Surgical Results in Rubella and Nonrubella Congenital Cataracts

Surgical Results in Rubella and Nonrubella Congenital Cataracts

V O L . 66, N O . 3 U P T A K E OF RADIO/ P and, further, that this ability decreases with time. There is little question that the surgical wound in...

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V O L . 66, N O . 3

U P T A K E OF RADIO/

P and, further, that this ability decreases with time. There is little question that the surgical wound in man also has the ability to concentrate P , but to what degree this is influenced by the type of surgery and the duration of the postoperative period remains to be determined. W e have begun a followup study to determine the postoperative period required in man for the surgical wound to return to normal and to determine whether this period might be influenced by the type of surgery and the degree of postoperative inflammatory reaction. 32

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SUMMARY

The radioactive phosphorus test has questionable value following ocular surgery. The

SURGICAL RESULTS IN

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healing surgical wound (of rabbits) behaves like neoplastic tissue in that it may give a positive test for malignancy up to four weeks after surgery when the radioactive phosphorus test is used. The surgical wound may continue to take up phosphorus at a rate higher than normal through the sixth postoperative week. REFERENCES

1. Carmîchael, P. L. and Leopold, I. H . The radioactive phosphorus test in ophthalmology. Am. J. Ophth. 49:484, 1960. 2. Temer, I. S., Leopold, I. H . and Eisenberg, I. J. The radioactive phosphorus P" uptake test in ophthalmology. Arch. Ophth. 55:52, 1956. 3. Solomon, O. D., Moses, L. and Eigner, E. H . Validity of the radioactive phosphorus uptake test following ocular surgery. Am. J. Ophth. 55:1237, 1963.

RUBELLA AND NONRUBELLA

CONGENITAL EDWARD

T I V E PHOSPHORUS

CATARACTS

COTLIER,

M.D.

St. Louis, Missouri

Since the rubella virus isolations from the lens and aqueous humor of patients with congenital rubella, ' questions have been raised as to whether a better differentiation could be made between rubella and other types of congenital cataract. For years it was believed that the clinical and surgical prognosis of rubella eyes was good. In a recent study, however, Scheie, Schaffer, Plotkin and Kertesz found that in 43% of eyes operated on for congenital rubella cataracts, severe postoperative complications led to the eventual loss of many eyes. These findings appear restricted to that rubella population, and are unusual in that relatively few congenital rubella eyes have been removed, despite the 1 2

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From the Department of Ophthalmology and the Oscar Johnson Institute, Washington University Medical School. This study was supported by U S P H S Grant N B 07393, and a Career Development Award of the National Institute of Neurological Diseases and Blindness.

widespread epidemic of 1964-1965 in the United States. Furthermore, although many correctly diagnosed rubella congenital cataracts have been subjected to surgery during the last 25 years, no reports of poor results in such cases are available. In the series of eyes reported here, there was little or no difference in the results of surgery for rubella and nonrubella congenital cataracts. MATERIAL PATIENTS

AND METHODS

A N D SURGERY

The infants included in this study had cataract extractions by various surgeons at McMillan Hospital, Washington University School of Medicine, between 1960-1968. Eleven rubella syndromes were diagnosed on the basis of maternal history, deafness, cardiac or ocular defects, and/or rubella virus recovery from the lens, aqueous humor, or throat swab. Thirty-six randomly selected

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patients who underwent congenital cataract surgery and who had no systemic signs of congenital rubella or history of maternal infection during pregnancy, were compared to patients with rubella syndromes. Both groups included newborns, older infants, and children up to 10 years of age at the time of surgery. The surgical procedures for both groups were needle aspiration of the lens material, and linear (Barkan) extraction or discission. The needle aspirations were done through 18-24 gauge needles with one anterior chamber opening. Lens aspiration material obtained from the rubella patients at the time of surgery was placed in Eagle's or Earl's media, then transferred to roller tubes for virologie studies. The eyes were treated postoperatively with mydriatics and, in most patients, topical corticosteroids. The patients were followed from six months to seven years after surgery. The results were judged good if a central pupillary opening allowing a clear view of fundus details was present, and considered bad if the clarity of the eye media was impaired. In those eyes with longer follow-ups, the range of visual acuities among so-called good results was from 20/20 to 3/400. There was a high incidence of sensorimotor anomalies limiting the visual potential of all eyes, rubella and nonrubella alike.

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VlROLOGIC STUDIES

The samples were tested for rubella virus by the interference procedure of Parkman, Buescher, and Artenstein with Echo 11 virus, as described in an earlier paper. Specimens were transferred through three passages before considered negative. Positive cultures were further identified by neutralization with rubella immune rabbit sera, and in some specimens virus titers were found.

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RESULTS CLINICAL CASES

Clinical and virologie findings in patients with the rubella syndrome and the results of cataract surgery in rubella and nonrubella eyes are summarized in Table 1. Ten of the

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V O L . 66, N O . 3

CONGENITAL CATARACTS

11 rubella patients had heart defects such as ductus arteriosus, ventricular septal defects, or abnormal auscultation or X-rays. However, none of them proved to be an operative risk under general anesthesia. The birth weight of the rubella patients was below that of patients without rubella, in agreement with findings in other series. Eye defects associated with most congenital cataracts— rubella or other types—were nystagmus, esotropia, and high refractive errors. Five rubella patients had, either isolated or in combination, pigmentary retinopathy, transient corneal edema or congenital glaucoma. Two rubella eyes had fixed pupils, atrophic irides, posterior synechia and microphthalmos. Three rubella infants had neonatal thrombocytopenic purpura, three had microcephaly, one had cleft palate, one had alterations of long bones (revealed by X-rays), three had anemia, three had hepatosplenomegaly, one had jaundice, one had bilateral cryptochidism and mental retardation, and one had omphalocele. In the other 36 congenital cataract patients, there were no major systemic pathologic findings, although none was subjected to extensive pediatric studies as were the rubella patients. Seven of the 36 infants in the nonrubella group bad a family history of cataracts. 8

POSTOPERATIVE PERIOD

In the immediate postoperative period, patients with the rubella syndrome and those with other congenital cataracts developed transient inflammatory reactions of the anterior segment, consisting of pericorneal injection, cells, and flare in the anterior chamber that observers correlated with the amount of remaining cortical lens material. No instance of pupillary-block glaucoma was recorded among rubella eyes, but two nonrubella eyes developed transient flat chambers (one to three days), attributed by the surgeons to pupillary block. SURGICAL RESULTS

The results shown in Table 1 indicate

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that the surgical outcome of both rubella and other congenital cataracts was good, with little or no difference between the groups. In the rubella patients, good surgical results were obtained in 15 of 17 eyes ( 8 8 % ) ; the two eyes with poor results were microphthalmia In other congenital cataracts, good surgical results were found in 55 of 60 eyes ( 9 0 % ) . The five eyes of nonrubella patients with poor results had the following secondary pathologic or surgical complications : ( 1 ) aniridia and congenital glaucoma, ( 2 ) preexisting retinal detachment, ( 3 ) vitreous loss with pupillary-block glaucoma, ( 4 ) retinal detachment following surgery, and ( 5 ) microphthalmos. A number of patients required needling of the secondary membrane, or had Elschnig pearls. The incidence of secondary membrane was similar in rubella (seven out of 17 eyes—41%), and nonrubella cataracts (26 out of 62 eyes—42% ) . DISCUSSION

The good surgical results in rubella cataracts operated in the St. Louis area by various surgeons, are in contrast with those of Scheie, Schaffer, Plotkin, and Kertesz in the Philadelphia area. In their series, postoperative complications such as active inflammation, posterior synechia, updrawn pupils, and iris bombé were found in 22 of 49 eyes (44.9%). Thirteen eyes became phthisical, with inflammatory membranes in the anterior chamber and corneal opacification, and many eyes were enucleated. In that series microphthalmia was present in 26 eyes of 33 patients. In contrast, only two of the patients in our series had microphthalmia. These eyes had postoperative complications, and one eye was enucleated following surgery. 8

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Although the association of rubella with congenital cataracts has been long known, and many such cataracts have been treated surgically, there are no other reports on poor surgical results in rubella cataracts. A recent 25-year follow-up of 50 congenital rubella patients indicates that as adults they are employable, and maintain occupations that re-

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quire good visual acuity." Murphy and associates regarded their cataract surgery results as good in nine of 12 eyes from which rubella virus was isolated. Again, in that series a correlation appears to exist between the three cases with poor cataract surgery outcome, and the presence of microphthalmos. In 1946 Goar and Potts reported good results in cataract surgery performed in 10 eyes of five patients, using a Ziegler discission procedure. Alfano obtained good results in seven eyes in which a discission, alone or followed by aspiration of lens material, had been done. 10

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Viral, immunologic or epidemiologic factors may play a role in the rubella virus effects on the eye. The 1964-1965 rubella epidemic affected a significant number of pregnant mothers in Houston, and on the northeast coast of the United States. The St. Louis area did not show a significant increase in the incidence of maternal rubella. It could be assumed, then, that the rubella outbreaks in other parts of the country had certain characteristics that resulted in more severe teratogenic effects on newborns. In the eye, these were probably manifested as microphthalmos, and in such eyes surgery presents a risk with poor prognosis. 18

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Surgery on infants with congenital cataracts is imperative for the adequate development of the sensorimotor coordinations of the eye. In view of the tremendous visual implications of early cataract surgery, we suggest that rubella eyes without microphthalmos or marked iris and pupillary involvement be operated on at the earliest possible time. SUMMARY

Results in a series of 17 congenital rubella cataracts in 11 patients operated on in the St. Louis area indicated a good surgical prognosis for 15 ( 8 8 % ) of such eyes, compared to 90% of nonrubella eyes. The congenital rubella

S E P T E M B E R , 1968

eyes in which a good surgical result was obtained were neither microphthalmic nor did they have iris and pupillary abnormalities. The postoperative course and the incidence of eyes requiring needling of secondary membranes were remarkably similar in rubella ( 4 1 % ) and nonrubella eyes ( 4 2 % ) . The results suggest that rubella cataracts in eyes of normal size or in eyes without iris or pupil alterations, should undergo early surgery. 660 South Euclid Avenue (63110) REFERENCES 1. Cotlier, E., Fox, J. and Smith, M . : Rubella virus in the cataractous lens of congenital rubella syndrome. Am. J. Ophth. 62:233, 1966. 2. Reid, R. R., Murphy, A . M., Gillespie, A . M., Dormán, D. C , Menser, M . A., Hertzberg, R. and Harley, J. D. : Isolation of rubella virus from congenital cataracts removed at operation. Med. J. Australia 1:540, 1966. 3. Scheie, H . G., Schaffer, D. B., Plotkin, S. A . and Kertesz, E. D. : Congenital rubella cataracts. Arch. Ophth. 77:440, 1967. 4. Boniuk, M . and Zimmerman, L. E. : Ocular pathology in the rubella syndrome. Arch. Ophth. 77:455, 1967. 5. Scheie, H . G. : Aspiration of congenital or soft cataracts: A new technique. Am. J. Ophth. 50:1048, 1960. 6. Cordes, F. C. : Surgery of congenital cataracts. In Symposium on Diseases and Surgery of the Lens: Haik, G. M . ( e d ) S t Louis, Mosby, 1957, p. 85. 7. Parkman, P. D., Buescher, E. L. and Artenstein, N . S.: Recovery of rubella virus from army recruits. Proc Soc Exp. BioL Med. I l l :225, 1962. 8. Sever, J. L., Nelson, K. B. and Gilkeson, M . R . : Rubella epidemic 1964: Effect on 6,000 pregnancies. Am. J. Dis. Child. 110:395, 1965. 9. Menser, M . A., Dods, L. and Harley, J. D. : A twenty-five year follow-up of congenital rubella. Lancet 2:1347, 1967. 10. Murphy, A . M . , Reid, R. R., Pollard, I , Gillespie, A . M . , Dormán, D. C , Menser, M . A., Harley, J. D. and Hertzberg, R . : Rubella cataracts. Am. J. Ophth. 64:1109, 1967. 11. Goar, E. L. and Potts, C. R . : The relationship of rubella in the mother to congenital cataracts in the child. Am. J. Ophth. 29:566, 1946. 12. Alfano, J. E. : Ocular aspects of the maternal rubella syndrome. Tr. Am. Acad. Ophth. Otolaryng. 70:235, 1966. 13. Baylor Rubella Group: Rubella epidemic in retrospect Hosp. Practice 27(March), 1967.