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PubMed1979

[Present status of retrolental fibroplasia in France].

A national survey showed that retrolental fibroplasia still exists in France, as it does in other countries of the world. Retrolental fibroplasia is a condition that principally affects low birth weight premature babies in whom oxygen therapy has not been adequately controlled. The premature babies who have apnaeic attacks are most risk. Strict control of all oxygen therapy, preferably using continuous monitoring techniques together with other changes in the methods of treatment should reduce the incidence of this condition.

Apnea↗

PubMed1982

International classification of retrolental fibroplasia: a proposal.

There is evidence that, owing to the increased rate of salvage of premature infants weighing less than 1500 g at birth, the incidence of both retinopathy of prematurity (acute retrolental fibroplasia) and cicatricial retrolental fibroplasia is increasing. Treatment of the acute process to prevent blindness is controversial. In order that adequate studies be conducted to settle this issue, an international classification of retrolental fibroplasia is proposed.

Blindness↗

PubMed1977

Retrolental fibroplasia: I. Clinical observations.

The changes occurring in retrolental fibroplasia were studied in 97 premature infants with fundus photography and fluorescein angiography. The macrovascular lesion of proliferative retrolental fibroplasia was identified as a functioning arteriovenous shunt. The microvascular abnormalities were capillary tufts, collaterals, capillary-free zones, and neovascular membranes. Regression was signaled by the ingrowth of capillaries from the shunt into the avascular retina. Cicatrization was characterized by persistence of the vascular abnormalities of the proliferative phase and organization of the avascular retina into a contracting scar.

Fluorescein Angiography↗

PubMed1984

A reexamination of the role of oxygen in retrolental fibroplasia.

A critical review of the literature of retrolental fibroplasia indicates that the cause of this disease is not yet known. Oxygen is certainly a critical factor but it is still not possible to make precise recommendations as to the amount or the duration of therapy that is safe. We have overemphasized the role of oxygen in the past, and as a result of this the false impression has been created that RLF is a disease that can be prevented. This gross oversimplification of a complex disease with multiple causes has resulted in many unjustified malpractice claims. A study of the present epidemic indicates that excessive oxygen administration probably plays a minor role, in contrast to the first epidemic in which prolonged oxygen administration was clearly a major factor. A reasonable working hypothesis is that the developing retina is highly sensitive to any disturbance in its oxygen supply, either hyperoxemic or hypoxemic. The retinal circulation is subject to the same wide fluctuations as the cerebral circulation in newborn infants. The very low-birth-weight, sick premature infant suffers from a number of conditions, many of which can seriously disturb the retinal circulation, resulting in hypoperfusion and ischemia. These factors (immaturity, hyperoxia, hypoxia, blood transfusions, intraventricular hemorrhage, apnea, infection, hypercarbia, hypocarbia, patent ductus arteriosus, prostaglandin synthetase inhibitors, vitamin E deficiency, lactic acidosis, prenatal complications, genetic factors) may all be present in an infant. They may interact to produce various degrees of retinal damage. Nearly all of these factors cannot be prevented or controlled by our present methods of care. Unfortunately, this means that RLF is an extremely difficult disease to prevent, treat, or investigate. A disease of this complexity with multiple causes will require very large numbers of infants in any controlled study of a therapy. Retrolental fibroplasia should not be considered an avoidable iatrogenic disease in very low-birth-weight infants. Its cause in these infants is not known.

Anencephaly↗

PubMed1980

Secondary angle-closure glaucoma in cicatricial retrolental fibroplasia.

Five patients had cicatricial retrolental fibroplasia with a complication of secondary angle-closure glaucoma. In all the patients, the glaucoma occurred after age 2 years. All were treated with a pars plana lensectomy and anterior vitrectomy, which alleviated the glaucoma. They all achieved normal intraocular pressure and a pain-free eye. However, visual acuity was poor, in the range of light perception to hand motions. All patients have been followed up for one year and in only one patient has the fellow eye developed secondary angle-closure glaucoma.

Child, Preschool↗

PubMed1981

Retrolental fibroplasia today.

The incidence of retrolental fibroplasia (RLF, retinopathy of prematurity) is increasing, as more premature infants of lower birth weight are surviving. Currently, about 17% of premature neonates at risk will develop this condition, although in 85% of these cases it will undergo spontaneous resolution. This paper reviews our current knowledge and management of RLF, and aims to remind us that sophisticated monitoring techniques have not eliminated the problems of hyperoxia.

Diagnosis, Differential↗

PubMed1986

Retrolental fibroplasia: a five-year experience in a tertiary perinatal center.

During a five-year period, 565 premature infants in a tertiary perinatal center were evaluated by binocular indirect ophthalmoscopy. One-hundred-ten infants had retinopathy of prematurity. Ten of these infants developed grade 5 cicatricial retrolental fibroplasia, whereas two fellow eyes remained stable at grade 2 retrolental fibroplasia. Surgical lensectomy and vitrectomy failed to improve vision or reattach the retina in any of those with grade 5 retrolental fibroplasia. In three operated eyes and two unoperated eyes secondary glaucomas developed. Five vitrectomized eyes are now known to have phthisis. The factors that modulate the transition from the benign, acute retinopathy of prematurity lesions to cicatrizing (scarring) lesions characteristic of retrolental fibroplasia remain unclear. Birth weight (less than 1000 g), multiple apneic episodes with concomitant ventilatory assistance with 100% oxygen, and inadvertent hyperoxemia during general anesthesia may be significant cicatrization factors.

Follow-Up Studies↗

PubMedDate not supplied

Combined cryosurgery and scleral buckling in acute proliferative retrolental fibroplasia.

The paper demonstrates that limited cryosurgery combined with scleral buckling in premature infants with acute proliferative retrolental fibroplasia in a procedure which can be extremely successful in the treatment of this devastating disease. Timing for surgical intervention is critical. The indications for the surgical intervention are stated. The continuous monitoring of those infants with retrolental fibroplasia is critical to the success of the operative procedure. Since critical timing for success is involved, a new schedule for ophthalmic examination of the premature infant is proposed. This new schedule differs significantly from that proposed by the American Academy of Pediatrics in 1971. At that time, there was no successful treatment for retrolental fibroplasia. In contrast, we submit that limited cryosurgery combined with scleral buckling is an efficacious form of therapy for this disease.

Child↗

PubMed1982

Lensectomy-vitrectomy for stage V cicatricial retrolental fibroplasia.

Ten infants (18 eyes) blind from stage V cicatricial retrolental fibroplasia were examined from January 1978 to January 1981 at North Carolina Memorial Hospital. The early perinatal factors which may have predisposed to the binding end-stage cicatrix are identified. To increase their visual potential and to prevent secondary angle closure glaucoma, 12 lensectomy-vitrectomies were performed on 12 eyes (eight infants). One eye (one infant) had a pars plana vitrectomy without lensectomy. Seven eyes (seven infants) were not operated. Five of these eyes (five infants) had stage VRLF while two eyes (two infants) remained stable at stage II RLF. All retinas remained detached immediately after surgery. Secondary vitrectomies with air injections (three eyes, two infants) similarly failed to attach any retinas. Adherence of ciliary epithelium to clear lens and retrolental membrane as visualized within the dilated pupil (Eifrig-Merritt sign) was an intra-operative indicator of imminent surgical failure. Two eyes (two infants) developed early vitreous hemorrhages, hyphemas, secondary glaucoma, and phthisis bulbi within 12 months. Follow-up data were unavailable on two infants. Blindness secondary to cicatricial retrolental fibroplasia is increasing due to increased survival of low birthweight premature infants. Since surgical visual rehabilitation is not possible after the retrolental cicatrix has formed, the risk factors common to this high risk population should prompt preventive therapies.

Cicatrix↗

PubMed1984

Retrolental fibroplasia in full-term infants without exposure to supplemental oxygen.

We observed retinal folds identical in appearance to Grade 3 cicatricial retrolental fibroplasia in three infants at birth. In two of them we observed the active lesion of acute retrolental fibroplasia in the fellow eye at birth. These observations lead us to suggest that the active lesion of retrolental fibroplasia can occur in utero and progress to cicatrization in the absence of any obvious precipitating factor.

Diagnosis, Differential↗

PubMed1978

Prevention of cicatricial retrolental fibroplasia by cryotherapy.

A premature infant who was developing cicatricial retrolental fibroplasia, was given cryotherapy to both eyes. This stopped the progress of the disease in almost all areas of the retina. The infant died three months later of complications related to prematurity. A comparison between the inadequately treated areas and those adequately treated during the clinical follow-up prior to death and in the pathology of the eyes confirms the value of cryotherapy in preventing cicatricial retrolental fibroplasia and indicates where it should be applied.

Choroid↗

PubMed1985

Risk factors for retrolental fibroplasia: experience with 3,025 premature infants. National Collaborative Study on Patent Ductus Arteriosus in Premature Infants.

The rate of retrolental fibroplasia in relation to prenatal and neonatal characteristics was explored on the basis of a cohort of 3,025 neonates with birth weight less than 1,750 g. The overall rate of retrolental fibroplasia of any degree at hospital discharge was 11%, varying from 43% for those with birth weight between 500 and 749 g to 3% for those in the 1,500- to 1,750-g category. Among the potential determinants, the main interest was in nonhyperoxic characteristics, conditional on measures of prematurity and oxygen supplementation. Maternal diabetes and antihistamine use during the last 2 weeks of pregnancy were associated with significantly higher rates of retrolental fibroplasia, whereas toxemia was associated with lower rates. Frequent apneic spells, bronchopulmonary dysplasia, and sepsis in the neonate were also associated with significantly higher rates. On the other hand, the data indicate no independent role of low Apgar score, intraventricular hemorrhage, exchange transfusion, patent ductus arteriosus, or certain other characteristics previously postulated as risk factors.

Anemia↗

PubMed1977

[Retrolental fibroplasia: retrospective study (author's transl)].

Retrolental fibroplasia (RLF) is an iatrogenic disease of immature or dystrophic newborns who had been given oxygen treatment. Exchange transfusions seem to be of aetiological importance because they change the oxygen dissociation curve. Oxygen treatment of the newborn requires exact monitoring which can be done only in specially equipped departments. The retinal changes, up to the full-blown picture of RLF, progress rapidly. Ophthalmological control is, therefore, necessary as early as four weeks of age. If early RLF changes are demonstrated serial examinations every two weeks are indicated so that, if progression is demonstrated, treatment can be instituted.

Age Factors↗

PubMed1980

Retinal detachment in retrolental fibroplasia.

Problems of retinal detachment in retrolental fibroplasia (RLF) based on observations during 10 years of 92 premature children treated with supplemental oxygen in incubators are discussed. Two essential types of retinal detachment can be distinguished: the oedematous type in the stages III-V of the active phase of RLF and the rhegmatogenous type of cicatricial RLF. In our material 2 cases of oedematous detachment, which regressed spontaneously, were found. The case of a 10-year-old boy with total retinal detachment in cicatricial RLF, which was diagnosed too late, is presented. Since good results have been described after scleral buckling procedures in cases diagnosed early enough, all children with RLF should remain under steady control.

Adolescent↗

PubMed1983

Asymmetry in retrolental fibroplasia.

Ten cases of asymmetry in retrolental fibroplasia vary from patients with unilateral to those with bilateral involvement. In the bilateral cases, one eye was always more severely affected. This article encourages the treatment of amblyopia when present in patients with retinopathy of prematurity.

Amblyopia↗

PubMed1981

Retrolental fibroplasia: a study of the incidence and aetiological factors, 1977-1979.

Between July, 1977, and December, 1979, a total of 699 babies was screened in special care nurseries for retrolental fibroplasia (RLF). Changes were found in 46 babies (6.6%): in 25 of these, RLF reached only Stage I or Stage II of the Keith classification, and later regressed completely. The remaining 21 reached Stage III or higher and in 25% the condition progressed to permanent visual damage. Retrolental fibroplasia did not occur in babies whose highest PaO2 did not exceed 10.7 kPa (80 mmHg), and in babies who had less then three days of oxygen therapy. It did not reach Stage III in babies who weighed more than 1600 g at birth or were of more than 32 weeks' gestation. Girls were significantly more affected than boys. This study supports the strict correlation of RLF with prematurity and oxygen therapy; we found retrolental fibroplasia only in the very small and very premature infants, and visual damage only occurred in approximately 5% of these.

Acute Disease↗

PubMed1952

Retrolental fibroplasia; blindness in infants of low weight at birth.

Retrolental fibroplasia is today the principal cause of blindness in children of preschool age, exceeding all other causes combined. The disease occurs in infants of low weight at birth, commonly those born prematurely. The incidence of the disease is rising at an alarming rate. Vitamin E deficiency, corticotropin (ACTH) deficiency, the use of cow's milk in place of mother's milk, and improper oxygenation have been suggested as etiologic factors but the cause remains a mystery. Often the incidence is high in institutions in which maximal care is given premature infants.Clinically, the disease advances through an "active" phase during which regression is possible, and a "subsiding" or "cicatricial" phase which terminates with the formation of a disorganized opaque mass behind the lens. The earliest manifestations are noted in the fundi. Hemorrhages, neovascularization, transudation commencing in the periphery, and retinal separation contribute to the formation of the characteristic retrolental membrane. The diagnosis may be made when the retrolental membrane is observed in the eye of an infant whose weight at birth was low. Differential diagnosis is required occasionally. Thus far, no form of therapy has prevented or reversed the pathologic changes successfully. Use of vitamin E, corticotropin and mother's milk has not influenced the incidence of the disease. Avoidance of premature delivery if possible is indicated.

Blindness↗

PubMed1981

Retrolental fibroplasia: efficacy of vitamin E in a double-blind clinical study of preterm infants.

We performed a double-blind study in 101 preterm infants who weighed less than or equal to 1500 g at birth, who had respiratory distress, and who survived for at least four weeks, to evaluate the efficacy of oral vitamin E in preventing the development of retrolental fibroplasia. Weekly indirect ophthalmologic examinations begun when the infants were three weeks old revealed a significant decrease in the incidence of retrolental fibroplasia greater than or equal to Grade III (P less than 0.03) and greater than or equal to Grade II (P less than 0.05) (McCormick classification) in the 50 infants given 100 mg of vitamin E per kilogram of body weight per day as compared with 51 given 5 mg per kilogram per day (controls). When multivariate analysis was applied to the controls, five risk factors were identified: gestational age, level and duration of administration oxygen, intraventricular hemorrhage, sepsis, and birth weight. When multivariate analysis was applied to both control and treatment groups, the severity of retrolental fibroplasia was found to be significantly reduced in infants given 100 mg of vitamin E (P = 0.012).

Administration, Oral↗