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FIBROBLASTIC OVERGROWTH OF PERSISTENT TUNICA VASCULOSA LENTIS IN INFANTS BORN PREMATURELY* II. Report of Cases-Clinical Aspects T. L. TERRY, M.D. Boston, Mas. So-called persistent tunica vasculosa lentis, or fibroblastic sheath behind the crystalline lens,t is known to occur sporad- ically. Usually the condition is discovered a few days after birth in infants born at full term, but the correct diagnosis often is not established until the eyes are examined patholog- ically after removal because of a clinical diagnosis of retino- blastoma. Almost invariably this lesion is unilateral. That the condition is infrequent is borne out by the paucity of literature on the subject and the scarcity of pathologic specimens.1'2,3'4,5 Within the last year a similar condition has been found in both eyes of a number of infants born about eight weeks prematurely. This bilateral fibroplasia at first appeared to be a new disease entity related to prematurity. Only after a study of Cases 5 and 7 was it apparent that the condition found sporadically at birth was a different phase of the same disease in the prematurely born infants. In these premature * This research study has been made possible through a Special Fund for Research for the Pathology Laboratory of the Massachusetts Eye and Ear Infirmary. t Fibroplasia: Considerable thought has been given to the selection of an appropriate term for this disease condition. Early in the study, when the condition was believed to be represented fully by a massive growth of embry- onic connective tissue behind the lens, the term "retrolental fibroplasia" was suggested by Dr. Harry K. Messenger. As the study progressed, it was found that many other conditions also belonged to this disease entity, but so far no all-inclusive name for the condition has been devised. Perhaps the term "fibroplasia" might be as satisfactory as any, because all the changes appear to be related to a mesodermal overgrowth or to a persistence of embryonic meso- dermal tissue, and, insofar as I know, this term has not been used to describe any other condition. 262 TERRY: Overgrowth of Persistent Tunica Vasculosa Lentis 263 infants the lesion evidently develops after birth, since in no case was the condition encountered before the age of four months. The apparent frequency of this bilateral condition in infants born prematurely is impressive. In less than one year I have observed it in seven infants, it is known to exist in eight others, and probably is the pathologic lesion in five more. These cases show changes based on a variable degree of the following three principles: I. Persistence of all or some part of the hyaloid vascular system. The remains may be in the form of either vessels carrying blood or parts of closed blood-vessel walls. II. Embryonic connective-tissue growth behind the crys- talline lens, referred to herein as fibroplasia. III. Persistence of fetal character of the vitreous humor, i. e., a fibrillar structure. The specific findings may include the following: 1. Persistence of pupillary membrane-this can arise inde- pendently, however. 2. Congenital crowfoot pigment on the crystalline lens6 this can arise independently, however. 3. Congenital posterior synechia through failure of the connection between the tunica vasculosa lentis and iris meso- derm to disappear.6 4. Persistence of tunica vasculosa lentis. 5. Persistence of hyaloid artery. 6. Retrolental fibroplasia. 7. A type of congenital cataract. 8. Mittendorf dot. 9. Pseudopapilledema with- hypermetropia because trans- parent, unyielding remains of hyaloid artery with eye growth prevent the papilla from receding or even cause the optic nerve fibers to be pulled into the globe. 10. Glial projections and congenital retinal folds resulting from traction, as the eye enlarges, of unyielding vitreous fibrils. 264 TERRY: Overgrowth of Persistent Tunica Vasculosa Lenrtis 11. Shallow anterior chamber. 12. Microlentia. 13. Microcornea. 14. Microphthalmia. CASE REPORTS CASE 1. -E. B.,7 a male infant, aged six months, was referred for an eye examination as the parents and the pediatrician believed that the patient had some visual defect. The fundus could not be seen because of a grayish opacity in the pupillary area of each eye. The general color of the opacity was that of a relatively mature cataract. The anterior chambers were extremely shallow. The pupils reacted to light, and, when a small, strong, shielded light was applied to the sclera, the eyes could be transilluminated normally with a yellowish-red color seen in the pupillary area. The examina- tion was unsatisfactory not only because of the lack of cooperation of the patient, but also because of an almost constant irregular searching movement of the eyes. The absence of any discernible iris shadow on the opaque membrane led to the erroneous conclu- sion that bilateral congenital cataracts were present. Owing to the diminutive pupil, and the distance between the iris and the opacity, the iris shadow was not seen. Because of the difficulty experienced in repeated attempts to dilate the pupils of young infants, perhaps owing to the slowness of post-natal differentiation of the dilator pupillae, no attempt was made to obtain mydriasis at the first examination. The presence of a pupillary response showed that a functioning retina was present in each eye, and the clear transillumination was additional evidence that no neoplasm was present. However, when the infant was hospitalized for an ether examination and for discission of the cataract, the full extent of the diagnostic error was manifest. When mydriasis was attempted the night of admission, it was evident that the opacity was not im- mediately behind the iris. Because of the original conclusion that the lesion represented a more or less total opacity of the lens, this was considered perhaps a subluxation of the lens, since movements of the eyes seemed to shift the depth and location of the opacity. S. H. Clifford, the pediatrician, agreed to await full mydriasis and a detailed examination under ether anesthesia before altering the plans for some type of operation. However, the following morning the true nature of the lesions was evident when blood-vessels were discovered in the membranes by P. A. Chandler. Only the day TERRY: Overgrowth of Persistent Tunica Vasculosa Lentis 265 before Clifford and Chandler had consulted F. H. Verhoeff regard- ing the eyes of another infant born prematurely, and in this case Verhoeff had arrived at a diagnosis of bilateral persistent tunica vasculosa lentis. Verhoeff confirmed the diagnosis in my case also. The findings in this case consisted in small eyes, shallow anterior chamber, and a grayish, vascularized tissue immediately behind the crystalline lens. At the extreme periphery dentate processes, cor- responding perhaps to attachment to ciliary processes, were evi- dent. Between these processes a red fundus reflex could be seen. The following prenatal history was obtained from the obstetrician and the pediatrician: The mother had had two previous pregnancies. The first was entirely normal, except that the placenta was retained. The child born of this pregnancy is now a normal boy. At her second preg- nancy the mother was delivered of a stillborn infant three weeks past term. During the third pregnancy twins were diagnosed at twenty- three weeks. The patient was under treatment for bacilluria, which had been discovered five weeks previously. The membranes ruptured at seven and one-half months. Four days later the mother's temperature rose to 1020 F. Termination of pregnancy was undertaken by the administration of two minims of pituitary extract. Soon after, the temperature rose to 1050 F. and the pituitrin was repeated in doses up to four minims. The first twin (Case 1) was delivered spontaneously, and the second twin about an hour later. Two days after delivery the mother had a sudden chill and the temperature rose to 107.80 F. Cultures of her uterus showed enterococci (streptococci) and gram-positive spores that were non- gas producing. The urine cultures showed enterococcus (strepto- coccus). Sulfanilamide therapy, later changed to sulfathiazole, was instituted. The infection subsided, and the mother was discharged from the hospital in two weeks. The pediatrician's notes on the twins were as follows: "The second twin weighed 1006 gm. (2 lbs. 3Y2 ozs.). This baby showed very gasping respirations, with marked retraction of the sternum and cyanosis. The lungs were almost completely dull to percussion. The baby died at about twelve hours of age. "The first twin (Case 1) weighed 1021 gm. (2 lbs. 4 ozs.). The child was examined at the age of ten minutes, at which time the respirations were gasping and irregular, with slight retraction of the costal margins and moderate cyanosis. The lungs were generally dull, with poor breath sounds and many fine rales. The baby was immediately placed in oxygen. After about ten hours his condition improved, although he still had considerable atelectasis, especially 266 TERRY: Overgrowth of Persistent Tunica Vasculosa Lentis on the right posterior. The muscle tone was good. The baby's temperature was 960 to 980 F. He was continuously in oxygen, and the environmental temperature was kept at 900 F. On the second day the baby's temperature ran 96.50 F., the bed temperature was 850 F., and the oxygen was continued. He was given a small clysis of saline and nothing by mouth. On the third day a second clysis of saline was given; the baby was slightly dusky, but other- wise the color and cry were good. "Two days after birth, gavage feedings were started by mouth; the baby's condition was considered good. His course was unevent- ful until the age of seventeen days, when he had two cyanotic attacks. At this time the right chest was found to be dull poste- riorly; the left was normal.

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