Spina bifida outcome: a 25-year prospective.

BACKGROUND: Open spina bifida is the most complex congenital abnormality compatible with long-term survival. This report outlines the 20- to 25-year outcome for our original cohort of patients with a myelomeningocele treated in a nonselective, prospective manner. METHODS: Of the initial 118 children, 71 patients were available for our most recent review. Nineteen patients have been lost to follow-up and 28 patients have died. Data were collected on: motor level, shunt status, education/employment, seizure history, mobility, bladder/bowel continence, tethered cord, scoliosis, latex allergy, posterior cervical decompression, tracheostomy and/or gastrostomy tube. RESULTS: Mortality (24%) continues to climb into young adulthood. Eighty-six percent of the cohort have cerebrospinal fluid diversion, with 95% having undergone at least one shunt revision. Thirty-two percent have undergone a tethered cord release, with 97% having an improvement or stabilization in their preoperative symptoms. Forty-nine percent have scoliosis, with 43% eventually requiring a spinal fusion. Sixteen patients (23%) have had at least one seizure. Eighty-five percent are attending or have graduated from high school and/or college. More than 80% of young adults have social bladder continence. Approximately 1/3 of patients are allergic to latex, with 6 patients having experienced a life-threatening reaction. CONCLUSION: At least 75% of children born with a myelomeningocele can be expected to reach their early adult years. Late deterioration is common. One of the greatest challenges in medicine today is establishing a network of care for these adults with spina bifida.

Outcome data and analysis in pediatric neurosurgery.

OBJECTIVE: The purpose of this study was to analyze the outcomes of five commonly performed pediatric neurosurgical operations: 1) initial shunt insertion; 2) first shunt revision; 3) craniotomy for brain tumor; 4) correction of sagittal synostosis; and 5) release of tethered cords. A second purpose was to analyze the neurological outcome data after tethered cord releases. METHODS: Morbidity and mortality records, patient charts, and operative records were reviewed to determine length of hospitalization and, for each disorder, the pertinent outcomes such as duration of shunt function and incidence of infection or neurological morbidity. RESULTS: Many outcome data were expected, such as a high long-term shunt function rate after primary shunt insertion (65% at 5 yr), a low mortality rate (1%) and permanent morbidity rate (10%) after craniotomy for brain tumor, and a low frequency of transfusion (20%) for sagittal synostosis operations. The outcomes among the three neurosurgeons varied more than expected, e.g., the duration of hospitalization after sagittal reconstructions ranged from 3.1 to 5.8 days; the frequency of infections of primary shunt revisions ranged from 0 to 15%; and the neurological morbidity after tethered cord releases ranged from 0 to 12%, with all neurological morbidity occurring in patients undergoing their second or third tether release. CONCLUSION: The data may serve as a basis for outcome comparisons for these procedures. Outcome data allow us to analyze factors to improve patient care, but outcome analysis is complex.

Open spina bifida: outcome for a complete cohort treated unselectively and followed into adulthood.

Between 1963 and 1970, 117 consecutive cases of open spina bifida were treated unselectively from birth, following a full neurological examination. The author reviews the outcome 16 to 20 years later. 48 died before their sixteenth birthday. Of the 69 survivors, 60 had had a shunt inserted and two were blind following shunt dysfunction; 22 were mentally retarded (IQ less than 80); 35 were wheelchair-dependent; 52 were incontinent, and 32 of these continued to need help with its management; nine weighed greater than 75 kg; one-third of both sexes had a precocious puberty; three had insulin-dependent diabetes; 12 continued to need anticonvulsant drugs; 32 had suffered from pressure sores; 33 were unable to live without help or supervision and only 17 were capable of open employment. These findings demonstrate the wide range of disabilities implied by the diagnosis of open spina bifida. The neurological findings recorded at birth were reflected in the mortality and disability of the survivors. The sensory level represented a yardstick for predicting the likely range of handicap into adult life, provided no additional impairment resulted from serious complications: such damage occurred in seven of the survivors.

Open spina bifida: does cesarean section delivery improve prognosis?

Records were reviewed retrospectively on 72 infants with open spina bifida followed from birth through one year of age. Thirty-two infants were born by cesarean section and 40 vaginally. The following variables were compared between the two groups: 1) mortality in the nursery and between nursery discharge and one year of age, 2) incidence of meningitis in the neonatal period, 3) length of initial hospital stay, and 4) neurologic and developmental status at one year. No significant differences were noted between the two groups. Although it has been suggested that cesarean section may improve the prognosis for infants with open spina bifida, our data do not support that conclusion.

A cohort study of neural tube defects (NTD) in Denmark covering the first seven years of life.

In the County of Fyn, Denmark, which is a well-delimited region comprising a 10% representative sample of the total population, ten complete one year cohorts of newborn were scrutinized for neural tube defects (NTD). Among 66,000 births, 71 cases with NTD were observed. The total prevalence at birth was 1.08/1,000; 0.29 for anencephaly; 0.20 for encephalocele, and 0.59 for spina bifida (SB). This is at the same level as in other Scandinavian countries. Considering only neonates born alive, 57% of the total survived until the age of 7 years, disregarding anencephaly, but only 4 of 18 SB cases and 6 of 11 encephalocele cases had no handicaps at age 7. On the average, SB patients spent 9% of their life before the age of 7 years in hospitals and encephaloceles 2%. The SB cases occupied 25 times more beds per year than the comparable age group in general and many were likely to need continued medical and social care.

[Evaluation of the incidence of anencephaly and spina bifida in Brittany (1975-1984)]. / Evaluation de l'incidence de l'anencéphalie et du spina bifida en Bretagne (1975-1984).

Brittany is celtic, like Ireland and Wales where the incidence of neural tube defects is raised. We searched the hospital files in Brittany for all live and still births, and terminations of pregnancy after prenatal diagnosis for the years 1975-1984. 225 cases of spina bifida and 210 cases of anencephaly were identified; giving an incidence of 0.60 per 1000 births for spina bifida and 0.56 per 1000 births for anencephaly. No seasonality was found for both malformations. Analysis of the sex ratio for anencephaly indicated significantly higher proportion of females to males. Maternal age in the affected group was similar to the normal population. Casual heterogeneity among neural tube defects patients was presumed because 14% of our cases had other congenital anomalies.

Survival of infants with spina bifida--Atlanta, 1972-1979.

We studied the survival of a population-based cohort of 154 infants with spina bifida who were born during the eight-year period from 1972 through 1979 to residents of Atlanta. Our objectives were to describe the cohort's survival experience and to explore relationships between clinical characteristics and survival. Overall, 57% of the cohort survived one year or more. This figure is misleading, however, because it conceals important differences in survival among subgroups of affected infants. From univariate analyses, we observed significant differences in survival among infants categorized by year of birth, birth weight, the open-closed status of the defect, the highest level of the defect on the spine, the presence of multiple major birth defects, and the presence of hydrocephalus at birth. More infants born in the late 1970s survived their first year of life than infants born in the early 1970s; infants with open defects had lower survival than those with closed defects; and infants whose defects were low on the spine had better survival than those whose defects were higher. When comparing the survival experience of this cohort with that of other groups from other areas or from more recent years, health workers must consider referral biases and differences in the distribution of clinical characteristics.

Fetal mortality in sibships of cases with neural tube defects.

It has been suggested that rates of fetal mortality in sibships of probands with a malformation inherited as a multifactorial threshold trait may reflect their liability to the malformation. If so, spontaneous abortion rates should be more frequent in sibships thought to have greater liability. For anencephaly and spina bifida (ASB), then, spontaneous abortion should be higher in the sibships of male probands and in families with more than one affected case (multiplex families). This hypothesis was tested using data on cases from The Montréal Children's Hospital and from the literature. Approximately 5000 pregnancies were analyzed. Rates of abortion did not vary with the sex or diagnosis of the proband. The spontaneous abortion rate was slightly higher in multiplex than in simplex families, but the difference was not statistically significant and most likely reflects the differing reproductive patterns in the two types of families. Thus, if male probands and multiplex sibships do have, on average, more liability for ASB, this liability cannot be detected in spontaneous abortion rates in the sibships available for this analysis.

Spina bifida and anencephaly in Scotland.

Data obtained from routine sources showed that from 1971 to 1982 the birth prevalences of spina bifida and anencephaly in Scotland fell. When known terminations after routine alpha fetoprotein screening were added to total births the adjusted birth prevalence could be calculated. In 1974-82 this fell by 40% for spina bifida (3 X 0-1 X 8) and 36% for anencephaly (2 X 2-1 X 4). These findings were compared with data on birth prevalences in England and Wales, Northern Ireland, and Glasgow. The fall in birth prevalences of spina bifida and anencephaly over the past decade appears to have been due both to a true fall in incidence as well as to increased screening and termination for these conditions.

Neural tube defects in New South Wales: management and preventive policies, 1965-1980.

This paper reviews the results of the programme of antenatal testing and selective termination of fetuses with anencephaly and spina bifida in New South Wales over the last eight years. It discusses the problems associated with pregnancy screening, with reference to those involved in selection for treatment of newborn infants with spina bifida. The incidence of neural tube defects has been declining, and this, although possibly the result of early termination of affected fetuses, may have its basis in a worldwide trend. Preventive action based on a more precise understanding of aetiological factors will further decrease the incidence, thereby reducing the load of long-term handicapped survivors.

Implications of a selective policy in the management of spina bifida.

Of 307 patients with myelomeningocele 132 were treated nonoperatively during the 6 yr period from 1973 to 1978. Of the nonoperatively managed patients, 86% died within 1 yr. The policy of nonoperative management was changed to one of active intervention in 25% of the infants. Despite this a large number died.

The mortality and birth rates of spina bifida during a period of treatment, selection and antenatal screening in Sheffield, 1963-1978.

The birth rates for all neural tube defects and the mortality rates from spina bifida and encephalocele have been obtained for Sheffield children by examining the congenital anomalies register and death certificates during the period from 1963-1978. The introduction of atenatal alpha foetoprotein screening of all pregnant women in 1977 is associated in time with a sharp fall in the birth rate of neural tube defects. The mortality rate for spina bifida with encephalocele rose gradually from 1967 to 1976, apparently unaffected by the introduction of clinical selection prior to surgery in 1971. The antenatal screening of previously affected families (since 1973) did not affect the mortality rate, but screening the total pregnant population since 1977 has been associated with a sharp reduction in the mortality rate. The mortality rate curve for spina bifida with encephalocele is markedly different from the birth rate curve which makes the common practice of inferring birth rates from mortality rates invalid. There was a gradual increase from 1967 to 1978 in the proportion of children born with spina bifida in any given year who died before their second birthday. This increase appears to have been uninfluenced by the introduction of selection or antenatal screening. The changes in mortality rate and survival may be as much due to unknown factors as to changes in medical management.

Practical problems in selection of spina bifida infants for treatment in the USA.

Seventy-five spina bifida infants were studied for results of selectin for treatment. The 31 nonoperated infants had a survival rate of 70% at 18 months, a figure higher than anticipated. Practical difficulties in adhering to the selectin protocol and controlling medical management in a large North American city were noted. Thirty-three surveyed parents of surgically repaired infants reported satisfaction with family function during the 18 months follow-up but discrepant scores increased with time.