Maurice F. Rabb » Unusual Presentation Of Retrolental Fibroplasia (RLF)

Case I
Infant was born at 26 weeks gestation to a gravida to a 15 year old mother. Birth weight was 740 grams, and the Apgar scores were 1 at one minute, and 2 at five minutes. The pregnancy was diamniotic. Intubation and 100% O2 were required after birth, with rapid weaning to 28% by age 11/2 days. At day number 3 the baby began to have recurrent episodes of apnea and bradycardia, which required constant O2 therapy up to 50% for 6 days. The baby was weaned to room air by age 12 days. A patent ductus arteriosus was noted on day 8, and was controlled with digoxin. One transfusion for anemia was needed. A work-up for sepsis (spinal tap, multiple blood cultures, urine cultures) was negative at birth. Antibiotics were discontinued after results were obtained.

The ophthalmologic picture of the right eye reveals a large arteriole venous shunt in the peripheral retina. The venules leading away from the shunt appear to be larger than normal. Superimposed on the venules and arterioles are a number of small, round, reddish-pink "bulbs" that are preretinal, since they seem to be located over the arterioles as well as the venules. Fluorescein angiography reveals hyperfluorescence of these unusual bulbs but no evidence of leakage. The A-V shunt can also be seen to fluoresce quite vividly. Another interesting finding is the presence of a "bulb" located on the disc. Angiography demonstrates that the lesions fluoresces vividly along with three other lesions, located 1 to 2 disc diameters from the disc. There was a vitreous hemorrhage in the left eye.

Case II
Infant was a 950 gram, appropriate for gestational age, 28 week premature who had perinatal asphysia, respiratory distress syndrome, and a patent ductus arteriosus with congestive heart failure. The baby needed a ligation of the patent ductus arteriosus for congestive heart failure that was unresponsive to digoxin. The pregnancy was complicated by premature rupture of membranes and a history of endometriosis. Apgars were 5 and 7. After birth the baby was placed on 100% O2 for a time period of one day, and the baby was weaned to room air. A work-up for sepsis was done at birth, which was negative and antibiotics were discontinued after several days. A transfusion for anemia was given at age day 2. The baby opened the patent ductus arteriosus at age three days, and began to have episodes of apnea and bradycardia. This required the use of O2 with concentrations up to 50% with continuous positive airway pressure. The ligation of the patent ductus arteriosus was done on day 26, and the O2 was discontinued 17 days later with O2 up to 70%. The baby was seen after the O2 was discontinued and found to have tortuous vessels in the superior temporal area. Two weeks later the baby had eviidence of RLF. There was no oxygen therapy between these two examinations. After weighing 2kg, the baby was discharged.

Color photographs of the left eye reveal a large avascular zone in the periphery of the retina. At the edge of the avascular zone is a thin, mesenchymal shunt. Leading to and from the shunt are arteriole and venous vasculature. Superimposed on the vasculature is a pinkish tissue, which in some areas coalesce. In other areas they are only mere "bulbs" which are superimposed over the arterioles and venules. Fluorescein angiography of the peripheral retina reveals possible areas of capillary dropout posterior to the mesenchymal shunt. Small fleshy nodules located on the vasculature fluoresce but do not leak. In the late venous stage the small saccules still fluoresce but there is still minimal leakage in only one area of the mesenchymal tissue.