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Elizabeth N. Kuhn, Betsy Hopson, Michael J. Conklin, and Jeffrey P. Blount

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 Children’s 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.

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Betsy Hopson, MSHA, Elizabeth N. Alford, Kathrin Zimmerman, Jeffrey P. Blount, and Brandon G. Rocque

OBJECTIVE

In spina bifida (SB), transition of care from the pediatric to adult healthcare settings remains an opportunity for improvement. Transition of care is necessarily multidimensional and focuses on increasing independence, autonomy, and personal responsibility for health-related tasks. While prior research has demonstrated that effective transition can improve health outcomes and quality of life while reducing healthcare utilization, little is known about the most advantageous transition program components/design. The individualized transition plan (ITP) was developed to optimize the readiness of the adolescent with SB for adult healthcare. The ITP is a set of clearly articulated, mutually developed goals that arise from best available data on successful transition and are individualized to meet the individual challenges, needs, and attributes of each patient and family.

METHODS

Prospectively completed ITPs were retrospectively reviewed from June 2018 to May 2019. Demographic and disease characteristics were collected, and specific goals were reviewed and categorized.

RESULTS

Thirty-two patients with an ITP were included. The cohort was 50% male and had a mean age of 16.4 years. For goal 1 (maximize education), the most common goal was to complete a career interest survey (44%), followed by researching application/admission requirements for programs of interest (25%), shadowing in and/or visiting a workplace (16%), and improving high school performance (16%). For goal 2 (bowel management), most patients (59%) had a working bowel program with few or no bowel accidents. Eight patients (25%) were having more than the desired number of bowel accidents and received formal consultation with a gastroenterologist. Five patients (16%) needed only minor adjustments to their bowel management regimen. Goal 3 (SB program coordinator goal) focused on documenting medical and/or surgical history for the majority of patients (66%). Other goals aimed to increase patient communication in healthcare settings or utilize available community resources.

CONCLUSIONS

The authors developed an evidence-based ITP that focuses around 5 goals: maximizing education, bowel continence, and goals set by the SB clinic coordinator, parent/caregiver, and patient. Although developed for the authors’ SB clinic, the ITP concept is applicable to transition of care in any chronic childhood illness.

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Brandon G. Rocque, Betsy D. Hopson, and Jeffrey P. Blount

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Daxa M. Patel, Brandon G. Rocque, Betsy Hopson, Anastasia Arynchyna, E. Ralee’ Bishop, David Lozano, and Jeffrey P. Blount

OBJECT

A paucity of literature examines sleep apnea in patients with myelomeningocele, Chiari malformation Type II (CM-II), and related hydrocephalus. Even less is known about the effect of hydrocephalus treatment or CM-II decompression on sleep hygiene. This study is an exploratory analysis of sleep-disordered breathing in patients with myelomeningocele and the effects of neurosurgical treatments, in particular CM-II decompression and hydrocephalus management, on sleep organization.

METHODS

The authors performed a retrospective review of all patients seen in their multidisciplinary spina bifida clinic (approximately 435 patients with myelomeningocele) to evaluate polysomnographs obtained between March 1999 and July 2013. They analyzed symptoms prompting evaluation, results, and recommended interventions by using descriptive statistics. They also conducted a subset analysis of 9 children who had undergone polysomnography both before and after neurosurgical intervention.

RESULTS

Fifty-two patients had polysomnographs available for review. Sleep apnea was diagnosed in 81% of these patients. The most common presenting symptom was “breathing difficulties” (18 cases [43%]). Mild sleep apnea was present in 26 cases (50%), moderate in 10 (19%), and severe in 6 (12%). Among the 42 patients with abnormal sleep architecture, 30 had predominantly obstructive apneas and 12 had predominantly central apneas. The most common pulmonology-recommended intervention was adjustment of peripheral oxygen supplementation (24 cases [57%]), followed by initiation of peripheral oxygen (10 cases [24%]).

In a subset analysis of 9 patients who had sleep studies before and after neurosurgical intervention, there was a trend toward a decrease in the mean number of respiratory events (from 34.8 to 15.9, p = 0.098), obstructive events (from 14.7 to 13.9, p = 0.85), and central events (from 20.1 to 2.25, p = 0.15) and in the apnea-hypopnea index (from 5.05 to 2.03, p = 0.038, not significant when corrected for multiple measures).

CONCLUSIONS

A large proportion of patients with myelomeningocele who had undergone polysomnography showed evidence of disordered sleep on an initial study. Furthermore, 31% of patients had moderate or severe obstructive sleep apnea. Myelomeningocele patients with an abnormal sleep structure who had undergone nonoperative treatment with peripheral oxygen supplementation showed improvement in the apnea-hypopnea index. Results in this study suggested that polysomnography in patients with myelomeningocele may present an opportunity to detect and classify sleep apnea, identify low-risk interventions, and prevent future implications of sleep-disordered breathing.

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Esther B. Dupépé, Daxa M. Patel, Brandon G. Rocque, Betsy Hopson, Anastasia A. Arynchyna, E. Ralee' Bishop, and Jeffrey P. Blount

OBJECTIVE

Although there are known risk factors for the development of neural tube defects (NTDs), little is known regarding the role of family history. The authors' goal in this study is to describe the family history in their population of patients with NTDs.

METHODS

Surveys were completed for 254 patients who were accompanied by their biological mother during their annual visit to the multidisciplinary Spina Bifida Clinic at Children's of Alabama. An NTD has been diagnosed in all patients who are seen in this clinic (myelomeningocele, lipomeningocele, split cord malformation, and congenital dermal sinus tract). Each mother answered questions regarding known NTD risk factors and their pregnancy, as well as the family history of NTDs, other CNS disorders, and birth defects.

RESULTS

The overall prevalence of family history of NTDs in children with an NTD was 16.9% (n = 43), of which 3.1% (n = 8) were in first-degree relatives. In patients with myelomeningocele, 17.7% (n = 37) had a positive family history for NTDs, with 3.8% in first-degree relatives. Family history in the paternal lineage for all NTDs was 8.7% versus 10.6% in the maternal lineage. Twenty-two patients (8.7%) had a family history of other congenital CNS disorders. Fifteen (5.9%) had a family history of Down syndrome, 12 (4.7%) had a family history of cerebral palsy, and 13 (5.1%) patients had a family history of clubfoot. Fourteen (5.5%) had a family history of cardiac defect, and 13 (5.1%) had a family history of cleft lip or palate.

CONCLUSIONS

The family history of NTDs was 16.9% in children with NTD without a difference between maternal and paternal lineage. This high rate of positive family history suggests that genetics and epigenetics may play a larger role in the pathogenesis of NTD in the modern era of widespread folate supplementation.

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Brandon G. Rocque, Raymond P. Waldrop, Isaac Shamblin, Anastasia A. Arynchyna, Betsy Hopson, Tammie Kerr, James M. Johnston, Curtis J. Rozzelle, and Jeffrey P. Blount

OBJECTIVE

Repeated failure of ventriculoperitoneal shunts (VPSs) is a problem familiar to pediatric neurosurgeons and patients. While there have been many studies to determine what factors are associated with the first shunt failure, studies of subsequent failures are much less common. The purpose of this study was to identify the prevalence and associated risk factors of clustered shunt failures (defined as 3 or more VPS operations within 3 months).

METHODS

The authors reviewed prospectively collected records from all patients who underwent VPS surgery from 2008 to 2017 at their institution and included only those children who had received all of their hydrocephalus care at that institution. Demographics, etiology of hydrocephalus, history of endoscopic third ventriculostomy or temporizing procedure, initial valve type, age at shunt placement, and other factors were analyzed. Logistic regression was used to test for the association of each variable with a history of shunt failure cluster.

RESULTS

Of the 465 included children, 28 (6.0%) had experienced at least one cluster of shunt failures. Among time-independent variables, etiology of hydrocephalus (OR 0.27 for non–intraventricular hemorrhage [IVH], nonmyelomeningocele, nonaqueductal stenosis etiology vs IVH, 95% CI 0.11–0.65; p = 0.003), younger gestational age at birth (OR 0.91, 95% CI 0.85–0.97; p = 0.003), history of a temporizing procedure (OR 2.77, 95% CI 1.12–6.85; p = 0.028), and smaller head circumference at time of initial shunt placement (OR 0.91, 95% CI 0.84–0.99; p = 0.044) showed significant association with shunt failure cluster on univariate analysis. None of these variables maintained significance in a multivariate model. Among children with a history of a shunt failure cluster, 21 (75%) had a shunt infection either prior to or during the shunt failure cluster. A comparison of the infecting organism between these children and 62 children with a history of infection but without a shunt failure cluster showed an association of cluster with gram-negative rod species.

CONCLUSIONS

Six percent of children in this institutional sample had at least one shunt failure cluster. These children accounted for 30% of the total shunt revisions in the sample. Shunt infection is an important factor associated with shunt failure cluster. Children with a history of prematurity and IVH may have a higher risk for failure cluster.

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Edward O. Komolafe, Ibironke O. Ogunbameru, Chiazor U. Onyia, Oluwafemi F. Owagbemi, and Fred S. Ige-Orhionkpaibima

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Brandon G. Rocque, E. Ralee' Bishop, Mallory A. Scogin, Betsy D. Hopson, Anastasia A. Arynchyna, Christina J. Boddiford, Chevis N. Shannon, and Jeffrey P. Blount

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.

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Dagoberto Estevez-Ordonez, Matthew C. Davis, Betsy Hopson, MSHA, Anastasia Arynchyna, Brandon G. Rocque, Graham Fieggen, Gail Rosseau, Godfrey Oakley, MSPM, and Jeffrey P. Blount

Neural tube defects (NTDs) are one of the greatest causes of childhood mortality and disability-adjusted life years worldwide. Global prevalence at birth is approximately 18.6 per 10,000 live births, with more than 300,000 infants with NTDs born every year. Substantial strides have been made in understanding the genetics, pathophysiology, and surgical treatment of NTDs, yet the natural history remains one of high morbidity and profound impairment of quality of life. Direct and indirect costs of care are enormous, which ensures profound inequities and disparities in the burden of disease in countries of low and moderate resources. All indices of disease burden are higher for NTDs in developing countries. The great tragedy is that the majority of NTDs can be prevented with folate fortification of commercially produced food. Unequivocal evidence of the effectiveness of folate to reduce the incidence of NTDs has existed for more than 25 years. Yet, the most comprehensive surveys of effectiveness of implementation strategies show that more than 100 countries fail to fortify, and consequently only 13% of folate-preventable spina bifida is actually prevented. Neurosurgeons harbor a disproportionate, central, and fundamental role in the management of NTDs and enjoy high standing in society. No organized group in medicine can speak as authoritatively or convincingly. As a result, neurosurgeons and organized neurosurgery harbor disproportionate potential to advocate for more comprehensive folate fortification, and thereby prevent the most common and severe birth defect to impact the human nervous system. Assertive, proactive, informed advocacy for folate fortification should be a central and integral part of the neurosurgical approach to NTDs. Only by making the prevention of dysraphism a priority can we best address the inequities often observed worldwide.

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Irene Kim, Betsy Hopson, Inmaculada Aban, Elias B. Rizk, Mark S. Dias, Robin Bowman, Laurie L. Ackerman, Michael D. Partington, Heidi Castillo, Jonathan Castillo, Paula R. Peterson, Jeffrey P. Blount, and Brandon G. Rocque

OBJECTIVE

Although the majority of patients with myelomeningocele have hydrocephalus, reported rates of hydrocephalus treatment vary widely. The purpose of this study was to determine the rate of surgical treatment for hydrocephalus in patients with myelomeningocele in the National Spina Bifida Patient Registry (NSBPR). In addition, the authors explored the variation in shunting rates across NSBPR institutions, examined the relationship between hydrocephalus, and the functional lesion level of the myelomeningocele, and evaluated for temporal trends in rates of treated hydrocephalus.

METHODS

The authors queried the NSBPR to identify all patients with myelomeningoceles. Individuals were identified as having been treated for hydrocephalus if they had undergone at least 1 hydrocephalus-related operation. For each participating NSBPR institution, the authors calculated the proportion of patients with treated hydrocephalus who were enrolled at that site. Logistic regression was performed to analyze the relationship between hydrocephalus and the functional lesion level of the myelomeningocele and to compare the rate of treated hydrocephalus in children born before 2005 with those born in 2005 or later.

RESULTS

A total of 4448 patients with myelomeningocele were identified from 26 institutions, of whom 3558 patients (79.99%) had undergone at least 1 hydrocephalus-related operation. The rate of treated hydrocephalus ranged from 72% to 96% among institutions enrolling more than 10 patients. This difference in treatment rates between centers was statistically significant (p < 0.001). Insufficient data were available in the NSBPR to analyze reasons for the different rates of hydrocephalus treatment between sites. Multivariate logistic regression demonstrated that more rostral functional lesion levels were associated with higher rates of treated hydrocephalus (p < 0.001) but demonstrated no significant difference in hydrocephalus treatment rates between children born before versus after 2005.

CONCLUSIONS

The rate of hydrocephalus treatment in patients with myelomeningocele in the NSBPR is 79.99%, which is consistent with the rates in previously published literature. The authors’ data demonstrate a clear association between functional lesion level of the myelomeningocele and the need for hydrocephalus treatment.