✓The treatment of a patient with symptoms of a tethered spinal cord and in whom a fatty infiltrated terminal filum is found is controversial. The authors review their experience and the literature regarding this aspect of occult spinal dysraphism. From experience, transection of a fatty terminal filum in patients with symptoms related to excessive caudal cord tension is a minor procedure that generally yields good results. The most problematic issue in the literature is what patients and symptoms are best suited to surgical treatment.
Cuong J. Bui, R. Shane Tubbs, and W. Jerry Oakes
Christopher Carr, Lora Kahn, Mansour Mathkour, Erin Biro, Cuong J. Bui, and Aaron S. Dumont
The Global Burden of Disease (GBD) is an international collaboration and the largest comprehensive investigation of global health disease burden ever conducted. It has been particularly insightful for understanding disease demographics in middle-income nations undergoing rapid development, such as Vietnam, where 6 of the top 10 causes of death are relevant to the neurosurgeon. The burden of stroke—the number one cause of death in Vietnam—is particularly impressive. Likewise, road injuries, with a disproportionate rate of traumatic brain injury, continue to increase in Vietnam following economic development. Low-back and neck pain is the number one cause of disability. Simultaneously, more patients have access to care, and healthcare spending is increased.
It is imperative that neurosurgical capital and infrastructure keep pace with Vietnam’s growth. The authors searched the existing literature for assessments of neurosurgical infrastructure or initiatives to address neurosurgical disease burden. Using GBD data, the authors also abstracted data for death by cause and prevalence of years of life lost due to disability (YLD) for common neurosurgical pathologies for Vietnam and comparison nations.
Interventions aimed at primary prevention of risk factors for neurosurgical disease and focused on the transference of self-sustainable technical skills were found to be analogous to those that have been successful in other regions. Efforts toward stroke prevention have been focused on causal risk factors. Multiple investigators have found that interventions aimed at increasing helmet use were successful in preventing traumatic brain injury. Government-led reforms and equipment donation programs have improved technical capacity. Nevertheless, Vietnam lags behind other nations in neurosurgeons per capita; cause-attributable death and YLD attributable to neurosurgical disease are considerably higher in Vietnam and middle-income nations compared to both lower-income nations and upper-income nations.
More than two-thirds of deaths attributable to neurosurgical pathologies in Vietnam and other middle-income nations were due to stroke, and one-fifth of both cause-attributable death and YLD was associated with neurosurgical pathologies. Vietnam and other middle-income nations continue to assume a global burden of disease profile that ever more closely resembles that of developed nations, with particular cerebrovascular, neurotrauma, and spinal disease burdens, leading to exponentially increased demand for neurosurgeons that threatens to outpace the training of neurosurgeons.
R. Shane Tubbs, Cuong J. Bui, Marios Loukas, Mohammadali M. Shoja, and W. Jerry Oakes
The authors report on symptomatic patients with myelomeningocele (MMC) and lipomyelomeningocele (LMMC) who were found to have changes in their lumbosacral angle (LSA) corresponding to the onset of symptoms indicative of a tethered spinal cord.
The authors review data obtained in these two cohorts of patients and compare the LSAs measured in the perinatal period with those seen when the patients presented with symptoms of a tethered spinal cord.
Children with LMMC, roughly one third of studied cases, were symptomatic due to a tethered spinal cord at their most recent follow-up. In children in whom the MMC was the closed form at birth, 20 of 30 had symptoms that could be indicative of a tethered spinal cord at their most recent follow-up. The LSA was altered in both groups with symptoms.
Signs and symptoms indicative of a tethered spinal cord appear to correspond to increases in the LSA.
John C. Wellons III, R. Shane Tubbs, Cuong J. Bui, Paul A. Grabb, and W. Jerry Oakes
✓Patients with Chiari malformation Type I (CM-I) most commonly present with chronic symptoms. A search of the current medical literature revealed scant information regarding acute presentations of CM-I in either pediatric or adult patients. The authors report on two children who presented with rapidly worsening neurological symptoms attributable to a previously undiagnosed CM-I. One patient became profoundly hypopneic with dysphagia and right hemiparesis over a less than 48-hour period. The second patient presented with a few days of worsening right hemiparesis, gait disturbance, and anisocoria. In addition to a CM-I, magnetic resonance imaging in the second patient revealed a holocord syrinx. Following urgent posterior fossa decompression, both patients rapidly improved in the 24 hours immediately following surgery and continued to improve in the subsequent weeks. Few reports detail acute symptoms due to CM-I and those that do exist almost exclusively involve adult patients. Although seemingly rare, the clinician should consider CM-I in the differential diagnosis in pediatric patients presenting with acute brainstem or long tract signs.
Blake Pearson, Cuong J. Bui, R. Shane Tubbs, and John C. Wellons III
R. Shane Tubbs, Cuong J. Bui, Marios Loukas, Mohammadali M. Shoja, and W. Jerry Oakes
✓ Cerebral palsy is a common affliction in childhood. In some cases, the spasticity that often occurs can be treated with dorsal rhizotomies. Classically, these procedures have not been performed in children in whom there are known specific congenital brain malformations.
The authors report on two patients with holoprosencephaly and unilateral schizencephaly who underwent dorsal rhizotomy to treat their spasticity. The results were good. The long-term benefits during a mean follow-up period of 3.5 years included the transition from using a walker to quad canes for ambulation. Additionally, the outcomes in these two children appeared comparable to those found in other children with spastic diplegia undergoing dorsal rhizotomy at the authors' institution.
Dorsal rhizotomy may prove useful for treating spasticity in children with known congenital brain deformities.
Mansour Mathkour, Tyler Scullen, Brendan Huang, Cassidy Werner, Edna E. Gouveia, Hussam Abou-Al-Shaar, Christopher M. Maulucci, Rodney B. Steiner, Hugo St. Hilaire, and Cuong J. Bui
Split notochord syndrome (SNS) is a rare congenital defect of the central nervous system and has been associated with several anomalies affecting multiple organ systems. One association has been communication with the gastrointestinal tract and the spine, previously identified as a neuroenteric fistula (NEF). Here, the authors describe the unique case of a female infant with SNS and NEF treated with a multistage surgical repair. The three-stage operative plan included a two-stage repair of the defect and temporary subgaleal shunting followed by delayed ventriculoperitoneal shunt placement. The infant recovered well postsurgery and over a 5-year follow-up. A case description, surgical techniques, and rationale are reported. Additionally, a systematic review of the literature utilizing the MEDLINE database was performed.
Treatment of SNS with NEF using a multidisciplinary multistaged approach to repair the intestinal defect, close the neural elements, and divert cerebrospinal fluid to the peritoneum is shown to be a safe and viable option for future cases.
Cuong J. Bui, R. Shane Tubbs, Chevis N. Shannon, Leslie Acakpo-Satchivi, John C. Wellons III, Jeffrey P. Blount, and W. Jerry Oakes
There is scant literature regarding the long-term outcome in patients with cranial vault encephaloceles, and what literature there is may underestimate long-term deficits. The goal of this study was to address this lack of information.
The authors performed a retrospective chart review of cranial vault encephaloceles performed at our institution between 1989 and 2003. Fifty-two total patients were identified and 44 of these cases were reviewed. Additionally, 34 of the 44 patients were contacted and given an outcome survey (Hydrocephalus Outcome Questionnarie [HOQ]) to evaluate physical, emotional, cognitive, and overall health outcomes.
The mean age for patients in this cohort was 9.6 years (range 4–17 years) and the mean follow-up time was 9.2 years. There was an equal sex distribution and there were no deaths. Hydrocephalus was found in 60% of occipital and 14% of frontal encephaloceles, and epilepsy was confirmed in 17% of occipital and 7% of frontal lesions. Outcome assessments performed using the HOQ showed that 50% of the patients with occipital encephaloceles had overall HOQ health scores of 0.5 or less and 55% had HOQ cognitive scores of 0.3 or less, compared with 0% of patients in both categories who had frontal encephaloceles. It was also found that the presence of hydrocephalus and epilepsy independently and significantly lowered the overall health scores.
Occipital encephaloceles carry a worse prognosis than frontal encephaloceles, with higher rates of hydrocephalus and seizure. Based on this study, the presence of hydrocephalus and epilepsy are significant additive adverse prognostic factors. Approximately half of the patients with occipital encephaloceles will be severely debilitated and will probably be unable to live and function independently in society. These data may be useful to clinicians in counseling patients and predicting long-term outcome following repair of cranial vault encephaloceles.
R. Shane Tubbs, Cuong J. Bui, William C. Rice, Marios Loukas, Robert P. Naftel, Michael Paul Holcombe, and W. Jerry Oakes
Occasional comments are found in the literature regarding patients with lipomyelomeningocele and concomitant Chiari malformation Type I (CM-I). The object of this study was to explore the association between these two conditions.
The authors performed a retrospective database analysis of lipomyelomeningocele cases to identify cases of concomitant CM-I. Analysis of posterior fossa volume (based on the Cavalieri principle) was performed in all identified cases in which appropriate neuroimages were available, and the results were compared with those obtained in age-matched controls.
Seven (13%) of 54 patients with lipomyelomeningocele were found to also have CM-I. Two of these were symptomatic (cervicothoracic syrinx and occipital headaches) and required posterior fossa decompression. No correlation was found between the amount of hindbrain herniation and the level of the conus medullaris or the type of lipomyelomeningocele (for example, caudal or transitional). Volumetric studies of the posterior fossa revealed normal age-matched volumes in all but one patient (who had asymptomatic CM-I).
The incidence of CM-I in patients with lipomyelomeningocele appears to be significantly greater than that of the general population and the association rate is too high for the finding to be a chance occurrence. Decreases in the volume of the posterior cranial fossa were not found in the majority of patients in this small cohort; therefore, the cause of the concomitant occurrence of lipomyelomeningocele and CM-I remains undetermined. Clinicians should consider obtaining imaging studies of the entire neuraxis in patients with lipomyelomeningoceles and should investigate other causes for syringes found in association with lipomyelomeningoceles.