17 February 1979 SA MEDICAL
Rubella Infection and Other Factors Associated with
Congenital Cataracts in Cape Town
A. KIPPS, D.SEVEL,
SUMMARY
An analysis of 56 patients with congenital cataracts was
made to detennine which factors played a role in the cau-
sation of these lenticular defects in Cape Town. There is
good evidence that 16% of the defects were caused by
intra-uterine rubella virus infection and that a further 30%
were hereditary in origin.
S. Atr. med. I., 55, 245 (1979).
Recent World Health Organization statistics estimate that
there are 40 million blind people in the world, and that
15 - 25 million blind people are suffering from preventable
or easily curable blindness.' Infectious agents are said to
be the most common cause of blindness, but congenital
rubella infection is not listed as one of them. Cataract
is noted as a 'frequent cause of blindness in older age
groups', but this specifically excludes the 'relatively rare
congenital conditions' which cause cataracts!
In order to gain some measure of the frequency with
which intra-uterine rubella infection might lead to cata-
ract formation in the local population, an analysis was
made of 56 patients with congenital cataracts seen in the
Cape Town group of teaching hospitals during the 7-year
period 1971 - 1977.
For the purpose of this investigation congenital cata-
racts are defined as those lens opacities which interfere
with normal vision, and which appear during the first
year of life; since they may not always be apparent at
birth.
MATERIALS AND METHODS
Lens aspirates were taken from one or both eyes of
48 patients for rubella virus isolation.
Blood samples did not regularly accompany the lens
material; from 15 patients lens material only was received.
From 8 patients with congenital cataracts blood alone
was received, because the lens aspiration had not yet been
performed. In a few instances blood was also received
from the patient's mother.
Rubella virus isolation and haemagglutination inhibition
(HAI) tests for rubella-specific IgO and IgM Il-globulins
were performed by methods described in an earlier article.'
Virus Research Unit, Department of Bacteriology, and De-
partment of Ophthalmology, University of Cape Town
A. KIPPS, M.D., F.R.C. PATH.
D. SEVEL, M.B. CH.B., PH.D., F.C.S. (S.A.), D.O. (Present address:
LSU Eye Center, New Orleans, Louisiana, USA)
.lA ET Md TYRE, M.B. B.S.
Date received: 3 October 1978.
JOURNAL 245
JANET McINTYRE
RESULTS
Of the 56 patients, 32 were male and 24 female. There
were 40 Coloured patients, 10 Whites and 6 Blacks. Nine-
teen of the patients were under the age of 12 months,
while the remaining 37 varied in age from 1 to 19
years. There were 44 patients with bilateral cataracts and
12 with unilateral lens opacities.
Congenital rubella infection was suspected on clinical
grounds in 9 of the 56 patients because of the presence
of one or more of the following features in addition to
the congenital cataract: congenital heart disease, deafness,
mental retardation or hepatosplenomegaly. In Table I
the patients are grouped according to the nature of the
specimens received in the laboratory. In group I, only
2 patients had clinical evidence of rubella, and from the
lenses of both of these patients rubella virus was isolated.
In group 11, 6 of the 8 patients had clinical signs sug-
gestive of congenital rubella. These 6 infants had rubella
IgO levels varying from 1 in 64 to 1 in 256, and in 3
of them rubella-specific IgM was demonstrated. One
patient in group III was suspected on clinical grounds
of having congenital rubella. The rubella IgO level was
1 in 64, but the test for IgM antibody was not done.
Three days after the birth of this child, however, the
mother's serum had a rubella antibody level of 1 in 256,
which might suggest that rubella infection had occurred
during pregnancy. These 9 patients were all under 9
months of age.
The laboratory tests thus confirmed the diagnosis in 5
of the 9 patients, and gave good support for the diagnosis
of congenital rubella in the remaining 4 patients. This sug-
gests that in 16°{, of the patients with congenital cataracts
rubella infection had played a part as a causal factor.
Congenital rubella infection was not seriously con-
sidered in the 22 patients who had no detectable rubella
antibodies at the lowest serum dilution (l : 8) tested. There
were, however, 13 patients in group TII with rubella HAI
antibodies varying in titre from 1 in 16 to 1 in 256. As
these patients were aged 1 - 19 years, postnatal infections
could not be excluded to account for the antibodies! None
had clinical evidence of congenital rubella.
Familial cataracts figured prominently. There were 13
children with one parent and / or one or more siblings
suffering from congenital cataracts. In one family, a
father and all 3 children had congenital cataracts, and in
another family tills was also the case with a mother and
all 3 of her children. In all 6 children the cataracts were
bilateral. Two other types of defect were encountered:
3 patients had Down syndrome (due to a well-defined
chromosomal abnormality) and 1 patient presented with a
mandibulofacial abnormality of the Pierre Robin syn-
drome (not so certainly familial in origin). In these 17
246 SA MEDIESE TYDSKRIF 17 Februarie 1979

TABLE I. ANALVSIS OF INVESTIGATIONS FOR RUBELLA VIRUS INFECTION OF 56 PATIENTS WITH CONGENITAL
CATARACTS

Number of Rubella Confirmation

patients Clinical virus Rubella Rubella of or good
with evidence of isolated HAI anti- HAI anti- support for
Specimens congenital congenital from lens bodies bodies not congenital
Group received cataract(s) rubella aspirate detected detected rubella
I Lens aspirate 15 2 2 2/2.
only
11 Blood only 8 6 6 2 6/6
III Lens aspira~e 33 1 0 13 20 111
and blood

Total 56 9 2 19 22 9/9
HAI haemagglutination inhibition.

patients, representing 30% of the total in this series, the The incidence of familial cataracts varies from 8% to
cataracts were most likely genetic in origin. 23%; excluding those associated with one of the numer-
ous syndromes caused by known genetic defects, of which
DISCUSSION Down syndrome is one, and the Pierre Robin syndrome
possibly another. In Table II the relative incidence of
The importance of a study of congenital cataracts in any rubella, and hereditary and other presumed causes of con-
geographical region is that some of them at least may be genital cataracts are shown by comparison of data from
preventable. Those caused by rubella can be forestalled by two reports with those of the present series. In patients with
immunization of the susceptible mothers-to-be; some of Down syndrome, abnormalities of the eyes are frequent,
those of hereditary origin by genetic counselling; and some with cataracts tending to develop at the end of the first
due to metabolic defects, e.g. galactosaemia, by early treat- decade, but congenital cataracts are much less common.
ment.' In many patients with congenital cataracts the familial
The problem involves not only those persons rendered origin of the cataract cannot be ascertained until subse-
totally blind, but also those whose vision is so impaired quent siblings or generations are born. Such examples are
as to limit development, education and employment, and included with the sporadic cases, and contribute to those
to enforce dependency on the community or family.' listed as being of unknown cause.
Patients with congenital rubella cataracts, for example, are
a severely handicapped group; those with bilateral cata-
racts, according to one report: were so subnonnal that 20 TABLE H. COMPARISON OF THE DISTRIBUTION OF
out of 31 were confined to institutions. Those with uni- CONGENITAL CATARACTS IN ASSOCIATION WITH OTHER
lateral cataracts were much better able to adapt to their FACTORS IN DIFFERING GEOGRAPHICAL REGIONS
handicaps. Cape Town
Rubella is a common cause of congenital cataracts, (56)
which results from a direct invasion of the lens by rubel- (present Australia" Nigeria'
la virus in up to 20% of all cases: This incidence is higher Association series) (136) (26)
in developed countries where rubella infections may be
Rubella C%) 16, t 21,1 7,7
delayed, leaving a greater proportion of pregnant women
Familial C%) 23,2 7,3 19,2
susceptible to infection than the 6 - 10% reported for Cape
Down syn-
Town" In this report 16% of children with congenital cata-
drome C%) 5,3 6,4
racts were presumed to have had intra-uterine rubella
Other syn-
infection, a figure which might have been greater if the
dromes C%) 1,8 5,5 3,8
13 patients in group III could have been more precisely
Unknown
assessed.
causes C%) 53,6 59,7 69,2
Not to consider the 22 patients with cataracts possibly
caused by rubella, but without detectable rubella anti-
bodies, may have been unjustified. It has been shown Certain metabolic disorders are associated with the de-
that, among 270 children known to have congenital rubel- velopment of congenital cataracts, but none was encoun-
la, 50 (18,5'%) had no detectable HAI antibodies by their tered in this series. Likewise, cytomegalovirus and toxo-
5th birthday.T Others have reported children with con- plasma infections in pregnancy may occasionally be as-
genital rubella, but in whom antibodies to rubella virus sociated with congenital cataracts. Antibodies to these two
were never detected. Nevertheless, there was no clinical agents were titrated in the available 41 sera, but in no
evidence of congenital rubella, although the ages of these single instance could an association between the agent and
22 patients varied from 4 months to 9 years. the cataract be established with any degree of certainty.
17 February 1979 SA MEDICAL
It is hoped that this brief analysis will stimulate an
interest in the problem and encourage practitioners to
make use of genetic counselling and rubella vaccination
to reduce the incidence of this serious physical handicap
and its inevitable consequences.
REFERENCES
1. Wkly epidem. Rec., 53, 165 (1978).
2. Leading article (1976): Brit. med. I., 2. 788.
Gemeenskapspsigiatrie en die Algemene Praktisyn
D. COETZEE
SUMMARY
As part of the development of a comprehensive com-
munity-orientated psychiatric service, a questionnaire with
mul.tiple-choice answers was sent to 244 general practi-
tioners in the catchment area of Stikland Hospital. The
attitudes and opinions expressed regarding certain prin-
-eiples of community psychiatry are analysed and dis-
cussed.
S. A fr. med. I., SS, 247 (1979).
Gemeenskapspsigiatrie beteken 'n omvattende, voorko-
mende diens in die gemeenskap. Minder klem word gele
op die psigiatriese hospitaal en meer klem op gemeen-
skapsorg. In beginsel verskil gemeenskapspsigiatrie nie van
goeie volksgesondheidsdienslewering nie. Dit dui ook op
'n diens aangepas by die behoeftes van 'n betrokke ge-
meenskap. Die bepaling van priotiteite met betrekking
tot dienslewering moet die behoeftes in ag neem. Gemeen-
skapspsigiatrie beteken ook diens gelewer deur lede van
'n betrokke gemeenskap. Die rol en verantwoordelikheid
van die gemeenskap wat betrefdie sorg van sogenaamde
psigiatriese pasiente word beklemtoon.
Die begrip 'gemeenskap' sluit al die professionele en
nie-professionele helpers van mense in die gemeenskap
in, asook die nodige fasiliteite vir die voorkoming en
behandeling van geestesongesteldheid.
In 'n gemeenskap-georienteerde program van psigiatriese
dienslewering sal die algemene praktisyn 'n sleutelrol moet
speel. Reeds in 1969 het die volgende stelling in 'n stan-
daardhandboek1 oor psigiatrie verskyn: '. . . in die toe-
komstige ontwikkeling van geestesgesondheidsdienste be-
hoort die algemene praktisyn 'n meer prominente rol te
speel in die behandeling van geestesstoomis. Die alter-
natief van 'n uitbreiding en proliferasie van psigiatriese
Departement Psigiatrie, Universiteit van Stellenbosc:h, Parow-
vallei en Stikland-hospitaal, BeUviIle, KP
D. COETZEE, M.B. CH.B., L.F. PSIG., Senior Lektor en Senior
Psigiater
Ontvangsdatum: 27 Iunie 1978.
10
JOURNAL 247
3. Merin, S. in Goldberg, M. F.. ed. (1974): Gen<tic and Metabolic
Eye Disease. Boston, Mass: Little, Brown.
4. Kipps, A., Moodie, I. W., Wychank, S. et al. (1977): S. Afr. med.
I., 52, 956.
5. Hall, D. (1969): Proc. roy. Soc. Med., 62, 694.
6. Gumpel, S.A. (1972): Arch. Dis. Childh., 47, 330.
7. Cooper, L. Z., Florman. A. L., Ziring, P. R. et al. (1971): Amer.
I. Dis. Child.• 122, 397.
8. Harley, I. D. and Hertzberg, R. (1965): Lancet, I, 1084.
9. Olurin, O. (1970): Amer. I. Ophtha!., 70, 533.
dienste is nog logies nog prakties uitvoerbaar.' Om te
bepaal wat die algemene praktisyn dink van die promi-
nente sleutelrol wat aan horn toegeken word, is 'n
vraelys opgestel en aan 'n groep geneeshere gestuur.
METODE
Die vraelys, met veelkeuse-antwoorde, het bestaan uit 20
items wat in die volgende kategoriee ingedeel kan word:
(i) besonderhede in verband met die algemene praktisyn
(opsioneel); (it) algemene houding en instelling teenoor
'senuweeprobleme' en psigiatrie, asook gevoel ten opsigte
van staats- psigiatriese hospitale (8 items); {iiO spesifieke
menings met betrekking tot sekere beginsels van gemeen-
skapspsigiatrie (l0 items); en (iv) algemene voorstelle,
probleme of kritiek met betrekking tot psigiatrie of
psigiatriese dienslewering (kortliks gespesifiseer).
Die geografiese gebied gedek was die volgende: die
suidelike en westelike Kaapprovinsie, waar Stikland-hospi-
taal en die Departement Psigiatrie, Fakulteit van Genees-
kunde, Universiteit van Stellenbosch, besig is met die
ontwikkeling van gemeenskapsdienste. Die totale area
kan as volg onderverdeel word: stedelike gebied -
noordelike voorstede (Kaapstad); groot dorpe - Paarl,
Wellington, Stellenbosch, Somerset-Wes, Strand, Malmes-
bury, Worcester, en Upington (buite die gemeenskapsbe-
dieningsgebied van Stikland-hospitaal); en kleiner platte-
landse dorpe.
Die vraelys is aan 244 algemene praktisyns gestuur
van wie 53 (21,7%) in die stedelike gebiede, 97 (39,7%)
in groot dorpe, en 94 (38,5'%) in kleiner dorpe gepraktiseer
het.
RESULTATE
'n Totaal van 52,4'% (128 uit 244) het hul Iyste terugge-
stuur; 58,4'% (31 uit 53) van die in die stedelike gebied,
58,7% (57 uit 97) van die in groot dorpe, en 42,5'% (40
uit 94) van die in kleiner dorpe.
Besonderhede insake opleiding is verstrek deur 121 ge-
neeshere: 29% het hul opleiding aan die Universiteit van
Stellenbosch gehad, 48% aan die Universiteit van Kaapstad,
17,3% aan die Universiteit van Pretoria, en 5,7% aan
die orige universiteite.