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Infants with retrolental fibroplasia have been followed at the Bascom Palmer Eye Institute from August 1969 through December 1974. Changes occurring acute proliferative RLF were documented with fundus photography and fluorescein angiography. These are presented and especially those that have prognostic significance are emphasized.
Acute proliferative retrolental fibroplasia (RLF) has been studied in premature infants employing a Zeiss fundus camera and fluorescein angiography. A total of 164 angiograms have been performed on 122 infants. At the present time, angiography is reserved for studying infants with peculiar or puzzling fundus pictures. A dose of 0.1-0.4 cc of 10% sodium fluoresceinate is employed, depending on the age and the weight of the baby. Fluorescein clearly outlines the major arteriovenous shunt in the retina, which is the hallmark of acute RLF. The shunt fills with fluorescein and leaks it profusely. On regression, a fine brush border of capillaries is seen in the region where the shunt previously had been located. Study of the population susceptible to RLF reveals it to be the smallest sickest babies in the premature nursery.
The incidence of retrolental fibroplasia in a general hospital for the period January 1952 to January 1955 was reviewed and it was noted that a sharp decrease in incidence was associated with a reduction in the intensity of oxygen therapy. Retrolental fibroplasia developed most frequently in the smallest premature infants and no cases occurred in infants weighing more than 2,000 gm. at birth.
Six patients demonstrating typical signs of the cicatricial phase of retrolental fibroplasia are presented. None of the patients had received supplemental oxygen treatment after birth, and three patients were full term with birth weights ever 6 1/2 pounds. Two of the three premature patients had been born prior to 1901, before the advent of oxygen therapy for premature infants. It is apparent from these observations that retrolental fibroplasia does occur without oxygen administration and also in full term infants.
In ten cases of retrolental fibroplasia no significant relationship could be found between the stage of the disease and the neonatological values. The treatment given - laser coagulation, subconjunctival cortisone injections (glaucoma, complicated cataract) and pars plana vitrectomy - triggered different reactions in the eyes. Laser coagulation interrupted the process but fresh proliferation occurred; pars plana vitrectomies were followed by rapid efflorescences of the proliferations: Only in two of eight cases in stage V was vision permitting orientation achieved, with strikingly good close-range vision in one case. The considerable efforts involved and the mental strain on parents, patients and physicians are discussed in the light of the low rate of surgical success. A positive result can only be achieved if certain prophylactic rules are observed.
Forty-five babies were seen with retrolental fibroplasia (RLF) stage III or more, that is, with a large vasoformative ridge protruding into the vitreous cavity. Thirty-four attended for follow-up, but subsequently 6 died. Of the remainder 12 had satisfactory vision, 3 unilateral and 8 bilateral high myopia, 3 were totally blind, and 2 were partially blind. It was found that stage III RLF could be subdivided according to its severity. Early stage III had a good prognosis, with 11 out of 18 achieving satisfactory vision, but only one out of 16 in RLF stage IIIa or more. Cryothermy was applied to 9 babies in the latter group, but it did not seem to influence the final outcome.
The misconception that retrolental fibroplasia (RLF) occurs only in premature infants who have been exposed to excessive concentrations of oxygen is discussed. The fact that RLF occurs without exposure to oxygen is documented. Basic principles of jurisprudence regarding medical malpractice relating to RLF are discussed. Since RLF can occur without exposure to oxygen the doctrine of res ipsa loquitur should not apply. The burden of proof should still rest with the plaintiff.
With the Calgary Code, clinical observation in retrolental fibroplasia can be translated into numerical and alphabetical symbols. This paper outlines the prerequisites for use of the code, the method of use and the potential applications.
A pathologic analysis of 19 cases of acute retrolental fibroplasia in autopsy eyes from paranatal infants is presented. The clinical profile of these subjects showed no clear-cut maternal factors. Infant factors included systemic congenital anomalies (14 cases); ocular congenital anomalies (1); birth weight--less than 1500 grams (12), more than 2000 grams (4); neonatal complications--"respiratory distress" (8), "apneic spells" (7), Erythroblastosis fetalis (3); oxygen therapy of varying amounts (14); periodic elevations of arterial oxygen (7); and varying survival times (intrauterine death, one case; less then 48 extrauterine hours, 9 cases). Considering all cases, the lesions showed a progressive pattern--beginning with proliferation of primitive vascular mesenchyme in vanguard of advancing vasculature (four cases), formation of intraretinal band of endothelial cells in rear guard (10 cases), and finally, extraretinal neovascularization from rear guard zone (five cases).
Four cases of exudative retinal detachment associated with retrolental fibroplasia are presented. The exudation appears to be secondary to leakage from neovascularization as well as vitreous traction on normal retinal vessels. The range in age of onset was between 13 and 17 years, with three of the four cases occurring in patients 23 years of age or younger. Treatment is directed at eliminating the abnormal vasculature which, in our experience, has been accomplished best by an encircling scleral-buckling procedure. One patient, however, required vitrectomy because of the severe vitreous membranes and traction which were present.
Aspirin administration, at a dosage producing plasma levels within the human therapeutic range, caused marked inhibition of production of both vascular prostacyclin (a vasodilator) and platelet thromboxane (a vasoconstrictor) in beagle puppies. In addition, aspirin-treated, oxygen-exposed puppies developed retinopathy of significantly greater severity than their unmedicated, oxygen-exposed littermates. Direct ophthalmoscopic observations indicated that whereas sustained oxygen breathing produced retinal vasoconstriction in unmedicated puppies, retinal vessels of aspirin-treated littermates became more dilated or remained unchanged. It is postulated that retinal vasoconstriction may be a normal physiologic mechanism to protect the immature retina from damaging effects of high blood oxygen levels; i.e., it may be a protective rather than a pathologic process in response to hyperoxia. Many vascular anomalies which characterize the human disease were present in the retinas of the puppies. Several of the most severely affected puppies treated with aspirin even displayed grade III cicatricial retinopathy (falciform retinal fold). Thus, a major criticism of the retrolental fibroplasia animal model has been addressed by producing cicatricial retrolental fibroplasia in puppies, and the confidence with which results from experimental animal studies might be extrapolated to the clinical situation is thereby strengthened.
From 1964 to 1976, 20 cases of retrolental fibroplasia were recorded in the south of France while 38 cases were recorded in the north from 1970 to 1977. The more children are immature, the more they are liable to suffer from this disease. Etiological factors are apparently not limited to oxygen, which however plays the most important part in the process.
Administration of control, oral or combined intramuscular and oral vitamin E to 418 high risk, preterm infants (1500 g or less birth weight; 32 weeks or less gestational age; 305 surviving more than 10 weeks) led to raised peak plasma levels of the vitamin of between 0.6 and 3.3 mg% in three consecutive clinical trials. This early, non-toxic, prophylactic supplementation suppressed the development of severe retrolental fibroplasia when the antioxidant was given continuously from the first day of life until retinal quiescence. Studies of 63 pairs of eyes by transmission electron microscopy revealed that spindle cells (the embryonic precursors of inner retinal capillaries) were the primary target of the vitamin E-induced suppression of severe retrolental fibroplasia. Extensive increases in the gap junctions between adjacent spindle cells in unsupplemented infants, or in supplemented infants of 27 weeks gestational age or less, triggered both neovascularization from the most nascent capillaries at the rearguard-vanguard retinal interface, and dilatation and tortuosity in the rearguard (posterior) retinal vessels. Continuous vitamin E supplementation permanently preserved the embryonic state of the spindle cells in infants of 28 weeks gestational age or more, and transiently retarded gap junction increases in infants of 27 weeks gestational age or less.
Three patients with temporal displacement of the macular secondary to retrolental fibroplasia had true strabismus in associations with amblyopia and pseudostrabismus related to a large, positive angle kappa. In one case, a headturn associated with the neutral point of nystagmus was also a presenting problem. Ocular deviation was measured by the light reflex test and the prism cover test, with particular attention paid to adequate cosmetic alignment. Large amounts of prism placed before the fixating eye resulted in loss of visual acuity, while eyes placed in a parallel position by surgical means may not appear to be straight. Thus, surgery can result in parallel ocular alignment while prismatic therapy may be required to center the pupillary light reflex and to provide satisfactory cosmetic appearance.
Four infants and one child with retinal detachments from cicatricial retrolental fibroplasia involving traction judged too severe to be relieved by scleral buckling alone underwent lensectomy and vitrectomy. Three retinas were successfully reattached. Because of the incomplete development of the pars plana in infants and the manner in which peripheral retina is pulled anteriorly to a position near or in contact with the posterior lens capsule, entry through the pars plana can create iatrogenic tears in the peripheral retina or introduction of irrigation fluid under the retina. Our experience has shown that a small contact lens makes it possible to work in the posterior vitreous cavity through the limbal approach in these infants. The circumferential traction produced by the ring of fibrovascular proliferation behind the lens can be relieved and tractional bands running forward from the posterior pole can be cut. Fibrovascular proliferative tissue lying on the posterior pole, however, proved resistant to membrane peeling or dissection, and its severity seemed to determine the success or failure of the surgery.
Three premature babies with advanced stages of retrolental fibroplasia underwent retinal surgery by buckling procedures, with satisfactory results. The indications and surgical technical problems are discussed.
The level of arterial oxygen tension [Pa,o2] at which retrolental fibroplasia (RLF) may develop in very-low-birthweight infants is not known, but may be less than 100 mmHg (13.3 kPa). We report an infant of 27 weeks gestation who developed RLF despite continuous monitoring of Pa,o2 from 2 h to 4 weeks of age, with no recorded values above 95 mmHg (12.7 kPa). We therefore decided to maintain the Pa,o2 below 80 mmHg (10.7 kPa) in very-low-birthweight infants, using when necessary a low oxygen gas mixture (fractional inspired oxygen concentration 0.16-0.20). We report the use of this approach in four infants, all of whom survived without evidence of RLF. The need for further clinical studies of such an approach is discussed.
Three adult male patients with keratoconus associated with severe bilateral retrolental fibroplasia were studied. Two of these presented with acute corneal hydrops. The association may be of some interest in determining the etiology of keratoconus.