School-aged children with spina bifida in Western Australia--parental perspectives on functional outcome.

A questionnaire mailed to the parent(s) of all 86 school-aged children with spina bifida resident in Western Australia in 1992 who were attending a spinal dysfunction clinic or were members of the Spina Bifida Association was returned by 72 parents. All 72 of these children (55 with myelomeningocele, 17 with meningocele) lived at home with their parent(s). All but two children with meningocele were mobile without aids, whereas only 42% of the children with myelomeningocele were. Twenty one per cent of the children were naturally continent of urine day and night, and 36% were naturally continent of faeces. 76% attended a mainstream school although performance at school was rated below average for 40%. Nine children with meningocele (56%) and 42 with myelomeningocele (76%) were reported to have learning difficulties. This information will be useful in counselling parents of unborn and newborn children with spina bifida, and in allocating resources for children and young adults with spina bifida and their families.

Survival of infants with neural tube defects in Western Australia 1966-1990.

To investigate the survival of infants with neural tube defects in Western Australia from 1966 to 1990, cases of neural tube defects were ascertained from multiple sources. Survival of infants with anencephaly, spina bifida, and encephalocoele was examined separately, and four birth cohorts were compared: 1966-1972 (when most surviving infants were actively treated), 1973-1979 (a period of stringent application of selection criteria for treatment), 1980-1985 (some relaxation of selection criteria), and 1986-1990 (further relaxation of selection criteria). There was an increase in terminations of pregnancies affected with anencephaly over the study period, and a fall in the proportion of both liveborn and stillborn infants with anencephaly. Most liveborn infants (76.4%) died in the first 24 hours, and none survived longer than 5 days. Most infants with encephalocoele (76.4%) were liveborn, and survival was poorest in the 1973-1979 cohort, although the difference in survival across cohorts just failed to attain a formal level of significance. For spina bifida, there was an increase in terminations of affected pregnancies over the study period. Survival of liveborn infants with spina bifida was poorest in the 1973-1979 cohort, and greatest in the most recent cohort. These improvements in survival are likely to be the result of relaxation of selection criteria for treatment, improvements in treatment and, to a lesser extent, selective termination of affected pregnancies.