Mandeep S. Tamber and Robert P. Naftel
Choosing between competing options (shunt or endoscopic third ventriculostomy) for the management of hydrocephalus requires patients and caregivers to make a subjective judgment about the relative importance of risks and benefits associated with each treatment. In the context of this particular decision, little is known about what treatment-related factors are important and how they are prioritized in order to arrive at a treatment preference.
The Hydrocephalus Association electronically distributed a survey to surgically treated hydrocephalus patients or their families. Respondents rated the importance of various surgical attributes in their decision-making about treatment choice, and also indicated their preference in hypothetical scenarios involving a trade-off between potential risks and benefits of treatment. Rank-order correlations were used to determine whether certain predictor variables affected the rating of factors or hypothetical treatment choice.
Eighty percent of 414 respondents rated procedural risks, minimizing repeat surgery, and improving long-term brain function as being very or extremely important factors when deciding on a treatment; 69% rated the need to implant a permanent device similarly. Parent-respondents rated procedural risks higher than patient-respondents. A majority of respondents (n = 209, 54%) chose a procedure with higher surgical risk if it meant that implantation of a permanent device was not required, and respondents were more likely to choose this option if they discussed both treatment options with their surgeon prior to their initial intervention (Spearman rho 0.198, p = 0.001).
Although only 144 of 384 total respondents (38%) chose a less established operation if it meant less repeat surgery, patient-respondents were more likely to choose this option compared to parent-respondents (Spearman rho 0.145, p = 0.005). Likewise, patient-respondents were more likely than parent-respondents to choose an operation that involved less repeat surgery and led to worse long-term brain function (Spearman rho 0.160, p = 0.002), an option that was chosen by only 23 (6%) of respondents overall.
This study is the first exploration of patient/parental factors that influence treatment preference in pediatric hydrocephalus. Procedural risks, minimizing repeat operations, and the desire to maximize long-term cognitive function appeared to be the most important attributes that influenced treatment decisions that the survey respondents had made in the past. Patients and/or their caregivers appear to see some inherent benefit in being shunt free. It appears that fear of multiple revision operations may drive treatment choice in some circumstances.
Abhaya V. Kulkarni
Robert P. Naftel, R. Shane Tubbs, Gavin T. Reed and John C. Wellons III
The authors describe a new technique that may be used in conjunction with neuronavigation or freehand techniques for obtaining small ventricular access. Using this modification, the introducer sheath and trocar can be guided down a ventriculostomy tract with endoscopic visual control. With increasing focus on endoscopic therapies in patients without hydrocephalus, this adjunct, based on the authors' experience, may provide an additional technique for safely treating patients.
Robert P. Naftel, Gavin T. Reed, Abhaya V. Kulkarni and John C. Wellons III
Endoscopic third ventriculostomy (ETV) success is dependent on patient characteristics including age, origin of hydrocephalus, and history of shunt therapy. Using these factors, an Endoscopic Third Ventriculostomy Success Score (ETVSS) model was constructed to predict success of therapy. This study reports a single-institution experience with ETV and explores the ETVSS model validity.
A retrospective chart review identified 151 consecutive patients who underwent ETV at a pediatric hospital between August 1995 and December 2009. Of these 151, 136 patients had at least 6 months of clinical follow-up. Data concerning patient characteristics, operative characteristics, radiological findings, complications, and success of ETV were collected. The actual success rates were compared with those predicted by the ETVSS model.
The actual success rate of ETV at 6 months was 68.4% (93 of 136 patients), which compared well to the predicted ETVSS of 76.5% ± 12.5% (± SD). The C-statistic was 0.74 (95% CI 0.65–0.83), suggesting that the ability of the ETVSS to discriminate failures from successes was good. Secondary ETV was found to have a hazard ratio for failure of 4.2 (95% CI 2.4–7.2) compared with primary ETV (p < 0.001). The complication rate was 9.3% with no deaths. At the first radiological follow-up, the increased size of ventricles had a hazard ratio for failure of 3.0 (95% CI 1.5–6.0) compared with patients in whom ventricle size either remained stable or decreased (p = 0.002).
The ETVSS closely predicts the actual success of ETV, fitting the statistical model well. Shortcomings of the model were identified in overestimating success in patients with ETVSS ≤ 70, which may be attributable to the poor success of secondary ETVs in the authors' patient population.
Paul M. Foreman, Robert P. Naftel, Thomas A. Moore II and Mark N. Hadley
Since its introduction in 1976, the lateral extracavitary approach (LECA) has been used to access ventral and ventrolateral pathology affecting the thoracolumbar spine. Reporting of outcomes and complications has been inconsistent. A case series and systematic review are presented to summarize the available data.
A retrospective review of medical records was performed, which identified 65 consecutive patients who underwent LECA for the treatment of thoracolumbar spine and spinal cord pathology. Cases were divided according to the presenting pathology. Neurological outcomes and complications were detailed. In addition, a systematic review of outcomes and complications in patients treated with the LECA as reported in the literature was completed.
Sixty-five patients underwent the LECA to the spine for the treatment of thoracic spine and spinal cord pathology. The most common indication for surgery was thoracic disc herniation (23/65, 35.4%). Neurological outcomes were excellent: 69.2% improved, 29.2% experienced no change, and 1.5% were worse. Two patients (3.1%) experienced a complication. The systematic review revealed comparable neurological outcomes (74.9% improved) but a notably higher complication rate (32.2%).
The LECA provides dorsal and unilateral ventrolateral access to and exposure of the thoracolumbar spine and spinal cord while allowing for posterior instrumentation through the same incision. Although excellent neurological results can be expected, the risk of pulmonary complications should be considered.
Nishit Mummareddy, Michael C. Dewan, Michael R. Mercier, Robert P. Naftel, John C. Wellons III and Christopher M. Bonfield
The authors aimed to provide an updated and consolidated report on the epidemiology, management, and functional outcome of cases of myelomeningocele (MMC) in patients with scoliosis.
A comprehensive literature search was performed using MEDLINE, Embase, Google Scholar, and the Cochrane Database of Systematic Reviews on cases of MMC in patients with scoliosis between 1980 and 2016. The initial search yielded 670 reports. After removing duplicates and applying inclusion criteria, we included 32 full-text original articles in this study.
Pooled statistical analysis of the included articles revealed the prevalence of scoliosis in MMC patients to be 53% (95% CI 0.42–0.64). Slightly more females (56%) are affected with both MMC and scoliosis than males. Motor level appears to be a significant predictor of prevalence, but not severity, of scoliosis in MMC patients. Treatment options for these patients include tethered cord release (TCR) and fusion surgeries. Curvature improvement and stabilization after TCR may be limited to patients with milder (< 50°) curves. Meanwhile, more aggressive fusion procedures such as a combined anterior-posterior approach may result in more favorable long-term scoliosis correction, albeit with greater complication rates. Quality of life metrics including ambulatory status and sitting stability are influenced by motor level of the lesion as well as the degree of the scoliosis curvature.
Scoliosis is among the most common and challenging comorbidities from which patients with MMC suffer. Although important epidemiological and management trends are evident, larger, prospective studies are needed to discover ways to more accurately counsel and more optimally treat these patients.
Christopher M. Bonfield, Rachel Pellegrino, Jillian Berkman, Robert P. Naftel, Chevis N. Shannon and John C. Wellons III
Both the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Section on Pediatric Neurological Surgery (AANS/CNS Pediatric Section) and the International Society for Pediatric Neurosurgery (ISPN) annual meetings provide a platform for pediatric neurosurgeons to present, discuss, and disseminate current academic research. An ultimate goal of these meetings is to publish presented results in peer-reviewed journals. The purpose of the present study was to investigate the publication rates of oral presentations from the 2009, 2010, and 2011 AANS/CNS Pediatric Section and ISPN annual meetings in peer-reviewed journals.
All oral presentations from the 2009, 2010, and 2011 AANS/CNS Pediatric Section and ISPN annual meetings were reviewed. Abstracts were obtained from the AANS/CNS Pediatric Section and ISPN conference proceedings, which are available online. Author and title information were used to search PubMed to identify those abstracts that had progressed to publication in peer-reviewed journals. The title of the journal, year of the publication, and authors’ country of origin were also recorded.
Overall, 60.6% of the presented oral abstracts from the AANS/CNS Pediatric Section meetings progressed to publication in peer-reviewed journals, as compared with 40.6% of the ISPN presented abstracts (p = 0.0001). The journals in which the AANS/CNS Pediatric Section abstract-based publications most commonly appeared were Journal of Neurosurgery: Pediatrics (52%), Child’s Nervous System (11%), and Journal of Neurosurgery (8%). The ISPN abstracts most often appeared in the journals Child’s Nervous System (29%), Journal of Neurosurgery: Pediatrics (14%), and Neurosurgery (9%). Overall, more than 90% of the abstract-based articles were published within 4 years after presentation of the abstracts on which they were based.
Oral abstract presentations at two annual pediatric neurosurgery meetings have publication rates in peer-reviewed journal comparable to those for oral abstracts at other national and international neurosurgery meetings. The vast majority of abstract-based papers are published within 4 years of the meeting at which the abstract was presented; however, the AANS/CNS Pediatric Section abstracts are published at a significantly higher rate than ISPN abstracts, which could indicate the different meeting sizes, research goals, and resources of US authors compared with those of authors from other countries.
Robert P. Naftel, Nicole A. Safiano, Michael Falola, Jeffrey P. Blount, W. Jerry Oakes and John C. Wellons III
Children experiencing frequent shunt failure consume medical resources and represent a disproportionate level of morbidity in hydrocephalus care. While biological causes of frequent shunt failure may exist, this study analyzed demographic and socioeconomic patient characteristics associated with frequent shunt failure.
A survey of 294 caregivers of children with shunt-treated hydrocephalus provided demographic and socioeconomic characteristics. Children experiencing at least 10 shunt failures were considered frequent shunt-failure patients. Multivariate regression models were used to control for variables.
Frequent shunt failure was experienced by 9.5% of the patients (28 of 294). By univariate analysis, white race (p = 0.006), etiology of hydrocephalus (p = 0.022), years-with-shunt (p < 0.0001), and surgeon (p = 0.02) were associated with frequent shunt failure. Upon multivariate analysis, white race remained the key independent factor associated with frequent shunt failure (OR 5.8, 95% CI 1.2–27.8, p = 0.027). Race acted independently from socioeconomic factors, including income, level of education, and geographic location, and clinical factors, such as etiology of hydrocephalus, surgeon, and years-with-shunt. Additionally, after multivariate analysis surgeon and years-with-shunt remained associated with frequent shunt failure (p = 0.043 and p = 0.0098, respectively), although etiology of hydrocephalus was no longer associated (p = 0.1).
White race was the primary independent factor associated with frequent shunt failure. Because races use health care differently and the diagnosis of shunt failure is often subjective, a disparity in diagnosis and treatment has arisen. These findings call for objective criteria for the preoperative and intraoperative diagnosis of shunt failure.
Robert P. Naftel, R. Shane Tubbs, Joshua Y. Menendez, John C. Wellons III, Ian F. Pollack and W. Jerry Oakes
The effects of posterior fossa decompression on Chiari malformation Type I–induced syringomyelia have been well described. However, treatment of worsening syringomyelia after Chiari decompression remains enigmatic. This paper defines patient and clinical characteristics as well as treatment and postoperative radiological and clinical outcomes in patients experiencing this complication.
The authors performed a retrospective review of patients at the Children's Hospital of Pittsburgh and Children's of Alabama who developed worsening syringomyelia after Chiari decompression was performed.
Fourteen children (age range 8 months to 15 years), 7 of whom had preoperative syringomyelia, underwent posterior fossa decompression. Aseptic meningitis (n = 3) and bacterial meningitis (n = 2) complicated 5 cases (4 of these patients were originally treated at outside hospitals). Worsening syringomyelia presented a median of 1.4 years (range 0.2–10.3 years) after the primary decompression. Ten children presented with new, recurrent, or persistent symptoms, and 4 were asymptomatic. Secondary Chiari decompression was performed in 11 of the 14 children. The other 3 children were advised to undergo secondary decompression. A structural cause for each failed primary Chiari decompression (for example, extensive scarring, suture in the obex, arachnoid web, residual posterior arch of C-1, and no duraplasty) was identified at the secondary operation. After secondary decompression, 8 patients' symptoms completely resolved, 1 patient's condition stabilized, and 2 patients remained asymptomatic. Radiologically, 10 of the 11 children had a decrease in the size of their syringes, and 1 child experienced no change (but improved clinically). The median follow-up from initial Chiari decompression was 3.1 years (range 0.8–14.1 years) and from secondary decompression, 1.3 years (range 0.3–4.5 years). No patient underwent syringopleural shunting or other nonposterior fossa treatment for syringomyelia.
Based on the authors' experience, children with worsening syringomyelia after decompression for Chiari malformation Type I generally have a surgically remediable structural etiology, and secondary exploration and decompression should be considered.