Presented at the Neonatal Society 2007 Spring Meeting.
Anthony MY1, Calvert JK1,2, Boyd PA2,3, Chamberlain PC3, Redshaw M2, Kurinczuk JJ2
1 Neonatal Unit, John Radcliffe Hospital, Oxford, UK
2 National Perinatal Epidemiology Unit, University of Oxford, UK
3 Department of Prenatal Diagnosis, John Radcliffe Hospital, Oxford, UK
Aims: To document the outcome of babies born alive with spina bifida from one tertiary centre in order to provide recent data and information for parents when spina bifida is diagnosed prenatally.
Methods: All cases of spina bifida born from 1991 to 2003 were ascertained from the Oxford Congenital Anomaly Register, the Neonatal Unit register and histopathology reports. The case notes of live born infants were studied for details of cerebral ventriculomegaly at birth, level of lesion, mobility at latest assessment, continence and schooling difficulties.
Results: A total of 139 pregnancies were identified. 24 babies survived beyond the late neonatal period. 23 case notes were traceable. Twenty of the 23 delivered between 37 and 42 weeks of gestation. The age at the last assessment ranged from 4 years to 15 years, median 8 years. There were two sacral lesions, 8 low lumbar, 6 medium thoraco-lumbar and 5 high thoraco-lumbar. Five were skin covered. Ventricular size was recorded in 20 cases. Seven had normal ventricles at birth. One had marked dilatation. Five children did not need postnatal shunts. Mobility: data were available for 21 cases, 7 were in wheelchairs, 3 could stand, 7 walk and 4 run. Development: information was available for 20 cases, 1 had severe problems, 5 moderate problems and 14 were considered normal developmentally. Continence: data were available on 20, 16 were incontinent of urine, and 13 incontinent of faeces. Ventriculomegaly: All wheelchair bound children and those who could only stand, and all of the severe or moderately disabled developmentally had ventriculomegaly at birth. Of the 4 normally mobile children data were available for 3 and they had normal ventricles at birth. Of the 14 with normal development data were available for 11 and of these 8 had normal ventricles at birth.
Skin covering conferred some advantage, but not universally. Mobility was affected by the level of the lesion but not directly. The level was not an indicator of those children who would need later surgery for posterior fossa decompression or other spinal surgery. After the late neonatal period there were no deaths.
Conclusions: This small cohort of selectively treated babies differs from those previously described in the literature (1,2). Shunt problems and renal complications were less than in previously reported cohorts. Incontinence is the commonest problem and ventriculomegaly at birth is an important prognostic feature. A parallel study has shown that if this is present at birth it was very likely to have been present at the 20 week anomaly scan.
Acknowledgements:
References
1. Hunt G. Dev Med & Child Neuro 1990; 32: 108-118.
2. Hunt G, Oakeshott P. BMJ 2003; 326: 1365-1366