PEDIATRICS Vol. 5 No. 5 May 1950, pp. 771-782
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RETROLENTAL FIBROPLASIA

(II. Encephalo-Ophthalmic Dysplasia) Study of 66 Cases

W. R. HEPNER M.D.1, ARLINGTON C. KRAUSE M.D.1, and HELEN E. NARDIN A.B.1

1 The Department of Pediatrics and Division of Ophthalmology, Department of Surgery, University of Chicago, Chicago, Ill.

Ninety-two per cent of the 66 cases of retrolental fibroplasia seen at the University of Chicago were born prematurely. No patients found to have retrolental fibroplasia were born at the Chicago Lying-in Hospital before 1937. The incidence of retrolental fibroplasia at the Chicago Lying-in Hospital from 1937 through 1945 was 6.8% of all survivals weighing less than 1600 gm. at birth; from 1946 through 1948, the incidence was 30.4% in the same weight group. The ratio of surviving infants born weighing less than 1600 gm. to the total number of surviving infants born at the Chicago Lying-in Hospital remained relatively constant from 1937 through 1948.

The etiology of retrolental fibroplasia cannot yet be explained by prenatal bleeding or specific infection. Cutaneous angiomas cannot be said to have etiologic significance in retrolental fibroplasia since the incidence of cutaneous angiomas is no greater in infants with the disease than in premature infants in general. No relationship between the use of sulfonamide drugs and the development of retrolental fibroplasia can be established in this series of patients.

An ocular disease of postnatal origin may be separated from the composite group. With this postnatal disease retinal detachment is the basic sign, prematurity is universal, and associated central nervous system and somatic defects are less common. A large increase in incidence of this postnatal disease occurred with a change in neonatal management. During the same period, no change in the incidence of the disease of prenatal origin was apparent.

The change in neonatal management that could be related to the rise and fall in incidence was the use of a multiple vitamin preparation containing a synthetic emulsifying agent. An attempt to correlate the first appearance of or the changes in incidence of the disease at the University of Chicago was the first use of vitamin A or modification of the dosage of vitamin A was unsuccessful.

It is suggested that the title retrolental fibroplasia be reserved tentatively for those infants who have bilateral retinal separation as a complication of extreme prematurity until an etiologically appropriate name may be proposed.