John R. W. Kestle
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
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).
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.
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).
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.
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
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.
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.
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.
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.
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
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.
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.
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).
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.
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
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.
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.
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).
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.