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Zachary S. Hubbard, Tsun Yee Law, Samuel Rosas, Sarah C. Jernigan and Harvey Chim

OBJECTIVE

The epidemiology of carpal tunnel syndrome (CTS) has been extensively researched. However, data describing the economic burden of CTS is limited. The purpose of this study was to quantify the disease burden of CTS and determine the economic benefit of its surgical management.

METHODS

The authors utilized the PearlDiver database to identify the number of individuals with CTS in the Medicare patient population, and then utilized CPT codes to identify which individuals underwent surgical management. These data were used to calculate the total number of disability-adjusted life years (DALYs) associated with CTS. A human capital approach was employed and gross national income per capita was used to calculate the economic burden.

RESULTS

From 2005 to 2012 there were 1,500,603 individuals identified in the Medicare patient population with the diagnosis of CTS. Without conservative or surgical management, this results in 804,113 DALYs without age weighting and discounting, and 450,235 DALYs with age weighting and a discount rate of 3%. This amounts to between $21.8 and $39 billion in total economic burden, or $2.7–$4.8 billion per year. Surgical management of CTS has resulted in the aversion of 173,000–309,000 DALYs. This has yielded between $780 million and $1.6 billion in economic benefit per year. Endoscopic carpal tunnel release provided between $11,683 and $23,186 per patient at 100% success while open carpal tunnel release provided between $10,711 and $22,132 per patient at 100% success. The benefit-cost ratio at its most conservative is 2.7:1, yet could be as high as 6.9:1.

CONCLUSIONS

CTS is prevalent in the Medicare patient population, and is associated with a large amount of economic burden. The surgical management of CTS leads to a large reduction in this burden, yielding extraordinary economic benefit.

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Sarah C. Jernigan, Scellig S. D. Stone, Joshua P. Aronson, Melissa Putman and Mark R. Proctor

Patients with shunted hydrocephalus presenting with altered mental status and ventriculomegaly are generally considered to be in shunt failure requiring surgical treatment. The authors describe a case of shunted hydrocephalus secondary to a disseminated neuroectodermal tumor in a pediatric patient in whom rapid fluctuations in sodium levels due to diabetes insipidus repeatedly led to significant changes in ventricle size, with invasively confirmed normal shunt function and low intracranial pressure. This clinical picture exactly mimics shunt malfunction, requires urgent nonsurgical therapy, and underscores the importance of considering serum osmolar abnormalities in the differential diagnosis for ventriculomegaly.

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Georgios Zenonos, Osama Jamil, Lance S. Governale, Sarah Jernigan, Daniel Hedequist and Mark R. Proctor

Object

Spinal aneurysmal bone cysts (ABCs) constitute a rare and clinically challenging disease, primarily affecting the pediatric population. Information regarding the management of spinal ABCs remains sparse. In this study the authors review their experience with spinal ABCs at Children's Hospital Boston.

Methods

The medical records of all patients treated surgically for primary spinal ABCs between January 1998 and July 2010 were retrospectively reviewed.

Results

Fourteen cases were identified (6 males and 8 females, ages 5–19 years old). The ABCs were located throughout the spine, with an equal number in the thoracic and lumbar spine, and rarely in the cervical spine. The majority of patients presented with back pain, but neurological deficits and spinal deformity were common. A variety of radiographic techniques were used to establish the diagnosis, including needle biopsy. Preoperative selective arterial embolization was performed in 7 cases (50%), and the majority of cases required spinal instrumentation along with resection. Mean follow-up was 55.9 months (range 15–154 months) after initial intervention. Two ABCs recurred (14%), at 9 months and 8 years after incomplete initial resection, and the patients underwent reoperation. Complete resection was ultimately achieved in all cases. All patients were asymptomatic and neurologically intact at their last follow-up evaluation, and showed no evidence of deformity or recurrence on imaging.

Conclusions

Computed tomography and MR imaging are adequate for an initial evaluation of spinal ABCs, although solid variants can present a diagnostic challenge. Given the high rates of recurrence with residual disease, complete obliteration of the lesion should be the goal of treatment. Preoperative embolization is often performed, although in the authors' opinion the degree of bleeding tends not to support its routine use. Long-term follow-up is warranted as recurrences can occur years after initial intervention. However, gross-total excision in conjunction with spinal stabilization, as needed, usually provides cure of the ABC and excellent long-term spinal alignment.

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Michael Ragheb, Ashish H. Shah, Sarah Jernigan, Tulay Koru-Sengul and John Ragheb

OBJECTIVE

Hydrocephalus is recognized as a common disabling pediatric disease afflicting infants and children disproportionately in the developing world, where access to neurosurgical care is limited and risk of perinatal infection is high. This surgical case series describes the Project Medishare Hydrocephalus Specialty Surgery (PMHSS) program experience treating hydrocephalus in Haiti between 2008 and 2015.

METHODS

The authors conducted a retrospective review of all cases involving children treated for hydrocephalus within the PMHSS program in Port-au-Prince, Haiti, from 2008 through 2015. All relevant epidemiological information of children treated were prospectively collected including relevant demographics, birth history, hydrocephalus etiology, head circumference, and operative notes. All appropriate associations and statistical tests were performed using univariate and multivariate logistic regression analyses.

RESULTS

Among the 401 children treated within PMHSS, postinfectious hydrocephalus (PIH) accounted for 39.4% (n = 158) of cases based on clinical, radiographic, and endoscopic findings. The majority of children with hydrocephalus in Haiti were male (54.8%, n = 197), born in the rainy season (59.7%, n = 233), and born in a coastal/inland location (43.3%, n = 61). The most common surgical intervention was endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) (45.7%, n = 175). Multivariate logistic regression analysis yielded coastal birth location (OR 3.76, 95% CI 1.16–12.18) as a statistically significant predictor of PIH. Increasing head circumference (adjusted OR 1.06, 95% CI 0.99–1.13) demonstrated a slight trend toward significance with the incidence of PIH.

CONCLUSIONS

This information will provide the foundation for future clinical and public health studies to better understand hydrocephalus in Haiti. The 39.4% prevalence of PIH falls within observed rates in Africa as does the apparently higher prevalence for those born during the rainy season. Although PIH was the most frequent etiology seen in almost all birth locations, the potential relationship with geography noted in this series will be the focus of further research in an effort to understand the link between climate and PIH in Haiti. The ultimate goal will be to develop an appropriate public health strategy to reduce the burden of PIH on the children of Haiti.

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Manish Kuchakulla, Ashish H. Shah, Valerie Armstrong, Sarah Jernigan, Sanjiv Bhatia and Toba N. Niazi

OBJECTIVE

Carotid body tumors (CBTs), extraadrenal paragangliomas, are extremely rare neoplasms in children that often require multimodal surgical treatment, including preoperative anesthesia workup, embolization, and resection. With only a few cases reported in the pediatric literature, treatment paradigms and surgical morbidity are loosely defined, especially when carotid artery infiltration is noted. Here, the authors report two cases of pediatric CBT and provide the results of a systematic review of the literature.

METHODS

The study was divided into two sections. First, the authors conducted a retrospective review of our series of pediatric CBT patients and screened for patients with evidence of a CBT over the last 10 years (2007–2017) at a single tertiary referral pediatric hospital. Second, they conducted a systematic review, according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, of all reported cases of pediatric CBTs to determine the characteristics (tumor size, vascularity, symptomatology), treatment paradigms, and complications.

RESULTS

In the systematic review (n = 21 patients [includes 19 cases found in the literature and 2 from the authors’ series]), the mean age at diagnosis was 11.8 years. The most common presenting symptoms were palpable neck mass (62%), cranial nerve palsies (33%), cough or dysphagia (14%), and neck pain (19%). Metastasis occurred only in 5% of patients, and 19% of cases were recurrent lesions. Only 10% of patients presented with elevated catecholamines and associated sympathetic involvement. Preoperative embolization was utilized in 24% of patients (external carotid artery in 4 and external carotid artery and vertebral artery in 1). Cranial nerve palsies (cranial nerve VII [n = 1], IX [n = 1], X [n = 4], XI [n = 1], and XII [n = 3]) were the most common cause of surgical morbidity (33% of cases). The patients in the authors’ illustrative cases underwent preoperative embolization and balloon test occlusion followed by resection, and both patients suffered from transient Horner’s syndrome after embolization.

CONCLUSIONS

Surgical management of CBTs requires an extensive preoperative workup, anesthesia, and multimodal surgical management. Due to a potentially high rate of surgical morbidity and vascularity, balloon test occlusion with embolization may be necessary in select patients prior to resection. Careful thorough preoperative counseling is vital to preparing families for the intensive management of these children.

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Sarah C. Jernigan, Jay G. Berry, Dionne A. Graham and Liliana Goumnerova

Object

The purpose of this study was to compare the effectiveness of CSF diversion with endoscopic third ventriculostomy (ETV) versus shunt therapy in infants with hydrocephalus.

Methods

The authors conducted a retrospective analysis of 5416 infants 1 year of age or younger with hydrocephalus (congenital or acquired) in whom CSF diversion was performed using either ETV or shunt placement at 41 children's hospitals between 2004 and 2009. Data were obtained from the Pediatric Health Information Systems database. Surgical failure was defined as the need for a repeat diversion operation within 1 year of initial surgery. The authors compared failure rates of ETV and shunt, as well as patient demographics and clinical characteristics, using hierarchical regression according to treatment group.

Results

During the period examined, 872 infants (16.1%) initially underwent ETV and 4544 (83.9%) underwent ventricular shunt placement. The median infant age was 37 days (IQR 11–122 days) for both ETV and shunt placement. More infants who underwent ETV rather than shunt placement were born prematurely (41.6% vs 23.9%, respectively; p < 0.01) and had intraventricular hemorrhage (45.4% vs 17.5%, respectively; p < 0.01). Higher operative failure rates at 1 year were observed in infants who underwent ETV as opposed to shunt surgery (64.5% vs 39.6%, respectively; OR 2.9 [95% CI 2.3–3.5], p < 0.01). After controlling for prematurity, intraventricular hemorrhage, and spina bifida, ETV remained associated with a higher risk of failure (OR 2.6 [95% CI 2.1–3.2]).

Conclusions

In infants with hydrocephalus, a greater 1-year CSF diversion failure rate may occur after ETV compared with shunt placement. This risk is most significant for procedures performed within the first 90 days of life. Further investigation of the need for multiple reoperations, cost, and impact of surgeon and hospital experience is necessary to distinguish which treatment is more effective in the long term.

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Sarah Jernigan, Armide Storey, Christine Hammer, Coleman Riordan, Darren B. Orbach, R. Michael Scott and Edward Smith

OBJECTIVE

PHACE syndrome (PHACES) has been linked to cervical and cerebral vascular anomalies, including persistent embryonic anastomoses and progressive steno-occlusive disease. However, no prior studies have documented the long-term response of PHACES patients with moyamoya disease to surgical revascularization with pial or myosynangiosis. The authors present their experience with 8 consecutive patients with PHACES and moyamoya disease.

METHODS

Retrospective review of patients who underwent pial synangiosis revascularization for moyamoya disease with concurrent diagnosis of PHACES.

RESULTS

A total of 8 patients out of 456 surgically treated moyamoya patients had a diagnosis of PHACES. All patients were female, and their average age at the time of surgical treatment was 9.3 years (range 1.8–25.8 years). Five patients had associated basilar artery anomalies or stenosis. All patients had symptomatic narrowing of the petrous segment of the internal carotid artery with tortuous collateralization. Three patients underwent unilateral pial or myo-synangiosis and 5 underwent bilateral procedures. The average hospital length of stay was 5.0 days (range 3–7 days). There were no postoperative complications. Follow-up ranged from 8 to 160 months (average 56 months). Seven of 8 patients have had follow-up angiograms and all had Matsushima grade A or B collateralization without progression of stenosis in other locations. All patients had reduced cortical FLAIR signal on 6-month follow-up MRI and no evidence of new radiographic or clinical strokes.

CONCLUSIONS

Patients with moyamoya disease and PHACES had an intracranial arteriopathy characterized by ectactic anterior vasculature with concomitant basilar artery stenosis, and were all female. The patients had both radiographic and clinical responses to pial synangiosis. The surgical treatment of these patients can be challenging given facial hemangiomas located near the surgical field. Patients with unilateral disease did not have evidence of progression in other cerebral circulation during the given follow-up period.

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Zachary S. Hubbard, Ashish H. Shah, Michael Ragheb, Shelly Wang, Sarah Jernigan and John Ragheb

OBJECTIVE

Previous models have been utilized in other low- and middle-income countries (LMICs) to explore and assess the cost, sustainability, and effectiveness of infant hydrocephalus treatment. However, similar models have not been implemented in Haiti due to a paucity of data, epidemiology, and outcomes for hydrocephalus. Therefore, the authors utilized previously described economic modeling to estimate the annual cost and benefit of treating hydrocephalus in infants at a neurosurgery referral center, Hospital Bernard Mevs (HBM), in Port-au-Prince, Haiti.

METHODS

The authors conducted a retrospective review of data obtained in all children treated for hydrocephalus at the HBM from 2008 to 2015. The raw data were pooled with previously described surgical outcomes for hydrocephalus in other LMICs. Modeling was performed to determine outcomes, neurosurgical costs, disability-adjusted life years (DALYs), and economic benefits of Haitian hydrocephalus treatment during this time frame. Standard account methodology was employed to calculate cost per procedure. Using these formulas, the net economic benefit and cost/DALY were determined for hydrocephalus treatment at HBM from 2008 to 2015.

RESULTS

Of the 401 patients treated during the study period, 158 (39.4%) met criteria for postinfectious hydrocephalus, 54 (13.5%) had congenital hydrocephalus, 38 (9.5%) had myelomeningocele, 19 (4.7%) had aqueductal stenosis, and 132 (33%) were not placed into a category. Overall, 317 individuals underwent surgical treatment of their hydrocephalus, averting 3077 DALYs. The total cost of the procedures was $754,000, and the cost per DALY ranged between $86 and $245. The resulting net economic benefit for neurosurgical intervention ranged from $2.5 to $5.5 million.

CONCLUSIONS

This work demonstrates the substantial economic benefit of neurosurgical intervention for the treatment of pediatric hydrocephalus at a single hospital in Haiti. Based on DALYs averted, the need for additional centers offering basic neurosurgical services is apparent. A single center offering these services for several days each month was able to generate between $2.5 to $5.5 million in economic benefits, suggesting the need to develop neurosurgical capacity building in Haiti. Ultimately, prevention, screening, and early surgical treatment of these infants represent a public health and socioeconomic requisite for Haiti.

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Sarah C. Jernigan, Jay G. Berry, Dionne A. Graham, Stuart B. Bauer, Lawrence I. Karlin, Nedda M. Hobbs, R. Michael Scott and Benjamin C. Warf

Object

Although survival for patients with myelomeningocele has dramatically improved in recent decades, the occasional occurrence of sudden, unexplained death in young adult patients with myelomeningocele has been noted by the authors. This study was undertaken to determine risk factors for sudden death in this population.

Methods

The authors performed a retrospective chart review of patients born between 1978 and 1990 who received care at Children's Hospital Boston. The relationship between sudden death and patient demographics, presence of CSF shunt and history of shunt revisions, midbrain length as a marker for severity of hindbrain malformation, seizures, pulmonary and ventilatory dysfunction, body mass index, scoliosis, renal dysfunction, and cardiac disease was evaluated using the t-test, Fisher exact test, and logistic regression analysis.

Results

The age range for 106 patients in the study cohort was 19–30 years, with 58 (54.7%) women and 48 (45.3%) men. Six patients, all of whom were young women, experienced sudden death. In multivariate analysis, female sex, sleep apnea, and midbrain elongation ≥ 15 mm on MR imaging remained significantly associated with a higher risk of sudden death. These risk factors were cumulative, and female patients with sleep apnea and midbrain length ≥ 15 mm had the greatest risk (adjusted risk ratio 24.0, 95% CI 7.3–79.0; p < 0.05). No other comorbidities were found to significantly increase the risk of sudden death.

Conclusions

Young adult women with myelomeningocele are at significantly increased risk of sudden death in the setting of midbrain elongation and sleep apnea. Further investigation is needed to determine the benefit of routine screening to identify at-risk patients for closer cardiopulmonary monitoring and treatment.