Life with spina bifida.

Abstract

Spina bifida is a broad term that includes minimal lesions such as spina bifida occulta and cystic swellings not containing any neural tissue (spina bifida with meningocele). Our main concern, however, is spina bifida with open myelomeningocele, in which the neural tissue is exposed on the surface of the cystic swelling. The neural tissue in this exposed plaque is abnormal and defective, so that the muscles innervated by it, and indeed distal to the plaque, may be partly or completely paralysed. Naturally, innervation of the bladder and bowels is almost always defective. In addition, about 90,, of the children with open myelo meningocele have hydrocephalus, but the degree of ventricular dilatation and intracranial tension varies and is not directly related to the appearance of the back lesion. If an infant had an operation to close the back wound in the neonatal period he might require a valve for the hydrocephalus within the first six to eight weeks. Complete paralysis of the legs may lead to no deformity whatever, but partial paralysis may lead to dislocation of the hip and to foot deformities. If it is decided to encourage the child to walk with appliances several operations may be needed on the feet and also a muscle transplant at the hip to keep the hip in joint after reduction. The other serious ortho paedic problem is the spinal deformity caused by unopposed action of certain muscles, which leads to kyphosis or kypho scoliosis and which may require major surgery for partial correction. In treating the hydrocephalus the excess ventricular fluid can be shunted through the Holter valve back into the bloodstream, and although some patients require revision of the valve several times in the first ten years, the hydrocephalus can be reasonably well controlled. We are learning in Sheffield how to prevent, and also recognise and treat, infection of the valve system; but as with any foreign body, the risk of infection remains. Defective bladder emptying may lead to stasis of urine within the renal tract and a tendency to infection, and in certain cases may lead to high pressures that cause the upper tract to dilate. Careful and frequent monitoring of the renal tract has enabled us to detect the early signs of trouble and give appropriate treatment, and there is no doubt that young children under treatment now have better kidneys than did children ten years ago. Even so, a ten-year review of survivors of an unselected series showed that nearly a quarter of them had normal or nearly normal bladder control. Expression of the bladder every two hours may enable the incontinent to be socially dry; but, if not, a penile appliance will help the boys, and an indwelling Foley catheter has been a tremendous boon in many girls. We still, however, have to undertake a urinary diversion in many of these patients. A typical 10-year-old with severe spina bifida will therefore spend most of his time in a wheelchair but can make some progress in long calipers; his hydrocephalus is controlled with a valve that may have needed two or three revisions during the growth period. Most children have an IQ within the normal range but below the mean; intellectual attainment will depend on the degree of hydrocephalus at the time when treatment was started, whether there has been infection, the speed with which obstruction has been relieved, and the opportunities for in tellectual stimulation. I noticed a remarkable change in the children when the spina bifida school was first opened in Sheffield, because many were receiving stimulation that had not been possible before. A boy will probably have a good upper renal tract and may be wearing a penile appliance if he cannot be kept dry by expression of the bladder. A girl may have an indwelling Foley catheter or may have needed an ileal conduit. This is not an unusual picture of the young person with spina bifida and hydrocephalus. I would emphasise that this is a *Paper presented at a symposium on Human Potential and the Physician at Withington Hospital, Manchester, on 17 September 1977.