Betsy Hopson

Assessing health-related quality of life in children with spina bifida

OBJECT The purpose of this study is to explore various aspects of health-related quality of life (HRQOL) in children with spinal dysraphism. METHODS The authors enrolled a prospective cohort of 159 patients from the multidisciplinary spina bifida clinic. Surveys were distributed to caregivers of patients with spina bifida who were 5 years old and older. Data were collected using the Health Utilities Index Mark 3 focusing on vision, speech, hearing, dexterity, ambulation, cognition, emotions, and pain. Each participant received an overall HRQOL utility score and individual domain subscores. These were correlated with demographic and treatment variables. Analysis was done using SPSS statistics (version 21). RESULTS There were 125 patients with myelomeningocele, 25 with lipomyelomeningocele, and 9 with other dysraphisms. Among patients with myelomeningocele, 107 (86%) had CSF shunts in place, 14 (11%) had undergone Chiari malformation Type II decompression, 59 (47%) were community ambulators, and 45 (36%) were nonambulatory. Patients with myelomeningocele had significantly lower overall HRQOL scores than patients with closed spinal dysraphism. Among patients with myelomeningocele, younger patients had higher HRQOL scores. Patients with impaired bowel continence had lower overall HRQOL scores. History of a ventriculoperitoneal shunt was associated with worse HRQOL (overall score, ambulation, and cognition subscores). History of Chiari malformation Type II decompression was associated with worse overall, speech, and cognition scores. Patients who could ambulate in the community had higher overall and ambulation scores. A history of tethered cord release was correlated with lower pain subscore. No association was found between sex, race, insurance type, or bladder continence and HRQOL. CONCLUSIONS Patients with myelomeningocele have significantly lower HRQOL scores than those with other spinal dysraphisms. History of shunt treatment and Chiari decompression correlate with lower HRQOL scores.

Rate of shunt revision as a function of age in patients with shunted hydrocephalus due to myelomeningocele

OBJECTIVE It is generally accepted that cerebrospinal fluid shunts fail most frequently in the first years of life. The purpose of this study was to describe the risk of shunt failure for a given patient age in a well-defined cohort with shunted hydrocephalus due to myelomeningocele (MMC). METHODS The authors analyzed data from their institutional spina bifida research database including all patients with MMC and shunted hydrocephalus. For the entire population, the number of shunt revisions in each year of life was determined. Then the number of patients at risk for shunt revision during each year of life was calculated, thus enabling them to calculate the rate of shunt revision per patient in each year of life. In this way, the timing of all shunt revision operations for the entire clinic population and the likelihood of having a shunt revision during each year of life were calculated. RESULTS A total of 655 patients were enrolled in the spina bifida research database, 519 of whom had a diagnosis of MMC and whose mean age was 17.48 ± 11.7 years (median 16 years, range 0-63 years). Four hundred seventeen patients had had a CSF shunt for the treatment of hydrocephalus and thus are included in this analysis. There were 94 shunt revisions in the 1st year of life, which represents a rate of 0.23 revisions per patient in that year. The rate of shunt revision per patient-year initially decreased as age increased, except for an increase in revision frequency in the early teen years. Shunt revisions continued to occur as late as 43 years of age. CONCLUSIONS These data substantiate the idea that shunt revision surgeries in patients with MMC are most common in the 1st year of life and decrease thereafter, except for an increase in the early teen years. A persistent risk of shunt failure was observed well into adult life. These findings underscore the importance of routine follow-up of all MMC patients with shunted hydrocephalus and will aid in counseling patients and families.

Decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry

OBJECTIVEThe purpose of this study was to determine the rate of decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry (NSBPR). In addition, the authors explored the variation in rates of Chiari II decompression across NSBPR institutions, examined the relationship between Chiari II decompression and functional lesion level of the myelomeningocele, age, and need for tracheostomy, and they evaluated for temporal trends in rates of Chiari II decompression.METHODSThe authors queried the NSBPR to identify all individuals with myelomeningocele between 2009 and 2015. Among these patients, they identified individuals who had undergone at least 1 Chiari II decompression as well as those who had undergone tracheostomy. For each participating NSBPR institution, the authors calculated the proportion of patients enrolled at that site who underwent Chiari II decompression. Logistic regression was performed to analyze the relationship between Chiari II decompression, functional lesion level, age at decompression, and history of tracheostomy.RESULTSOf 4448 individuals with myelomeningocele identified from 26 institutions, 407 (9.15%) had undergone at least 1 Chiari II decompression. Fifty-one patients had undergone tracheostomy. Logistic regression demonstrated a statistically significant relationship between Chiari II decompression and functional lesion level of the myelomeningocele, with a more rostral lesion level associated with a higher likelihood of posterior fossa decompression. Similarly, children born before 2005 and those with history of tracheostomy had a significantly higher likelihood of Chiari II decompression. There was no association between functional lesion level and need for tracheostomy. However, among those children who underwent Chiari II decompression, the likelihood of also undergoing tracheostomy increased significantly with younger age at decompression.CONCLUSIONSThe rate of Chiari II decompression in patients with myelomeningocele in the NSBPR is consistent with that in previously published literature. There is a significant relationship between Chiari II decompression and functional lesion level of the myelomeningocele, which has not previously been reported. Younger children who undergo Chiari II decompression are more likely to have undergone tracheostomy. There appears to be a shift away from Chiari II decompression, as children born before 2005 were more likely to undergo Chiari II decompression than those born in 2005 or later.

Intradural spine surgery may not carry an increased risk of shunt revision compared with extradural spine surgery in pediatric patients with myelomeningocele

OBJECTIVE Patients with myelomeningocele are often affected by scoliosis and tethered cord syndrome, and frequently require spine surgery. Intradural spine surgeries may carry an inherently higher risk of inducing shunt malfunction due to entry into the subarachnoid space. In this study, the authors sought to compare rates of shunt malfunction after intradural and extradural spine surgeries among pediatric patients with myelomeningocele. METHODS The authors reviewed records of the National Spina Bifida Program Registry for Childrens Hospital of Alabama. The Exago reporting function was used to identify patients who had received at least one of the following procedures: shunt revision, tethered cord release (TCR), or spinal fusion for deformity. The registry records were reviewed for all identified patients to determine if a shunt revision was performed within the 1st year after TCR or spinal fusion. RESULTS Final analyses included 117 patients, of whom 39 underwent spinal fusion and 78 underwent TCR. Among patients who underwent spinal fusion, shunt revision was performed within 30 days in 2 patients (5.1%), within 60 days in 2 (5.1%), within 90 days in 4 (10.3%), and within 1 year in 5 (12.8%). Among patients who underwent TCR, shunt revision was performed within 30 days in 7 patients (9.0%), within 60 days in 10 (12.8%), within 90 days in 11 (14.1%), and within 1 year in 17 (21.8%). Using the log-rank test, there was no significant difference in Kaplan-Meier curves between intradural and extradural groups (p = 0.59). CONCLUSIONS In a review of single-institution registry data, the authors found no statistically significant difference in the risk of shunt malfunction after intradural and extradural spine surgeries.