What has changed in pediatric neurosurgical care in spina bifida? A 30-year UAB/Children's of Alabama observational overview.

Spina bifida (SB) remains the most serious and most common congenital anomaly of the human nervous system that is compatible with life. The open myelomeningocele on the back is perhaps the most obvious initial problem, but the collective impact of dysraphism upon the entirety of the nervous system and innervated organs is an equal or greater longitudinal threat. As such, patients with myelomeningocele (MMC) are best managed in a multi-disciplinary clinic that brings together experienced medical, nursing, and therapy teams that provide high standards of care while studying outcomes and sharing insights and experiences. Since its inception 30 years ago, the spina bifida program at UAB/Children's of Alabama has remained dedicated to providing exemplary multi-disciplinary care for affected children and their families. During this time, there has been great change in the care landscape, but many of the neurosurgical principles and primary issues have remained the same. In utero myelomeningocele closure (IUMC) has revolutionized initial care and has favorable impact on several important co-morbidities of SB including hydrocephalus, the Chiari II malformation, and the functional level of the neurologic deficit. Hydrocephalus however is not solved by IUMC, and hydrocephalus management remains at the center of neurosurgical care in SB. Ventricular shunts were long the cornerstone of treatment for hydrocephalus, but we came to assess and incorporate endoscopic third ventriculostomy with choroid plexus coagulation (ETV-CPC). Educated and nurtured by an experienced senior mentor, we dedicated ourselves to fundamental concepts but persistently evaluated our care outcomes and evolved our protocols and paradigms for improvement. Active conversations amidst networks of treasured colleagues were central to this development and growth. While hydrocephalus support and treatment of tethered spinal cord remained our principal neurosurgical charges, we evolved to embrace a holistic perspective and approach that is reflected and captured in the Lifetime Care Plan. Our team engaged actively in important workshops and guideline initiatives and was central to the development and support of the National Spina Bifida Patient Registry. We started and developed an adult SB clinic to support our patients who aged out of pediatric care. Lessons there taught us the importance of a model of transition that emphasized personal responsibility and awareness of health and the crucial role of dedicated support over time. Support for sleep, bowel health, and personal intimate cares are important contributors to overall health and care. This paper details our growth, learning, and evolution of care provision over the past 30 years.

Global Neurosurgery: Progress and Resolutions at the 75th World Health Assembly.

Neurosurgical advocates for global surgery/neurosurgery at the 75th World Health Assembly gathered in person for the first time after the COVID-19 pandemic in Geneva, Switzerland, in May 2022. This article reviews the significant progress in the global health landscape targeting neglected neurosurgical patients, emphasizing high-level policy advocacy and international efforts to support a new World Health Assembly resolution in mandatory folic acid fortification to prevent neural tube defects. The process of developing global resolutions through the World Health Organization and its member states is summarized. Two new global initiatives focused on the surgical patients among the most vulnerable member states are discussed, the Global Surgery Foundation and the Global Action Plan on Epilepsy and other Neurological Disorders. Progress toward a neurosurgery-inspired resolution on mandatory folic acid fortification to prevent spina bifida-folate is described. In addition, priorities for moving the global health agenda forward for the neurosurgical patient as it relates to the global burden of neurological disease are reviewed after the COVID-19 pandemic.

The role of neurosurgeons in global public health: the case of folic acid fortification of staple foods to prevent spina bifida.

OBJECTIVE: The global neurosurgery movement arose at the crossroads of unmet neurosurgical needs and public health to address the global burden of neurosurgical disease. The case of folic acid fortification (FAF) of staple foods for the prevention of spina bifida and anencephaly (SBA) represents an example of a new neurosurgical paradigm focused on public health intervention in addition to the treatment of individual cases. The Global Alliance for the Prevention of Spina Bifida-F (GAPSBiF), a multidisciplinary coalition of neurosurgeons, pediatricians, geneticists, epidemiologists, food scientists, and fortification policy experts, was formed to advocate for FAF of staple foods worldwide. This paper serves as a review of the work of GAPSBiF thus far in advocating for universal FAF of commonly consumed staple foods to equitably prevent SBA caused by folic acid insufficiency. METHODS: A narrative review was performed using the PubMed and Google Scholar databases. RESULTS: In this review, the authors describe the impact of SBA on patients, caregivers, and health systems, as well as characterize the multifaceted requirements for proper spina bifida care, including multidisciplinary clinics and the transition of care, while highlighting the role of neurosurgeons. Then they discuss prevention policy approaches, including supplementation, fortification, and hybrid efforts with folic acid. Next, they use the example of FAF of staple foods as a model for neurosurgeons' involvement in global public health through clinical practice, research, education and training, and advocacy. Last, they describe mechanisms for involvement in the above initiatives as a potential academic tenure track, including institutional partnerships, organized neurosurgery, neurosurgical expert groups, nongovernmental organizations, national or international governments, and multidisciplinary coalitions. CONCLUSIONS: The role of neurosurgeons in caring for children with spina bifida extends beyond treating patients in clinical practice and includes research, education and training, and advocacy initiatives to promote context-specific, evidence-based initiatives to public health problems. Promoting and championing FAF serves as an example of the far-reaching, impactful role that neurosurgeons worldwide may play at the intersection of neurosurgery and public health.

Preventing birth defects, saving lives, and promoting health equity: an urgent call to action for universal mandatory food fortification with folic acid.

July 20, 2021 marked the 30th anniversary of the publication of the landmark trial by the British Medical Research Council showing unequivocally that maternal intake of folic acid (vitamin B9) starting before pregnancy prevents most cases of infant spina bifida and anencephaly-two major neural tube defects that are severe, disabling, and often fatal. Mandatory food fortification with folic acid is a safe, cost-effective, and sustainable intervention to prevent spina bifida and anencephaly. Yet few countries implement fortification with folic acid; only a quarter of all preventable spina bifida and anencephaly cases worldwide are currently avoided by food fortification. We summarise scientific evidence supporting immediate, mandatory fortification with folic acid to prevent the development of spina bifida and anencephaly. We make an urgent call to action for the World Health Assembly to pass a resolution for universal mandatory folic acid fortification. Such a resolution could accelerate the slow pace of spina bifida and anencephaly prevention globally, and will assist countries to reach their 2030 Sustainable Development Goals on child mortality and health equity. The cost of inaction is profound, and disproportionately impacts susceptible populations in low-income and middle-income countries.

Program Evaluation of Camp V.I.P: Promoting Self-confidence and Independence for Patients with Spina Bifida.

PURPOSE: Camp V.I·P (Victory, Independence, Possibilities) was established by the Spina Bifida Association of Alabama in 2012. The goal is to provide children with spina bifida (SB) and their families a traditional-style summer camp focusing on self-empowerment and fostering independence in medical self-management. DESIGN AND METHODS: Part 1 of this study describes Camp V.I·P. structure. Part 2 describes an online survey to caregivers of campers gauging camp effectiveness and value and evaluating perceptions of confidence/independence and family connections. Camper characteristics were compared to the overall SB clinic population. RESULTS: 63 children with SB, 81 siblings, and over 100 caregivers have attended camp. Campers are similar to the overall clinic population in race, gender, and ambulation status. At camp, there are significantly more children with myelomeningocele (versus other spinal dysraphism) and lumbar functional level (versus thoracic). Survey results show that confidence grew in 93% of campers, and 86% gained greater independence. Parents stated that they created lifelong friendships. The majority of caregivers (91%) indicated "certainty" that they would attend camp again. CONCLUSION: Camp V.I·P is a family-centered environment that emphasizes respite, nurture, and the continuum between family and clinic. Our research shows that camp can be a useful tool to aid in independence, confidence, and social adaption. PRACTICE IMPLICATIONS: Camp has become an integral part of our comprehensive care of children with SB and has been shown to reinforce what is taught through the clinic by creating trust between the care team and campers.

Reducing inequities in preventable neural tube defects: the critical and underutilized role of neurosurgical advocacy for folate fortification.

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.

Approach to the cervical portion of the vagus nerve via the posterior cervical triangle: a cadaveric feasibility study with potential use in vagus nerve stimulation procedures.

OBJECT: The authors describe a technique in which the cervical portion of the vagus nerve is exposed during procedures such as neuroma resection or, more commonly, during the placement of a vagus nerve stimulator. METHODS: To test their hypothesis that a posterolateral approach to the vagus nerve may be feasible and efficacious, the authors performed dissection of the left-sided vagus nerve in 13 adult cadavers. The carotid sheath was exposed via the posterior cervical triangle, and the vagus nerve was identified posterolaterally. Measurements were made of the length of available nerve, and the anatomical approach was documented. As part of a comparison study regarding the available length of nerve, the authors exposed the left vagus nerve in five additional adult cadavers via a standard anterior approach to the carotid sheath, and compared the results obtained with each technique. A mean length of 12 cm of the vagus nerve was isolated when using the posterior approach to the carotid sheath, whereas a mean length of 11 cm of the nerve was documented when using the anterior approach. With the aforementioned posterior approach, no obvious injury occurred to the vagus nerve or other local neurovascular structures such as the spinal accessory nerve. CONCLUSIONS: Evaluation of the findings obtained in the present cadaveric study showed that a posterior approach to the vagus nerve is feasible. The technique for posterior exposure of the carotid sheath may prove useful in surgical exposures of the vagus nerve when a standard anterior method is not possible.

Vagus nerve stimulation in children less than 5 years old.

INTRODUCTION: Vagus nerve stimulation (VNS) has been used in both adults and older children with varying success. MATERIALS AND METHODS: We retrospectively reviewed our experience with VNS in very young children (below 5 years old). The mean age at stimulator implantation was 20.5 months. Two patients were below 2 years old at implantation and two patients were below 1 year old at their initial surgery. The average follow up time for this group was 22 months. RESULTS: Of the six patients (three males and three females) with long-term follow up, 83% had a significant decrease in the frequency of their seizure. Of these, two are seizure-free (33%), three are improved (50%), and one (17%) has had no change in seizure status at their most recent clinical examination. Age at implantation of the vagus nerve stimulator did not seem to correlate with patient success. In this group, atonic seizures were found to best respond to VNS with cessation of this type of seizure in two patients. No patients were made worse by the procedure and no morbidity was observed related to VNS. CONCLUSIONS: Based on our small patient cohort, it appears that VNS in very young children with life-threatening epilepsy can be efficacious. Larger groups and other institutional experiences are now needed to verify our findings.

The use of a sump antibiotic irrigation system to save infected hardware in a patient with a vagal nerve stimulator: technical note.

The authors describe the use of a sump irrigation system that was used to successfully treat the battery implantation site of a vagal nerve stimulator (VNS). Irrigation was composed of a dilution of vancomycin in lactated Ringer's solution. At long-term follow up, the patient has not returned with signs or symptoms of wound infection. She continues to effectively combat her epilepsy with VNS. The authors believe this to be the first description of this technique for salvaging an implanted VNS.

Vagus nerve stimulation for induced spinal cord seizures: insights into seizure cessation.

OBJECT: Vagus nerve stimulation is known to decrease the frequency, duration, and intensity of some types of intracranial seizures in both humans and animals. Although many theories abound concerning the mechanism for this action, the true cause remains speculative. To potentially elucidate a pathway in which vagus nerve stimulation aborts seizure activity, seizures were initiated not in the cerebral cortex but in the spinal cord and then vagus nerve stimulation was performed. METHODS: Ten pigs were anesthetized and placed in the lateral position, and a small laminectomy was performed in the lumbar region. Topical penicillin, a known epileptogenic drug to the cerebral cortex and spinal cord, was applied to the dorsal surface of the exposed cord. With the exception of two animals that were used as controls, once seizure activity was discernible via motor convulsion or increased electrical activity the left vagus nerve, which had been previously isolated in the neck, was stimulated. Following multiple stimulations of the vagus nerve and with seizure activity confirmed, the cord was transected in the midthoracic region and vagus nerve stimulation was performed. Vagus nerve stimulation resulted in cessation of spinal cord seizure activity in all (87.5%) but one experimented animal. Transection of the spinal cord superior to the site of seizure induction resulted in the ineffectiveness of vagus nerve stimulation to cause cessation of seizure activity in all study animals. CONCLUSIONS: The effects of vagus nerve stimulation on induced spinal cord seizures involve descending spinal pathways. The authors believe that this experiment is the first to demonstrate that spinal cord neuronal hyperactivity can be suppressed by stimulation of a cranial nerve. These data may aid in the development of alternative mechanisms for electrical stimulation in patients with medically intractable seizures. Further studies are now necessary to isolate which specific tracts, nuclei, and neurotransmitters are involved in this process.

Complications of chronic vagus nerve stimulation for epilepsy in children.

OBJECT: The aim of this study was to define better the incidence of surgical complications and untoward side effects of chronic vagus nerve stimulation (VNS) in a population of children with medically refractory epilepsy. METHODS: The authors retrospectively reviewed the cases of 74 consecutive patients (41 male and 33 female) 18 years of age or younger (mean age 8.8 years, range 11 months-18 years) who had undergone implantation of a vagal stimulator between 1998 and 2001 with a minimum follow up of 1 year (mean 2.2 years). Of the 74 patients treated, seven (9.4%) had a complication ultimately resulting in removal of the stimulator. The rate of deep infections necessitating device removal was 3.5% (three of 74 patients who had undergone 85 implantation and/or revision procedures). An additional three superficial infections occurred in patients in whom the stimulators were not removed: one was treated with superficial operative debridement and antibiotic agents and the other two with oral antibiotics only. Another four stimulators (5.4%) were removed because of the absence of clinical benefit and device intolerance. Two devices were revised because of lead fracture (2.7%). Among the cohort, 11 battery changes have been performed thus far, although none less than 33 months after initial implantation. Several patients experienced stimulation-induced symptoms (hoarseness, cough, drooling, outbursts of laughter, shoulder abduction, dysphagia, or urinary retention) that did not require device removal. Ipsilateral vocal cord paralysis was identified in one patient. One patient died of aspiration pneumonia more than 30 days after device implantation. CONCLUSIONS: Vagus nerve stimulation remains a viable option for improving seizure control in difficult to treat pediatric patients with epilepsy. Surgical complications such as hardware failure (2.7%) or deep infection (3.5%) occurred, resulting in device removal or revision. Occasional stimulation-induced symptoms such as hoarseness, dysphagia, or torticollis may be expected (5.4%).

Alternative uses for the subgaleal shunt in pediatric neurosurgery.

The subgaleal shunt has been used for the temporary bypass of the normal cerebrospinal fluid (CSF) pathways. We retrospectively reviewed all subgaleal shunts placed at the Children's Hospital, Birmingham, Ala., USA, from 1997 to the present and examined all uses (e.g. indication, length of follow-up) of the subgaleal shunt outside its use for temporary CSF diversion in premature infants with intraventricular hemorrhage and subsequent hydrocephalus. The average length of survival of the primary subgaleal shunt in this population was 32.2 days. We have had good success with subgaleal shunts in children with malignant brain tumors, intraventricular abscesses, chronic truncal wounds, chronic subdural hygromas and meningitis. However, the greatest utility has been in those scenarios in which the peritoneal cavities were not currently, but with time would be, candidates for distal shunt implantation. Examples of these instances are patients with hydrocephalus and necrotizing enterocolitis or hydrocephalus and preoperative abdominal wall pathology such as omphalocele.