David B. Shurtleff

CESAREAN SECTION BEFORE THE ONSET OF LABOR AND SUBSEQUENT MOTOR FUNCTION IN INFANTS WITH MENINGOMYELOCELE DIAGNOSED ANTENATALLY

Methods. To assess the effect of labor and type of delivery on the level of motor function in fetuses with uncomplicated meningomyelocele, we identified 200 cases of this disorder, accounting for 95 percent of the cases that occurred in the state of Washington during our 10-year study period. We compared the outcomes of 47 infants delivered by cesarean section before labor began, 35 delivered by cesarean section after a period of labor, and 78 who were delivered vaginally (another 40 were ineligible for the study)

Long-Term Survival of Individuals with Myelomeningocele

The objectives of this study were to extend survival analysis into adulthood for patients with myelomeningocele (MM) and to compare survival curves for patients born with varying defect severity before and after 1975. We have reviewed existing data for 904 patients with MM seen in a large multidisciplinary children’s clinic over 43 years. Before 1975, a major contributor to decreased survival is death during infancy. The presence of cerebral spinal fluid shunting is a major contributor to increased survival. After 1975, survival to adolescence is similar regardless of shunt status (p = 0.17). For all patients alive at age 16, a significant decrease in survival probability after age 34 years was found for individuals with shunted hydrocephalus compared to those without a shunt (p = 0.03). Although childhood survival for individuals born after 1975 is not related to shunt status, adults with MM and shunted hydrocephalus may be at risk for decreased longevity.

Experience with 1201 cerebrospinal fluid shunt procedures

This report of our experience with 1,201 cerebrospinal fluid shunt procedures over a 17-year period includes two time periods: from 1965 to 1974 and from 1975 to 1982. The overall operative infection rate fell from 10.7% in the former period to 3.6% in the latter period. The overall average number of revisions fell from 1.8 to 0.77. The number of revisions for patients with infected shunts in the latter period was 4.07. Three hundred and eighty-four shunts were studied in the latter period for the effects of prophylactic antibiotics. No significant difference was found between the infection rate of 5.6% in the nonantibiotic group and the infection rate of 2.1% in the group receiving intravenous and intraventricular methicillin and gentamicin. Differences in the operative infection rates for patients with and without meningomyelocele and those operated on by different surgeons were not significant. The only mortalities were from nonoperative and noncerebrospinal fluid shunt causes.

Influence of Prognosis on Decisions Regarding the Care of Newborns with Myelodysplasia

Treatment of newborns with myelodysplasia (meningomyelocele and related disorders) continues to be a controversial subject. We have used a consistent plan of care and have employed the same prognostic criteria over the period from 1965 to 1982 to address the needs of 212 affected newborns. A good prognosis and early surgical care were given to 42 per cent of 53 newborns during the period 1965 to 1970, to 58 per cent of 65 newborns from 1971 to 1976, and to 71 per cent of 94 newborns from 1977 to 1982. Of the newborns with an initially poor prognosis, 19 per cent of 31 received early surgery between 1965 and 1970, as compared with 33 per cent of 27 between 1971 and 1976 and 52 per cent of 27 between 1977 and 1982. Life-table analyses of survival in the three periods revealed significant improvement over time in the survival of newborns receiving early surgical care, regardless of the initial prognosis (log-rank statistic = 8.240, P = 0.016) and in comparison to recipients of supportive care alone (log-rank statistic = 5.975, P = 0.05). We conclude that early surgery permits the survival of an increasing percentage of patients with myelodysplasia.

Myelodysplasia: Decision for Death or Disability

Abstract Treatment of myelodysplastic patients must avoid neglecting a potentially functional child on the one hand and the perpetuation of rejected, brain-damaged cripples on the other. Immediate, complete assessment for brain mass and function and other system anomalies were documented before treatment of 371 patients, with fully informed consent. Of these, 283 had maximum treatment because their brain mass was estimated to be 60 per cent of normal, or greater, and there were no noteworthy complications. The others were treated symptomatically. Seventy-five per cent of patients of normal intellect but paralyzed at high and 90 to 95 per cent of those paralyzed at low levels survived with maximum treatment. Of the patients treated only symptomatically because of criteria excluding maximum treatment, 10 per cent survived to the age of 2 1/2 years. There was a slightly better survival in a group treated symptomatically (42 per cent survival to teen age) before establishment of selection criteria. Children s...

Acquisition of autonomy skills in adolescents with myelomeningocele

This study describes ages of acquisition (AOA) of typical adolescent autonomy skills in a regional cohort of individuals with myelomeningocele (MM), aged 12 to 18 years, with a mean age of 14 years 11 months (SD 2y 5mo). Prospectively collected data over 10 years were analyzed. Regression analysis, using Generalized Estimation Equation, provided 50th centile and 75th centile AOA for each skill. One hundred and fifty‐eight participants (90 males, 68 females) attended 378 annual patient visits. Patient contacts were equally distributed across age and physical severity groupings. Twenty‐four percent of participants had functional lesion levels at or above L2, and 38% between L3‐L5, 38% at S1 or below. Eighty‐four percent had shunted hydrocephalus, 24% were independent in ambulation, and 69% achieved toileting independence before adolescence. AOA for autonomy skills were delayed by 25 to 30% when compared with typically developing adolescents. Differences in cognitive ability explained the variance in median ages for skill acquisition (p=0.01) more than physical lesion level. Participants acquired community skills at a median age of 16 years 6 months. Twelve percent of 18‐year‐olds drove cars (9% with supervision, 3% independently), regardless of physical lesion severity. We conclude that adolescents with MM acquire the majority of autonomy skills 2 to 5 years later than their typically developing peers. This study establishes AOA timelines for autonomy skills in adolescents with MM.

A LONGITUDINAL STUDY OF SHUNT FUNCTION IN 360 PATIENTS WITH HYDROCEPHALUS

Data from an eight‐year clinical study of shunt‐treated hydrocephalus indicated continued shunt dependency in 289 of 360 patients. 919 combined pressure‐radionuclide clearance studies were performed. In 307 studies in which the shunt was identified as nonfunctional, 208 patients were symptomatic for increased intracranial pressure and had the shunt revised. Of the other 99 patients, 71 have remained asymptomatic for three to 60 months (mean 27 months) and 28 subsequently required shunt revision after one to 67 months (mean 24 months). Five other clinical patterns are described. Various methods of monitoring shunt function are recommended.

Epidemiology of neural tube defects and folic acid

This review article combines four disparate observations about Neural Tube Defects (NTDs). They are the worldwide decline in the birth incidence that began prior to prenatal diagnosis; family recurrence risks; the effect of prenatal diagnosis and termination of affected pregnancies; and the effect of folic acid.

Myelodysplasia: The Natural History of Kyphosis and Scoliosis. A Preliminary Report

The results of this study support the concept that the levels of the bifid vertebral arch and of functional motor activity are significant influences on the development of scoliosis and kyphosis in patients born with myelodysplasia. ‘Significant’ degrees of scoliosis and kyphosis may appear at any time during life, but most frequently occur during late childhood or adolescence. Not all ‘significant’ curvatures require treatment, nor do all children with myelodysplasia and factors predisposing to kyphosis and scoliosis develop these complications. It is believed that the preliminary cumulative percentage curves presented in this study will be of value in counselling parents of afflicted newborn infants, and in planning the management of patients born with myelodysplasia.

MOBILITY AIDS FOR CHILDREN WITH HIGH‐LEVEL MYELOMENINGOCELE: PARAPODIUM VERSUS WHEELCHAIR

The optimal method for providing mobility for children with myelomeningocele remains controversial. 39 children using a parapodium were compared with 29 children in wheelchairs. There were no significant differences between the two groups for medical complications, use of health‐care services or activities of daily living, although there were differences in the patterns of complications. Children using the parapodium were more likely to develop lesions of the lower extremities, to have dislocated hips, to be more obese and to watch more television; children using wheelchairs were more likely to develop lesions of the gluteal region, to have knee‐flexion contractures and to have fewer fecal accidents. The parapodium was judged by families to be less effective as a mobility aid; however, the upright posture it allows was considered extremely advantageous. A combined approach allowing upright posture and wheeled mobility would appear to be optimal.