaucoma in advanced cicatricial retrolental fibroplasia.

A study of 15 patients with secondary angle-closure glaucoma associated with advanced cicatricial retrolental fibroplasia is presented. Eight patients developed glaucoma acutely, while 7 showed a chronic course. In four patients the fellow eye became involved acutely within 1 month of the first eye. Seven others were also bilateral, but on a chronic basis. Twelve of the 15 presented after age 2. All were treated with lensectomy which cured the glaucoma. All have extremely poor vision in the range of light perception to hand motion, but have retained their eyes on a pain-free basis.

Cataract Extraction↗

PubMed1977

Retrolental fibroplasia and factors influencing oxygen transport.

The hospital records of 58 premature infants in whom proliferative retrolental fibroplasia (RLF) developed were matched with the records of 58 infants without RLF for birth weight, gestational age, and duration oxygen therapy. The two groups were compared for factors likely to influence tissue delivery of oxygen by blood. No significant difference was found in incidence of blood transfusions or exchange transfusions, use of phototherapy, or occurrence of acidosis.

Acidosis↗

PubMed1982

A model for the pathogenesis of retrolental fibroplasia based on the metabolic control of blood vessel development.

We postulate that the pathogenesis of retrolental fibroplasia (RLF) is related to the disruption of the normally coordinated ocular development and maturation in which there is an interplay between function, metabolism, and blood vessel development. The normal retina is avascular until the fourth month of gestation, at which time vascularization proceeds from the optic disk towards the peripheral retina. Until this time, the underlying choroidal circulation provides for full-thickness retinal oxygenation. We hypothesize that the centrifugal maturation of the retina, including the retinal photoreceptors (the major oxygen-consuming cell type of the adult retina), precedes this vascular outgrowth. The maturation of the photoreceptors and the concomitant increased oxygen consumption would make it impossible for the choroid to provide adequate full-thickness retinal oxygenation. The resulting progressive change in the inner retinal metabolism thus provides an orderly, controlled stimulus for the subsequent progression of retinal vascularization from the optic disk to the peripheral retina. Exposure of the premature neonate to abnormal oxygen levels would selectively retard inner retinal blood vessel development while the photoreceptors continue to mature and increase their oxygen consumption. Thus, when the infant is removed from abnormal oxygen, a neovascular proliferative stimulus will develop in direct relation to the mass of non-vascularized but metabolically active retina, initiating uncoordinated vessel growth in the inner retina.

Fetus↗

PubMed1979

Retrolental fibroplasia in hypoxic newborn.

A premature infant with hypoxia caused by severe cardiac malformations developed stage IB retrolental fibroplasia, which was documented after death. Despite arterial oxygen partial pressures of 91 mm Hg or less the classical histologic changes were observed in the temporal periphery of each eye.

Cyanosis↗

PubMed1983

Risk factors for retrolental fibroplasia.

Besides oxygen administration and immaturity of the premature retinal vessels, there are other risk factors for retrolental fibroplasia: (1) respiratory distress syndrome; (2) multiple episodes of bradycardia apnoea; (3) exchange transfusions; (4) hyaline membrane disease; (5) anemia of prematurity; (6) hyperbilirubinemia; (7) avitaminosis E; (8) cardiovascular defects; (9) infectious diseases; (10) multiple births; (11) hypocalcemia; (12) hypothermia; (13) hemorrhagic tendency; (14) delayed coaptation of the retina, and (15) spastic diplegia.

Apnea↗

PubMed1982

Cryotherapy for retinopathy of prematurity to prevent retrolental fibroplasia.

This report analyses the results of cryotherapy in 22 eyes with severe retinopathy of prematurity (acute retrolental fibroplasia) and compares the results with data from other sources on untreated eyes. The evolution of the indications for and methods of treatment, the results of treatment, and related features are discussed. The need for a common terminology and classification of the disease is emphasized.

Child↗

PubMed1983

Vitamin E and retrolental fibroplasia: prevention of serious ocular sequelae.

In an attempt to determine the role of vitamin E in retrolental fibroplasia (RLF) we report our experience with 191 infants of less than 1500 g birth weight. Of these infants, 16.75% had evidence of acute RLF in hospital, 8.4% had cicatricial RLF at follow-up, and four infants (2.1%) were blind, none of whom had received supplementary vitamin E. The incidence of cicatricial RLF at follow-up was significantly lower in infants who received vitamin E early after birth (12 h) than in those who did not (3 of 105 versus 13 of 86, X2 = 9.26, P = 0.002), as was the incidence of Grade III or greater cicatrix (0 of 105 versus 7 of 86, X2 with Yates = 6.72, P = 0.01). Stepwise multiple linear regression analysis revealed three factors distinguishing infants who developed cicatricial RLF from those who did not: the lack of early vitamin E supplements (P = 0.0023), the significantly larger number of arterial PO2 values over 100 mmHg (P = 0.0056), and the presence of an intraventricular haemorrhage (P = 0.0032). The incidence and severity of necrotizing enterocolitis was similar in infants who received vitamin E and in those who did not. It is recommended that vitamin E be given within the first 12 hours of birth to all infants of less than 1500 g who require supplementary oxygen.

Administration, Oral↗

PubMed1980

Treatment of acute retrolental fibroplasia by cryopexy.

Fifteen to 20% of premature infants weighing below 1500 g develop variable degrees of acute retrolental fibroplasia (RLF). Approximately 5% of those infants who develop RLF can be expected to become blind. A prospective study was carried out to evaluate if early cryopexy can prevent this blindness. The study showed that, among 533 infants admitted to the neonatal intensive care unit between July 1976 and December 1978, 55 had some degree of active RLF. In 18 eyes of 9 infants in whom the retinopathy progressed beyond active stage II cryopexy treatment was applied to the avascular retina. In all the treated eyes the active retinopathy regressed, and there were no complications due to the procedure. The long-term follow-up showed that in 15 eyes there was good vision and that the remaining 3 eyes had some impairment of vision but were not blind. These results suggest that cryotherapy may prove useful in treating premature infants with RLF who show progression of their active stage, without the danger of further visual loss or complete blindness.

Acute Disease↗

PubMed1978

Retrolental fibroplasia. Cases seen at the Helsinki University Eye Hospital 1956-1974.

In the period 1956-1974, 31 new cases of retrolental fibroplasia (RLF) were diagnosed at the Helsinki University Eye Hospital. All were alive in 1974. 90% weighed less than 1500 g at birth and the gestational age of less than 30 weeks at birth was present in 85%. All patients had required supplementary oxygen during the first weeks of life. 84% of the eyes had visual acuity of finger counting 1 m or less and when classified according to the corrected visual acuity in the better eye, 71% of the patients were practically blind according to the classification by Rintelen. 81% of the eyes showed one of the cicatricial stages of RLF, III to V, and 45% showed the grade V of the disease. 11% of the eyes showed corneal opacities, 44% a flat or absent anterior chamber, 11% had intraocular pressure of greater than or equal to 30 mmHg and 21% showed lenticular opacities. When the period of the observation was surveyed, it was found that during the latter half of the observation period, twice as many new cases of RLF were born. At a rough estimate it was calculated that in 1956-1974 at least 50 new cases of RLF developed in Finland.

Birth Weight↗

PubMed1982

Retrolental fibroplasia--controlled study of 4 years' experience in a neonatal intensive care unit.

During the 4 years 1977-80, 14 infants developed retrolental fibroplasia (RLF) in the neonatal unit at this medical centre. All were very low birthweight (VLBW) infants who weighed 1500 g or less at birth. The incidence of RLF was 3.5% for all VLBW infants admitted for neonatal intensive care and 4.7% for VLBW survivors. The mean birthweight of the affected infants was 970 (range 730-1310) g and mean gestational age 26 (range 24-29) weeks. Seven of the affected infants (2.4% of VLBW survivors) had significant scarring with temporal dragging of the optic disc and retinal detachment. Each of the 14 infants was matched with 2 control infants in order to see whether any factors predisposing to the development of RLF, including those related to oxygen therapy and monitoring, could be identified. The only factor associated with RLF was a higher volume of blood given with replacement transfusions. The occurrence of RLF was unrelated to an increase in requirement for or duration of oxygen therapy, arterial oxygen tensions as determined by intermittent sampling, or the availability of transcutaneous oxygen monitoring. The care taken in oxygen therapy may have been responsible for failure to show a quantitative association between hyperoxaemia and RLF. Although the problem of oxygen therapy in preterm infants is far from being resolved, current neonatal intensive care methods have limited the occurrence of RLF to VLBW infants. This study demonstrated a lower incidence of RLF in VLBW infants despite an improved survival rate compared with that previously reported.

Humans↗

PubMed1990

Endothelial specular microscopy in children with retrolental fibroplasia undergoing open-sky vitrectomy.

Thirty-three patients aged 4 to 63 months with extensive retinal detachment due to cicatricial retrolental fibroplasia (RLF) and two non-RLF patients aged 26 and 81 months with normal corneas underwent specular microscopy and ultrasonic pachymetry during examination under anesthesia. Thirty-six eyes of preoperative RLF patients without anterior synechiae and 4 eyes of the two non-RLF patients had high endothelial cell density (ECD) of 5,105 +/- 790.4/mm2 (mean +/- SD) and 4,377 +/- 141.7/mm2, respectively, and increased corneal thickness. The ECD value showed significant negative correlation with vertical corneal diameter (P less than 0.005), but not with age. The ECD values, corrected to average adult corneal size, were 3,900 +/- 550/mm2, which were close to values extrapolated from adult ECD data. The average cell loss for 10 eyes undergoing specular microscopy before and after open-sky vitrectomy was 15%, but 8 of these eyes showed an unchanged ECD ratio of 0.79-1.3 between the two measurements. Furthermore, 17 eyes which were examined only postoperatively also showed a high average ECD of 4,175 +/- 1,012/mm2.

Cell Count↗

PubMed1978

Retrolental fibroplasia: a new analysis of risk factors based on recent cases.

Over an 18-month period, four severe and eight mild cases of retrolental fibroplasia (RLF) were diagnosed. All infants had gestational age less than 34 weeks. While the percentage of infants with RLF increased with decreasing gestational age, the severity of RLF was not related to birth weight or gestational age. These RLF infants were compared with premature infants having normal fundi. The arterial blood gas values in the first week of life were similar for the two groups. However, during the subsequent weeks of oxygen therapy, the infants who developed severe RLF had a significantly greater number of hours of capillary oxygen tension at undesirable levels (Pcap O2 greater than 50 mm Hg) than the mild RLF or control infants. These results suggest that infants may be at greatest risk of severe retinal damage from oxygen relatively late in the course of their oxygen therapy.

Humans↗