Spina Bifida Outcome: A 25-Year Perspective
by E.M. Bowman, D.G. McLone, J.A. Grant, T. Tomita, and J.A. Ito, Pediatric Neurosurgery 2001;34:114–120.
Knowing the long-term impairments and functional limitations affecting individuals with spina bifida assists physical therapists in designing plans of care across the patient’s life-span. The purpose of this study was to examine outcomes of patients with open myelomeningocele who had undergone surgical repair during a 20- to 25-year period. The following variables were examined: motor level, shunt status, education/employment, seizure history, mobility, bladder/bowel continence, tethered cord, scoliosis, latex allergy, posterior cervical decompression, tracheostomy, and gastrostomy tube.
This study is an extension of an earlier study involving 118 children who had undergone closure of an open myelomeningocele at Children’s Memorial Hospital in Chicago, Ill, between 1975 and 1979. Of the initial 118 children, 71 patients were available for inclusion in this study. Nineteen patients had moved, and 28 patients had died. Of the 71 patients, 59 were actively followed in the hospital follow-up clinic, which allowed retrieval of detailed clinic data. The remaining 12 patients were interviewed to obtain recent information. A retrospective chart review was conducted of the patients who had been lost to follow-up or had died. Data were collected on the following parameters: motor level, shunt status, education/employment, seizure history, mobility, bladder/bowel continence, tethered cord, scoliosis, latex allergy, posterior cervical decompression, tracheostomy, and gastrostomy tube.
Motor levels, based on a manual motor test, were categorized by the authors as follows: thoracic (thoracic and L1), L2/L3, L4, L5, sacral, and asymmetrical. Of the 71 subjects in the study group, 25 were categorized at the thoracic level, five at the L2/L3 level, 14 at the L4 level, 11 at the L5 level, 15 at the sacral level, and one was classified as asymmetrical. When compared with the motor level at birth, motor level had remained stable in 73%, had improved in 11%, and had worsened in 16%. In the cohort of the 28 who died, 40% were categorized at the thoracic level, 14% at the L2/L3 level, 11% at the L4 level, 26% at the L5 level, and 11% at the sacral level.
Of the 71 subjects in the study group, 86% had cerebrospinal fluid shunts. Nine-five percent had undergone at least one shunt revision. Forty-one percent had undergone two to three shunt revisions. All 16 patients who had a seizure disorder had a shunted hydrocephalus.
Education, employment, and living arrangements.
Eighty-five percent of the subjects were either attending high school or college. Sixty-three percent attended regular classes without additional assistance. Fourteen percent required additional assistance with their studies, and 23% were in special education. Forty-five percent were employed in the community, and 18% volunteered in their community. Seventy-seven percent of the subjects lived at home with their parents. Fifteen percent lived independently, and 4% were living in residential homes. Two young adults were married and lived with their spouses. No offspring were reported from the study cohort.
Mobility decreased from early childhood to the early teen years. However, the subjects who remained mobile in their teens continued to ambulate the majority (75% to 100%) of the time in their young-adult years. Of the 71, 33 (47%) continued to ambulate. Nine ambulated 25% to 50% of the time, and 29 (41%) relied solely on a wheelchair for mobility. The group of patients with the greatest lower extremity motor function had the highest percentage of individuals who ambulated. Fourteen of the 15 patients with a sacral motor level ambulated 100% of the time. The other patient with a sacral myelomeningocele used a wheelchair to navigate around his college campus. Ten of the 11 young adults (91%) with an L5 motor level ambulated the majority of the time, compared with 57% of the patients with an L4 level. None of the patients with a thoracic/L1 or L2/3 level relied on ambulation for the majority of their mobility.
Bladder and bowel continence.
Eighty-five percent of the study group was maintained on clean intermittent catheterization of their bladder. Ninety percent of the patients perform their own catheterization. Fifteen percent always had urinary continence. Eighty-nine percent reported bowel control the majority (75% to 100%) of the time. Only 38% were actively involved in a bowel program.
Tethered cord and scoliosis.
A tethered cord, requiring surgical release, developed in 23 of the 71 patients. The average age at which the tethered cord became symptomatic was 10.9 years. The most common symptoms associated with the tethered cord were increased scoliosis, gait changes, and spasticity. Some patients reported back pain, decreased muscle strength, lower extremity contractures, or urinary bladder changes. Postoperatively, 71% had improvement in the preoperative symptoms. Nearly half of the study group (35 of 71) exhibited scoliosis. More than 40% of the patients with scoliosis (15 of 35) required spinal fusion.
Twenty-three patients (32%) had an allergic reaction to latex; six of these patients had a severe, life-threatening anaphylactic reaction. The average age at which the patients became allergic to latex was 12.5 years.
Posterior cervical decompression, tracheostomy, and gastrostomy.
Six percent of the patients in the study group had undergone posterior cervical decompression, 6% had undergone tracheostomy, and 4% have a gastrostomy tube. The percentages were higher in the group of patients who died.
Although the outlook for patients with a myelomeningocele has improved over the past 30 years, very little information has been published about the long-term prognosis and early adult-onset problems associated with spina bifida. This study revealed that the majority of the patients had maintained stable motor function into early adulthood. Eleven percent of patients had improved motor strength, and 16% exhibited a decline in motor function by at least one level. After ruling out shunt malfunction, a tethered spinal cord was the second most common reason for loss of function. Regarding mobility, most patients with L5 or sacral level motor function continued to ambulate into early adulthood. Of the 71 subjects, nearly 50% had some degree of scoliosis. Eighty-five percent of the young adults in the study attended or graduated from high school and/or college. Most of the study group resided with their parents; however, 11 lived independently. Approximately one-third of the study group was allergic to latex. Six patients had experienced a severe, life-threatening reaction to latex. Achievement of social bladder and bowel continence seemed to be a realistic goal, with 80% of the study group continent the majority of the time. Although renal failure had been previously published as a cause of death, no deaths secondary to renal failure were noted.
In addition to examining the impairments and functional limitations of the study group, the charts of the patients who had died were examined. Twenty-four percent of the original cohort had died. The majority of deaths occurred during infancy and the preschool years, with a plateau during the school age and early teen years. However, the death rate continued to climb into early adulthood, with the most common cause of death being unrecognized shunt malfunction. Hunt1 suggested a 1% risk of death per year from age five to 30 years for all patents with open myelomeningocele.
Implications and Limitations
Individuals born with myelomeningocele require medical follow-up throughout their lives. At least 75% of children born with spina bifida are expected to reach their early adult years. This study suggests that death can occur at any age because of complications associated with spina bifida. It is important for physical therapists to be aware of signs associated with shunt malfunction and a tethered cord. In this study, the most common symptoms associated with a tethered cord were increased scoliosis, gait changes, and spasticity. Some patients reported back pain, decreased muscle strength, lower extremity contractures, or urinary tract changes. Changes in any of these areas warrant notification of the patient’s physician. In addition, physical therapists treating individuals with spina bifida need to be aware of the possibility of latex allergy. Allergic reactions to latex, some life-threatening, were noted in nearly one third of the patients in the study group. The authors suggest following appropriate precautions for use of latex materials when treating all patients with spina bifida.
Physical therapists providing intervention services to younger children with spina bifida may be asked by the family, “What does the future hold for our child in regard to education beyond high school and living independently in the community?” This study suggests that the majority of individuals with spina bifida will attend or graduate from high school or college. The majority of the patients in this study group reside with their parents. Although the authors do not question why or pose an explanation, it would be valuable to determine if the patients in the study resided at home because of financial reasons, need for personal care assistance, or lack of handicapped-accessible housing.
The authors conclude that as the individual with spina bifida ages, the medical problems will also increase and could include increased depression, visual impairments, severe renal disease, obesity, hypertension, and pressure sores. With this in mind, physical therapists must be prepared to design plans of care sensitive to lifetime changes for individuals with spina bifida.
The primary limitation of this study is the incomplete information retrieved from the charts or obtained from the interviews. Tools that assess functional limitation and disability across the life-span were not available when the subjects in this study were first examined. However, achievements in self-care and home management (activities of daily living and instrumental activities of daily living) at the time of the interview could have been assessed more thoroughly. A detailed investigation of why the subjects still lived in their parents’ home or why only 50% were employed would also have been valuable.
Lana Svien, MA, PT, University of South Dakota