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Jay Riva-Cambrin, John R. W. Kestle, Curtis J. Rozzelle, Robert P. Naftel, Jessica S. Alvey, Ron W. Reeder, Richard Holubkov, Samuel R. Browd, D. Douglas Cochrane, David D. Limbrick Jr., Chevis N. Shannon, Tamara D. Simon, Mandeep S. Tamber, John C. Wellons III, William E. Whitehead, Abhaya V. Kulkarni and for the Hydrocephalus Clinical Research Network

OBJECTIVE

Endoscopic third ventriculostomy combined with choroid plexus cauterization (ETV+CPC) has been adopted by many pediatric neurosurgeons as an alternative to placing shunts in infants with hydrocephalus. However, reported success rates have been highly variable, which may be secondary to patient selection, operative technique, and/or surgeon training. The objective of this prospective multicenter cohort study was to identify independent patient selection, operative technique, or surgical training predictors of ETV+CPC success in infants.

METHODS

This was a prospective cohort study nested within the Hydrocephalus Clinical Research Network’s (HCRN) Core Data Project (registry). All infants under the age of 2 years who underwent a first ETV+CPC between June 2006 and March 2015 from 8 HCRN centers were included. Each patient had a minimum of 6 months of follow-up unless censored by an ETV+CPC failure. Patient and operative risk factors of failure were examined, as well as formal ETV+CPC training, which was defined as traveling to and working with the experienced surgeons at CURE Children’s Hospital of Uganda. ETV+CPC failure was defined as the need for repeat ETV, shunting, or death.

RESULTS

The study contained 191 patients with a primary ETV+CPC conducted by 17 pediatric neurosurgeons within the HCRN. Infants under 6 months corrected age at the time of ETV+CPC represented 79% of the cohort. Myelomeningocele (26%), intraventricular hemorrhage associated with prematurity (24%), and aqueductal stenosis (17%) were the most common etiologies. A total of 115 (60%) of the ETV+CPCs were conducted by surgeons after formal training. Overall, ETV+CPC was successful in 48%, 46%, and 45% of infants at 6 months, 1 year, and 18 months, respectively. Young age (< 1 month) (adjusted hazard ratio [aHR] 1.9, 95% CI 1.0–3.6) and an etiology of post–intraventricular hemorrhage secondary to prematurity (aHR 2.0, 95% CI 1.1–3.6) were the only two independent predictors of ETV+CPC failure. Specific subgroups of ages within etiology categories were identified as having higher ETV+CPC success rates. Although training led to more frequent use of the flexible scope (p < 0.001) and higher rates of complete (> 90%) CPC (p < 0.001), training itself was not independently associated (aHR 1.1, 95% CI 0.7–1.8; p = 0.63) with ETV+CPC success.

CONCLUSIONS

This is the largest prospective multicenter North American study to date examining ETV+CPC. Formal ETV+CPC training was not found to be associated with improved procedure outcomes. Specific subgroups of ages within specific hydrocephalus etiologies were identified that may preferentially benefit from ETV+CPC.

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Abhaya V. Kulkarni, Jay Riva-Cambrin, Curtis J. Rozzelle, Robert P. Naftel, Jessica S. Alvey, Ron W. Reeder, Richard Holubkov, Samuel R. Browd, D. Douglas Cochrane, David D. Limbrick Jr., Tamara D. Simon, Mandeep Tamber, John C. Wellons III, William E. Whitehead and John R. W. Kestle

OBJECTIVE

High-quality data comparing endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) to shunt and ETV alone in North America are greatly lacking. To address this, the Hydrocephalus Clinical Research Network (HCRN) conducted a prospective study of ETV+CPC in infants. Here, these prospective data are presented and compared to prospectively collected data from a historical cohort of infants treated with shunt or ETV alone.

METHODS

From June 2014 to September 2015, infants (corrected age ≤ 24 months) requiring treatment for hydrocephalus with anatomy suitable for ETV+CPC were entered into a prospective study at 9 HCRN centers. The rate of procedural failure (i.e., the need for repeat hydrocephalus surgery, hydrocephalus-related death, or major postoperative neurological deficit) was determined. These data were compared with a cohort of similar infants who were treated with either a shunt (n = 969) or ETV alone (n = 74) by creating matched pairs on the basis of age and etiology. These data were obtained from the existing prospective HCRN Core Data Project. All patients were observed for at least 6 months.

RESULTS

A total of 118 infants underwent ETV+CPC (median corrected age 1.3 months; common etiologies including myelomeningocele [30.5%], intraventricular hemorrhage of prematurity [22.9%], and aqueductal stenosis [21.2%]). The 6-month success rate was 36%. The most common complications included seizures (5.1%) and CSF leak (3.4%). Important predictors of treatment success in the survival regression model included older age (p = 0.002), smaller preoperative ventricle size (p = 0.009), and greater degree of CPC (p = 0.02). The matching algorithm resulted in 112 matched pairs for ETV+CPC versus shunt alone and 34 matched pairs for ETV+CPC versus ETV alone. ETV+CPC was found to have significantly higher failure rate than shunt placement (p < 0.001). Although ETV+CPC had a similar failure rate compared with ETV alone (p = 0.73), the matched pairs included mostly infants with aqueductal stenosis and miscellaneous other etiologies but very few patients with intraventricular hemorrhage of prematurity.

CONCLUSIONS

Within a large and broad cohort of North American infants, our data show that overall ETV+CPC appears to have a higher failure rate than shunt alone. Although the ETV+CPC results were similar to ETV alone, this comparison was limited by the small sample size and skewed etiological distribution. Within the ETV+CPC group, greater extent of CPC was associated with treatment success, thereby suggesting that there are subgroups who might benefit from the addition of CPC. Further work will focus on identifying these subgroups.

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William E. Whitehead, Jay Riva-Cambrin, Abhaya V. Kulkarni, John C. Wellons III, Curtis J. Rozzelle, Mandeep S. Tamber, David D. Limbrick Jr., Samuel R. Browd, Robert P. Naftel, Chevis N. Shannon, Tamara D. Simon, Richard Holubkov, Anna Illner, D. Douglas Cochrane, James M. Drake, Thomas G. Luerssen, W. Jerry Oakes and John R. W. Kestle

OBJECTIVE

Accurate placement of ventricular catheters may result in prolonged shunt survival, but the best target for the hole-bearing segment of the catheter has not been rigorously defined. The goal of the study was to define a target within the ventricle with the lowest risk of shunt failure.

METHODS

Five catheter placement variables (ventricular catheter tip location, ventricular catheter tip environment, relationship to choroid plexus, catheter tip holes within ventricle, and crosses midline) were defined, assessed for interobserver agreement, and evaluated for their effect on shunt survival in univariate and multivariate analyses. De-identified subjects from the Shunt Design Trial, the Endoscopic Shunt Insertion Trial, and a Hydrocephalus Clinical Research Network study on ultrasound-guided catheter placement were combined (n = 858 subjects, all first-time shunt insertions, all patients < 18 years old). The first postoperative brain imaging study was used to determine ventricular catheter placement for each of the catheter placement variables.

RESULTS

Ventricular catheter tip location, environment, catheter tip holes within the ventricle, and crosses midline all achieved sufficient interobserver agreement (κ > 0.60). In the univariate survival analysis, however, only ventricular catheter tip location was useful in distinguishing a target within the ventricle with a survival advantage (frontal horn; log-rank, p = 0.0015). None of the other catheter placement variables yielded a significant survival advantage unless they were compared with catheter tips completely not in the ventricle. Cox regression analysis was performed, examining ventricular catheter tip location with age, etiology, surgeon, decade of surgery, and catheter entry site (anterior vs posterior). Only age (p < 0.001) and entry site (p = 0.005) were associated with shunt survival; ventricular catheter tip location was not (p = 0.37). Anterior entry site lowered the risk of shunt failure compared with posterior entry site by approximately one-third (HR 0.65, 95% CI 0.51–0.83).

CONCLUSIONS

This analysis failed to identify an ideal target within the ventricle for the ventricular catheter tip. Unexpectedly, the choice of an anterior versus posterior catheter entry site was more important in determining shunt survival than the location of the ventricular catheter tip within the ventricle. Entry site may represent a modifiable risk factor for shunt failure, but, due to inherent limitations in study design and previous clinical research on entry site, a randomized controlled trial is necessary before treatment recommendations can be made.

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Abhaya V. Kulkarni, Jay Riva-Cambrin, Richard Holubkov, Samuel R. Browd, D. Douglas Cochrane, James M. Drake, David D. Limbrick, Curtis J. Rozzelle, Tamara D. Simon, Mandeep S. Tamber, John C. Wellons III, William E. Whitehead, John R. W. Kestle and for the Hydrocephalus Clinical Research Network

OBJECTIVE

Endoscopic third ventriculostomy (ETV) is now established as a viable treatment option for a subgroup of children with hydrocephalus. Here, the authors report prospective, multicenter results from the Hydrocephalus Clinical Research Network (HCRN) to provide the most accurate determination of morbidity, complication incidence, and efficacy of ETV in children and to determine if intraoperative predictors of ETV success add substantially to preoperative predictors.

METHODS

All children undergoing a first ETV (without choroid plexus cauterization) at 1 of 7 HCRN centers up to June 2013 were included in the study and followed up for a minimum of 18 months. Data, including detailed intraoperative data, were prospectively collected as part of the HCRN's Core Data Project and included details of patient characteristics, ETV failure (need for repeat hydrocephalus surgery), and, in a subset of patients, postoperative complications up to the time of discharge.

RESULTS

Three hundred thirty-six eligible children underwent initial ETV, 18.8% of whom had undergone shunt placement prior to the ETV. The median age at ETV was 6.9 years (IQR 1.7–12.6), with 15.2% of the study cohort younger than 12 months of age. The most common etiologies were aqueductal stenosis (24.8%) and midbrain or tectal lesions (21.2%). Visible forniceal injury (16.6%) was more common than previously reported, whereas severe bleeding (1.8%), thalamic contusion (1.8%), venous injury (1.5%), hypothalamic contusion (1.5%), and major arterial injury (0.3%) were rare. The most common postoperative complications were CSF leak (4.4%), hyponatremia (3.9%), and pseudomeningocele (3.9%). New neurological deficit occurred in 1.5% cases, with 0.5% being permanent.

One hundred forty-one patients had documented failure of their ETV requiring repeat hydrocephalus surgery during follow-up, 117 of them during the first 6 months postprocedure. Kaplan-Meier rates of 30-day, 90-day, 6-month, 1-year, and 2-year failure-free survival were 73.7%, 66.7%, 64.8%, 61.7%, and 57.8%, respectively. According to multivariate modeling, the preoperative ETV Success Score (ETVSS) was associated with ETV success (p < 0.001), as was the intraoperative ability to visualize a “naked” basilar artery (p = 0.023).

CONCLUSIONS

The authors' documented experience represents the most detailed account of ETV results in North America and provides the most accurate picture to date of ETV success and complications, based on contemporaneously collected prospective data. Serious complications with ETV are low. In addition to the ETVSS, visualization of a naked basilar artery is predictive of ETV success.

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Abhaya V. Kulkarni, Jay Riva-Cambrin, Samuel R. Browd, James M. Drake, Richard Holubkov, John R. W. Kestle, David D. Limbrick, Curtis J. Rozzelle, Tamara D. Simon, Mandeep S. Tamber, John C. Wellons III and William E. Whitehead

Object

The use of endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has been advocated as an alternative to CSF shunting in infants with hydrocephalus. There are limited reports of this procedure in the North American population, however. The authors provide a retrospective review of the experience with combined ETV + CPC within the North American Hydrocephalus Clinical Research Network (HCRN).

Methods

All children (< 2 years old) who underwent an ETV + CPC at one of 7 HCRN centers before November 2012 were included. Data were collected retrospectively through review of hospital records and the HCRN registry. Comparisons were made to a contemporaneous cohort of 758 children who received their first shunt at < 2 years of age within the HCRN.

Results

Thirty-six patients with ETV + CPC were included (13 with previous shunt). The etiologies of hydrocephalus were as follows: intraventricular hemorrhage of prematurity (9 patients), aqueductal stenosis (8), myelomeningocele (4), and other (15). There were no major intraoperative or early postoperative complications. There were 2 postoperative CSF infections. There were 2 deaths unrelated to hydrocephalus and 1 death from seizure. In 18 patients ETV + CPC failed at a median time of 30 days after surgery (range 4–484 days). The actuarial 3-, 6-, and 12-month success for ETV + CPC was 58%, 52%, and 52%. Time to treatment failure was slightly worse for the 36 patients with ETV + CPC compared with the 758 infants treated with shunts (p = 0.012). Near-complete CPC (≥ 90%) was achieved in 11 cases (31%) overall, but in 50% (10 of 20 cases) in 2012 versus 6% (1 of 16 cases) before 2012 (p = 0.009). Failure was higher in children with < 90% CPC (HR 4.39, 95% CI 0.999–19.2, p = 0.0501).

Conclusions

The early North American multicenter experience with ETV + CPC in infants demonstrates that the procedure has reasonable safety in selected cases. The degree of CPC achieved might be associated with a surgeon's learning curve and appears to affect success, suggesting that surgeon training might improve results.

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Joshua J. Chern, Baran Aksut, Jennifer L. Kirkman, Mohammadali M. Shoja, R. Shane Tubbs, Stuart A. Royal, John C. Wellons III and Curtis J. Rozzelle

Object

The purpose of this study was to correlate lumbar ultrasound (LUS) and MRI findings in patients suspected of having occult spinal dysraphism (OSD).

Methods

Over a 5-year period, 1273 consecutive infants underwent an LUS study at a major pediatric tertiary referral center. Of these, 106 patients had abnormal LUS findings suggestive of an OSD, and 103 underwent subsequent MRI studies. The anatomical descriptions of the 2 studies were compared for agreement.

Results

The average age of the infants was 34 days at the time of the LUS study; OSD was suspected in these patients because of the presence of cutaneous stigmata and congenital defects. The most common anatomical descriptions from the LUS study included a thickened or fatty filum (32 cases), filum cyst (11 cases), and presence of a terminal ventricle or syrinx (9 cases). Using MRI findings as the standard reference, the sensitivity of LUS in detecting a thickened or fatty filum was 20%. The sensitivity of detecting an abnormal conus level at or below L-3 was 76.9%.

Conclusions

In the patient population chosen to undergo LUS studies, abnormal findings had poor sensitivity at detecting anatomical findings consistent with OSD.

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Joshua J. Chern, Mitchel Muhleman, R. Shane Tubbs, Joseph H. Miller, James M. Johnston, John C. Wellons III, Jeffrey P. Blount, W. Jerry Oakes and Curtis J. Rozzelle

Object

Most children with spina bifida aperta have implanted CSF shunts. However, the efficacy of adding surveillance imaging to clinical evaluation during routine follow-up as a means to minimize the hazard of shunt failure has not been thoroughly studied.

Methods

A total of 396 clinic visits were made by patients with spina bifida aperta and shunt-treated hydrocephalus in a spina bifida specialty clinic during the calendar years 2008 and 2009 (initial clinic visit). All visits were preceded by a 6-month period during which no shunt evaluation of any kind was performed and were followed by a subsequent visit in the same clinic. At the initial clinic visit, 230 patients were evaluated by a neurosurgeon (clinical evaluation group), and 166 patients underwent previously scheduled surveillance CT scans in addition to clinical evaluation (surveillance imaging group). Subsequent unexpected events, defined as emergency department (ED) visits and caregiver-requested clinic visits, were reviewed. The time to an unexpected event and the likelihood of event occurrence in each of the 2 groups were compared using Cox proportional hazards survival analysis. The outcome and complications of shunt surgeries were also reviewed.

Results

The clinical characteristics of the 2 groups were similar. In the clinical evaluation group, 2 patients underwent shunt revision based on clinical findings in the initial visit. In the subsequent follow-up period, there were 27 visits to the ED and 25 requested clinic visits that resulted in 12 shunt revisions. In the surveillance imaging group, 11 patients underwent shunt revision based on clinical and imaging findings in the initial visit. In the subsequent follow-up period, there were 15 visits to the ED and 9 requested clinic visits that resulted in 8 shunt revisions. Patients who underwent surveillance imaging on the day of initial clinic visit were less likely to have an unexpected event in the subsequent follow-up period (relative risk 0.579, p = 0.026). The likelihood of needing shunt revision and the morbidity of shunt malfunction was not significantly different between the 2 groups.

Conclusions

Surveillance imaging in children with spina bifida aperta and shunted hydrocephalus decreases the likelihood of ED visits and caregiver-requested clinic visits in the follow-up period, but based on this study, its effect on mortality and morbidity related to shunt malfunction was less clear.

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Joshua J. Chern, Jennifer L. Kirkman, Chevis N. Shannon, R. Shane Tubbs, Jeffrey D. Stone, Stuart A. Royal, W. Jerry Oakes, Curtis J. Rozzelle and John C. Wellons

Object

Various cutaneous stigmata and congenital anomalies are accepted as sufficient reasons to perform lumbar ultrasonography as a screening tool to rule out occult spinal dysraphism (OSD). The purpose of this study was to correlate presenting cutaneous findings with lumbar ultrasonography results based on a large number of lumbar ultrasonography tests obtained by regional primary care providers.

Methods

Over the course of 5 years, 1273 infants underwent lumbar ultrasonography screening at a major pediatric tertiary referral center. Of these infants, 1116 had adequate documentation for retrospective chart review. Referral sources included urban academic, urban private practice, and surrounding rural private practitioners. Presence of cutaneous stigmata and/or congenital anomalies and lumbar ultrasonography results were reviewed for all patients. When present, surgical findings were reviewed.

Results

A total of 943 infants were referred for presumed cutaneous stigmata, the most common of which was a sacral dimple (638 patients [68%]) followed by hairy patch (96 patients [10%]). Other reported cutaneous findings included hemangioma, deviated gluteal fold, skin tag, and skin discoloration. In comparison, 173 patients presented with congenital anomalies, such as imperforate anus (56 patients [32%]) and tracheoesophageal fistula/esophageal atresia (37 patients [21%]), most of which were detected prenatally by fetal ultrasonography. A total of 17 infants underwent surgical exploration. Occult spinal dysraphism was diagnosed in 7 infants in the cutaneous stigmata group and in 10 infants in the group with congenital abnormalities. None of the cutaneous stigmata as recorded were found to be indicative of the presence of OSD.

Conclusions

Cutaneous markers as currently defined by general practitioners are not useful markers for predicting OSD. The vast majority of findings on lumbar ultrasonography studies performed under these circumstances will be negative.

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Joshua J. Chern, Joseph H. Miller, R. Shane Tubbs, Thomas R. Whisenhunt, James M. Johnston, John C. Wellons III, Curtis J. Rozzelle, Jeffrey P. Blount and W. Jerry Oakes

Object

A large volume of patients presented to a Level I pediatric trauma center during and after a recent tornado disaster. Injuries of the central and peripheral nervous systems and the medical responses of a pediatric neurosurgical team are reviewed.

Methods

The clinical courses of patients who suffered cranial, spinal, and peripheral nerve injuries due to the tornado storm are reported. The clinical actions taken by the neurosurgical team during and after the event are reviewed and the lessons learned are discussed.

Results

The tornado storm system moved through the Tuscaloosa and Birmingham metropolitan areas on the early evening hours of April 27, 2011. Twenty-four patients received care from the neurosurgical team. A total of 11 cranial (including placement of an external ventricular drain), 2 spine, and 2 peripheral procedures were performed for the victims. Nine procedures were performed within the first 12 hours of the event, and an additional 6 surgeries were performed in the following 24 hours. Injuries of the peripheral nervous system often presented in a delayed fashion. Several key components were identified that enabled adequate neurosurgical care for a large influx of acute patients.

Conclusions

Massive casualties due to tornados are rare. A well-organized physician team working with the hospital administration may decrease the mortality and morbidity of such events.

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R. Shane Tubbs, Joshua Beckman, Robert P. Naftel, Joshua J. Chern, John C. Wellons III, Curtis J. Rozzelle, Jeffrey P. Blount and W. Jerry Oakes

Object

The diagnosis and treatment of Chiari malformation Type I (CM-I) has evolved over the last few decades. The authors present their surgical experience of over 2 decades of treating children with this form of hindbrain herniation.

Methods

The authors conducted a retrospective review of their institutional experience with the surgical treatment of the pediatric CM-I from 1989 to 2010.

Results

The 2 most common presentations were headache/neck pain (40%) and scoliosis (18%). Common associated diagnoses included neurofibromatosis Type 1 (5%) and idiopathic growth hormone deficiency (4.2%). Spine anomalies included scoliosis (18%), retroversion of the odontoid process (24%), Klippel-Feil anomaly (3%), and atlantooccipital fusion (8%). Approximately 3% of patients had a known family member with CM-I. Hydrocephalus was present in 48 patients (9.6%). Syringomyelia was present in 285 patients (57%), and at operation, 12% of patients with syringomyelia were found to have an arachnoid veil occluding the fourth ventricular outlet. Fifteen patients (3%) have undergone reoperation for continued symptoms or persistent large syringomyelia. The most likely symptoms and signs to resolve following surgery were Valsalva-induced headache and syringomyelia. The average hospital stay and “return to school” time were 3 and 12 days, respectively. The follow-up for this group ranged from 2 months to 15 years (mean 5 years). Complications occurred in 2.4% of cases; there was no mortality. No patient required acute return to the operating room, and no blood transfusions were performed.

Conclusions

The authors believe this to be the largest reported series of surgically treated pediatric CM-I patients and hope that their experience will be of use to others who treat this surgical entity.