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


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.


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.


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.


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, Jay G. Berry, Dionne A. Graham and Liliana Goumnerova


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.


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.


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]).


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


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.


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.


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.


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.