The aim of the study was to document the frequency of neural tube defects (NTD) over a 20‐year period in Cape Town and to determine the effects of race, gender, maternal age, parity, and season of conception on the prevalence. Multiple sources of ascertainment were used, including all maternity hospital records, neurosurgical and spinal defects clinic data, as well as those from the Human Genetics Department and Fetal Abnormality Group. The prevalence rates for NTD fluctuated between 1.74 and 0.63 per 1,000 births, but showed no significant trends over the 20‐year period. Prevalence rates were highest for the white population group at 2.56 per 1,000 births compared to 0.95 per 1,000 for blacks and 1.05 per 1,000 for those of mixed ancestry. The higher rates in the whites, who are of British and European extraction and belong to the more affluent section of the community, would suggest that the possible effects of nutrition and infection are overshadowed by genetic factors. There was a female preponderance for both spina bifida (M: F ratio 0. 89) and anencephaly (M: F ratio 0. 67). The highest NTD rates were found at both ends of the maternal age range (<20 years and >35 years of age). The prevalence was highest at the extremes of birth order (1.65 and 1.58 for birth order 1and > 7, respectively, and 0.56 and 0.45 for birth order 5 and 6, respectively). A seasonal variation occurred which differed from that reported for the Northern Hemisphere and may reflect local climatic conditions. © 1994 Wiley‐Liss, Inc.

A cohort of South African children with myelomeningocele was followed for 5 years. Fifty-three were from metropolitan Cape Town and 65 from rural areas including the tribal region of Transkei. The mean general developmental quotient (GQ) at 5 years of age was lower than that previously reported. Black and coloured children had lower GQs than white children, but there were no significant differences between those from the urban and the rural areas. Early closure of the lesion and delivery by caesarean section were associated with higher levels of general developmental functioning. Central nervous system infections and the placement of more than one shunt for hydrocephalus resulted in lower GQs. Lesions above L2 were associated with non-ambulation. Ambulation was more likely in children in Cape Town than in rural children. Urinary incontinence occurred more frequently in rural children and among those in lower socio-economic circumstances.

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A prospective 2-year follow-up study of infants with birth weights of less than 1250 g was undertaken at Groote Schuur Hospital Neonatal Intensive Care Unit. For a 12-month period beginning July 1988, all live infants born at Groote Schuur Hospital or referred to the Neonatal Intensive Care Unit were included in the study cohort. The aim of the study was to document the morbidity, mortality, and neurodevelopmental outcome of these infants to 2 years of age. Of 235 liveborn infants, 143 (61%) survived to discharge. One hundred twenty-six infants were born weighing less than 1000 g; 42% survived to discharge. One hundred nine infants weighed 1000 g or more at birth, and 83% survived to discharge. Better survival was documented for infants whose mothers attended antenatal care, who weighed more than 900 g, and who were of greater than 30 weeks' gestation. Eleven infants died in the first 6 months after discharge. One hundred six infants (83% of survivors) underwent Griffiths developmental testing and clinical assessment at 1 year of age. Ninety-six (91%) of these survivors were seen and tested at 2 years of age. Of the 106 infants assessed at 1 year of age, 6 infants had cerebral palsy, 6 were globally developmentally delayed without signs of cerebral palsy, and 1 infant showed significant motor delay with a normal developmental quotient. At 2 years of age 1 additional infant had cerebral palsy and 9 more infants are likely to be mentally retarded. At 2 years of age the major handicap rate was, therefore, 22% . Sixty-nine percent of surviving infants, and all but 1 of the infants with cerebral palsy, were underweight for gestational age at birth. There was a tendency for these underweight-for-gestational-age infants to score less well at 2 years of age. Infants who received ventilation and infants with a birth weight of less than 1000 g were not found to score less well than other infants in the cohort.

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