The Natural History of Spina Bifida Cystica: Detailed Analysis of 407 Cases

Abstract

This investigation was based on 407 children with spina bifida cystica born between 1947 and 1956, seen at The Hospital for Sick Children or the Westminster Children''s Hospital, London, by the end of 1957. All cases were followed up in the summer of 1958 and the survivors were re-examined. It was considered appropriate to subdivide them into meningoceles and myeloceles only. The series was found to be fairly representative of the disease for infants surviving beyond the age of 3 months, and 55% of the whole series were still alive. When prognosis is calculated on actuarial principles, using a life table, it is found that only 35. 4% of cases attending a large children''s hospital can be expected to survive to 12 years. The meningoceles, which are a difficult diagnostic problem and which formed 9% of the series, all survived. Only a few suffered from mild paralysis or hydrocephalus. The 368 myeloceles had an expectation of survival to the age of 12 years of 29%. Their neurological condition usually becomes worse soon after birth, probably because of biochemical change, stretching or infection of the neural plate. Nevertheless, the pattern of paralysis appeared to be largely determined by the position and extent of the lesion, with maximal skeletal paralysis with lumbar lesions, while sphincter paralysis became more frequent and severe the lower the lesion along the neural axis. Moderate or severe hydrocephalus that is due to malformations or post-inflammatory lesions from ascending meningitis, or one superimposed upon the other, was present in 235 cases. It was clinically present at birth in at least 28% of these and was diagnosed in all but one by 6 months. The incidence was highest in the dorsi-lumbar (88%) and lowest in the sacral myeloceles (37%). Death occurred soon except in the encephaloceles (40%) who seem to have a better prognosis with regard to survival but not intelligence. In 53 cases arrest took place spontaneously, mostly between the ages of 9 months and 1 year and 9 months, and in 3 following an operation; 9 of these died subsequently from other causes. 185 cases died, all but 3 having hydrocephalus of moderate or severe degrees. Infection was the commonest immediate cause (83 cases) followed by progressive hydrocephalus (59 cases). Eight cases died of renal failure or infection. Although intracranial infection was the commonest cause of death the development of hydrocephalus appears to be a grave prognostic sign, as comparatively few of these cases survived. It is suggested that results may well be improved if the raw spinal lesion is covered with skin grafts as a surgical emergency within hours of birth without dissecting any neural elements from the sac wall or even removing part of the sac. This should lessen the risk of damage to any functioning cord tissue in the myelocele and also reduce the incidence of ascending infection and aggravation of any hydrocephalus. The mortality in this condition should also be reduced as a result of the newer surgical methods for the treatment of hydrocephalus.