Complications of neuroendoscopic septostomy.

OBJECTIVE: The advent of neuroendoscopy revolutionized the management of complex hydrocephalus. Fenestration of the septum pellucidum (septostomy) is often a therapeutic and/or necessary intervention in neuroendoscopy. However, these procedures are not without risk. The authors sought to record the incidence and types of complications. They attempted to discern if there was decreased likelihood of septostomy complications in patients who underwent endoscopic third ventriculostomy (ETV)/choroid plexus cauterization (CPC) as compared with those who underwent other procedures and those with larger ventricles preoperatively. The authors investigated different operative techniques and their possible relationships to septostomy complications. METHODS: The authors retrospectively reviewed all neuroendoscopic procedures with Current Procedural Terminology code 62161 performed from January 2003 until June 2019 at their institution. Septostomy, either alone or in conjunction with other procedures, was performed in 118 cases. Basic demographic characteristics, clinical histories, operative details/findings, and adverse events (intraoperative and postoperative) were collected. Pearson chi-square and univariate logistic regression analyses were performed. Patients with incomplete records were excluded. RESULTS: Of 118 procedures, 29 (24.5%) septostomies had either intraoperative or postoperative complications. The most common intraoperative complication was bleeding, as noted in 12 (10.2%) septostomies. Neuroendocrine dysfunction, including apnea, bradycardia, neurological deficit, seizure, etc., was the most common postoperative complication and seen after 15 (12.7%) procedures. No significant differences in complications were noted between ventricular size or morphology or between different operative techniques or ventricular approaches. There was no significant difference between the complication rate of patients who underwent ETV/CPC and that of patients who underwent septostomy as a part of other procedures. Greater length of surgery (OR 1.013) was associated with septostomy complications. CONCLUSIONS: Neuroendoscopy for hydrocephalus due to varying etiologies provides significant utility but is not without risk. The authors did not find associations between larger ventricular size or posterior endoscope approach and lower complication rates, as hypothesized. No significant difference in complication rates was noted between septostomy performed during ETV/CPC and other endoscopic procedures requiring septostomy.

Care for Chiari malformation type I: the role of socioeconomic disadvantage and race.

OBJECTIVE: There is little research on the effect of social determinants of health on Chiari malformation type I (CM-I). The authors analyzed data on all children evaluated for CM-I at a single institution to assess how socioeconomic factors and race affect the surgical treatment of this population. METHODS: Medical records of patients treated for CM-I at the authors' institution between 1992 and 2017 were reviewed. Area Deprivation Index (ADI) and Rural-Urban Commuting Area (RUCA) codes for each patient were used to measure neighborhood disadvantage. Non-Hispanic White patients were compared to non-White patients and Hispanic patients of any race (grouped together as non-White in this study) in terms of insurance status, ADI, and RUCA. Patients with initially benign CM-I, defined as not having undergone surgery within 9 months of their initial visit, were then stratified by having delayed symptom presentation or not, and compared on these same measures. RESULTS: The sample included 665 patients with CM-I: 82% non-Hispanic White and 18% non-White. The non-White patients were more likely to reside in disadvantaged (OR 3.4, p < 0.001) and urban (OR 4.66, p < 0.001) neighborhoods and to have public health insurance (OR 3.11, p < 0.001). More than one-quarter (29%) of patients underwent surgery. The non-White and non-Hispanic White patients had similar surgery rates (29.5% vs 28.9%, p = 0.895) at similar ages (8.8 vs 9.7 years, p = 0.406). There were no differences by race/ethnicity for symptoms at presentation. Surgical and nonsurgical patients had similar ADI scores (3.9 vs 4.2, p = 0.194), RUCA scores (2.1 vs 2.3, p = 0.252), and private health insurance rates (73.6% vs 74.2%, p = 0.878). A total of 153 patients underwent surgery within 9 months of their initial visit. The remaining 512 were deemed to have benign CM-I. Of these, 40 (7.8%) underwent decompression surgery for delayed symptom presentation. Patients with delayed symptom presentation were from less disadvantaged (ADI 3.2 vs 4.2; p = 0.025) and less rural (RUCA 1.8 vs 2.3; p = 0.023) areas than those who never underwent surgery. CONCLUSIONS: Although non-White patients were more likely to be socioeconomically disadvantaged, race and socioeconomic disadvantage were not associated with undergoing surgical treatment. However, among patients with benign CM-I, those undergoing decompression for delayed symptom presentation resided in more affluent and urban areas.

Imaging characteristics associated with surgery in Chiari malformation type I.

OBJECTIVE: In Chiari malformation type I (CM-I), a variety of imaging findings have been purported to be important; however, results have been inconclusive, inconsistent, or not replicated in independent studies. The purpose of this study was to report imaging characteristics for a large cohort of patients with CM-I and identify the imaging findings associated with surgical decompression. METHODS: Patients were identified using ICD-9 codes for CM-I for the period from 1996 to 2017. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, or 3) did not have available preoperative MRI. Retrospective chart review was performed to collect demographic and clinical data. Imaging parameters were measured according to the Chiari I Malformation Common Data Elements. RESULTS: A total of 731 patients were included for analysis, having a mean follow-up duration of 25.5 months. The mean age at presentation was 8.5 years. The mean tonsil position was 11.4 mm below the foramen magnum, and 62.8% of patients had a pegged tonsil shape. Two hundred patients (27.4%) underwent surgery for life-dominating tussive headache, lower cranial nerve dysfunction, syrinx, and/or brainstem dysfunction. Surgical treatment was associated with a syrinx (OR 20.4, 95% CI 12.3-33.3, p < 0.0001), CM-1.5 (OR 1.797, 95% CI 1.08-2.98, p = 0.023), lower tonsil position (OR 1.130, 95% CI 1.08-1.18, p < 0.0001), and congenital fusion of cervical vertebrae (OR 5.473, 95% CI 1.08-27.8, p = 0.040). Among patients with benign CM-I, tonsil position was statistically significantly associated with future surgery. CONCLUSIONS: Comprehensive imaging characteristics for a large cohort of patients with CM-I are reported. Analysis showed that a lower tonsillar position, a syrinx, and CM-1.5 were associated with undergoing posterior fossa decompression. This study demonstrates the importance of considering imaging findings in the context of patient symptomatology.

Prevalence of Sleep Disordered Breathing in Children With Myelomeningocele.

BACKGROUND: Retrospective studies have shown high rates of sleep disordered breathing in children with myelomeningocele. However, most patients included in those studies underwent polysomnography because of symptoms, so the prevalence of sleep disordered breathing in this population is unknown. OBJECTIVE: To determine the prevalence of sleep disordered breathing in children with myelomeningocele using screening polysomnography. METHODS: In this cross-sectional study, all children with myelomeningocele seen in a multi-disciplinary spina bifida clinic between 2016 and 2020 were referred for polysomnography regardless of clinical symptoms. Included children had not previously undergone polysomnography. The primary outcome for this study was presence of sleep disordered breathing, defined as Apnea-Hypopnea Index (AHI, number of apnea or hypopnea events per hour of sleep) greater than 2.5. Clinical and demographic variables relevant to myelomeningocele were also prospectively collected and tested for association with presence of sleep apnea. RESULTS: A total of 117 participants underwent polysomnography (age 1 mo to 21 yr, 49% male). The majority were white, non-Hispanic. Median AHI was 1.9 (interquartile range 0.6-4.2). A total of 49 children had AHI 2.5 or greater, yielding a sleep disordered breathing prevalence of 42% (95% CI 33%-51%). In multivariable logistic regression analysis, children with more rostral neurological lesion levels had higher odds of sleep disordered breathing (OR for thoracic, mid-lumbar, and low-lumbar: 7.34, 3.70, 4.04, respectively, compared to sacral level, P = .043). CONCLUSION: Over 40% of a sample of children with myelomeningocele, who underwent screening polysomnography, had significant sleep disordered breathing. Routine screening polysomnography may be indicated in this population.

Shunt failure clusters: an analysis of multiple, frequent shunt failures.

OBJECTIVE: Repeated failure of ventriculoperitoneal shunts (VPSs) is a problem familiar to pediatric neurosurgeons and patients. While there have been many studies to determine what factors are associated with the first shunt failure, studies of subsequent failures are much less common. The purpose of this study was to identify the prevalence and associated risk factors of clustered shunt failures (defined as 3 or more VPS operations within 3 months). METHODS: The authors reviewed prospectively collected records from all patients who underwent VPS surgery from 2008 to 2017 at their institution and included only those children who had received all of their hydrocephalus care at that institution. Demographics, etiology of hydrocephalus, history of endoscopic third ventriculostomy or temporizing procedure, initial valve type, age at shunt placement, and other factors were analyzed. Logistic regression was used to test for the association of each variable with a history of shunt failure cluster. RESULTS: Of the 465 included children, 28 (6.0%) had experienced at least one cluster of shunt failures. Among time-independent variables, etiology of hydrocephalus (OR 0.27 for non-intraventricular hemorrhage [IVH], nonmyelomeningocele, nonaqueductal stenosis etiology vs IVH, 95% CI 0.11-0.65; p = 0.003), younger gestational age at birth (OR 0.91, 95% CI 0.85-0.97; p = 0.003), history of a temporizing procedure (OR 2.77, 95% CI 1.12-6.85; p = 0.028), and smaller head circumference at time of initial shunt placement (OR 0.91, 95% CI 0.84-0.99; p = 0.044) showed significant association with shunt failure cluster on univariate analysis. None of these variables maintained significance in a multivariate model. Among children with a history of a shunt failure cluster, 21 (75%) had a shunt infection either prior to or during the shunt failure cluster. A comparison of the infecting organism between these children and 62 children with a history of infection but without a shunt failure cluster showed an association of cluster with gram-negative rod species. CONCLUSIONS: Six percent of children in this institutional sample had at least one shunt failure cluster. These children accounted for 30% of the total shunt revisions in the sample. Shunt infection is an important factor associated with shunt failure cluster. Children with a history of prematurity and IVH may have a higher risk for failure cluster.

Pediatric herniated lumbar disc: a population-based risk factor analysis.

OBJECTIVE: Surgical treatment of herniated lumbar disc (HLD) remains rare in children. The purpose of this study was to evaluate for potential disease risk factors leading to surgery based on a large single-center experience. METHODS: Data for all patients who had undergone surgical treatment for HLD between December 2008 and December 2016 at a single pediatric tertiary care referral center were collected and compared to data for a healthy control population obtained through a Youth Risk Behavior Surveillance System (YRBSS) survey in order to determine relevant disease risk factors. Univariate and multivariate logistic regression were used to determine the effect of potential risk factors. RESULTS: Twenty-seven patients in the disease cohort and 5212 healthy controls from the general population were included in the risk factor analysis. The mean body mass index was significantly higher in the disease population (30.2 vs 24.0 kg/m2, p < 0.0001). Children who had undergone microdiscectomy were more likely to be obese (OR 7.4, 95% CI 3.46-15.8, p < 0.001). No association was found between lumbar microdiscectomy and sports participation (OR 1.0, 95% CI -0.002 to 0.005, p = 0.37). CONCLUSIONS: Microdiscectomy remains a viable and safe option in the setting of failed conservative management for pediatric HLD. Childhood obesity is a risk factor for HLD and many other diseases, which increases its importance as a public health priority.

Patients with "benign" Chiari I malformations require surgical decompression at a low rate.

OBJECTIVE: There are sparse published data on the natural history of "benign" Chiari I malformation (CM-I)-i.e., Chiari with minimal or no symptoms at presentation and no imaging evidence of syrinx, hydrocephalus, or spinal cord signal abnormality. The purpose of this study was to review a large cohort of children with benign CM-I and to determine whether these children become symptomatic and require surgical treatment. METHODS: Patients were identified from institutional outpatient records using International Classification of Diseases, 9th Revision, diagnosis codes for CM-I from 1996 to 2016. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, 3) had previously undergone posterior fossa decompression, or 4) had imaging evidence of syringomyelia at their first appointment. To include only patients with benign Chiari (without syrinx or classic Chiari symptoms that could prompt immediate intervention), any patient who underwent decompression within 9 months of initial evaluation was excluded. After a detailed chart review, patients were excluded if they had classical Chiari malformation symptoms at presentation. The authors then determined what changes in the clinical picture prompted surgical treatment. Patients were excluded from the multivariate logistic regression analysis if they had missing data such as race and insurance; however, these patients were included in the overall survival analysis. RESULTS: A total of 427 patients were included for analysis with a median follow-up duration of 25.5 months (range 0.17-179.1 months) after initial evaluation. Fifteen patients had surgery at a median time of 21.0 months (range 11.3-139.3 months) after initial evaluation. The most common indications for surgery were tussive headache in 5 (33.3%), syringomyelia in 5 (33.3%), and nontussive headache in 5 (33.3%). Using the Kaplan-Meier method, rate of freedom from posterior fossa decompression was 95.8%, 94.1%, and 93.1% at 3, 5, and 10 years, respectively. CONCLUSIONS: Among a large cohort of patients with benign CM-I, progression of imaging abnormalities or symptoms that warrant surgical treatment is infrequent. Therefore, these patients should be managed conservatively. However, clinical follow-up of such individuals is justified, as there is a low, but nonzero, rate of new symptom or syringomyelia development. Future analyses will determine whether imaging or clinical features present at initial evaluation are associated with progression and future need for treatment.

Surveillance survey of family history in children with neural tube defects.

OBJECTIVE Although there are known risk factors for the development of neural tube defects (NTDs), little is known regarding the role of family history. The authors' goal in this study is to describe the family history in their population of patients with NTDs. METHODS Surveys were completed for 254 patients who were accompanied by their biological mother during their annual visit to the multidisciplinary Spina Bifida Clinic at Children's of Alabama. An NTD has been diagnosed in all patients who are seen in this clinic (myelomeningocele, lipomeningocele, split cord malformation, and congenital dermal sinus tract). Each mother answered questions regarding known NTD risk factors and their pregnancy, as well as the family history of NTDs, other CNS disorders, and birth defects. RESULTS The overall prevalence of family history of NTDs in children with an NTD was 16.9% (n = 43), of which 3.1% (n = 8) were in first-degree relatives. In patients with myelomeningocele, 17.7% (n = 37) had a positive family history for NTDs, with 3.8% in first-degree relatives. Family history in the paternal lineage for all NTDs was 8.7% versus 10.6% in the maternal lineage. Twenty-two patients (8.7%) had a family history of other congenital CNS disorders. Fifteen (5.9%) had a family history of Down syndrome, 12 (4.7%) had a family history of cerebral palsy, and 13 (5.1%) patients had a family history of clubfoot. Fourteen (5.5%) had a family history of cardiac defect, and 13 (5.1%) had a family history of cleft lip or palate. CONCLUSIONS The family history of NTDs was 16.9% in children with NTD without a difference between maternal and paternal lineage. This high rate of positive family history suggests that genetics and epigenetics may play a larger role in the pathogenesis of NTD in the modern era of widespread folate supplementation.

A comparison of the MOMS trial results to a contemporaneous, single-institution, postnatal closure cohort.

PURPOSE: We evaluate a single-institution cohort of mothers contemporaneous with the Management of Myelomeningocele Study (MOMS) trial to determine the generalizability of MOMS results and compare shunt rates. METHODS: A retrospective chart review identified patients with myelomeningocele born between 2003 and 2009. We applied MOMS eligibility criteria and compared sociodemographic variables between patients at our institution who would have been eligible or ineligible and MOMS participants. Finally, we applied the original MOMS primary outcome and the revised primary outcome to our cohort. RESULTS: Of the 78 patients, 55 (70.5%) were eligible for the MOMS trial. Mean maternal age, race, and marital status were different from both MOMS groups. Comparing our series to MOMS postnatal shows fewer female infants (44.9 vs. 63.8%, p = 0.017) and more thoracic lesions (12.8 vs. 3.8%, p = 0.038). Shunt rates in our cohort (84.6%) were higher than MOMS prenatal and similar to MOMS postnatal (44.0 and 83.7%, respectively). Fewer children met the original primary outcome than the postnatal group (84.6 vs. 97.8%, p = 0.002). There was no significant difference between our cohort and the prenatal group (84.6 vs. 72.5%, p = 0.058). When applying the revised criteria, we find the opposite: a significant difference between local and MOMS prenatal (84.6 vs. 49.5%, p < 0.001) but no difference between the local group and MOMS postnatal (84.6 vs. 87.0%, p = 0.662). CONCLUSIONS: Mothers in our cohort differ from mothers enrolled in MOMS via several sociodemographic factors. Baseline fetal characteristics show a significantly higher functional lesion level in between our cohort and MOMS. Treatment of hydrocephalus in our series tracks almost identically with original MOMS shunt criteria. Revision of the criteria led to greater concordance between meeting criteria and receiving a shunt in MOMS patients, but changes the results in our series.

Soluble membrane attack complex is diagnostic for intraventricular shunt infection in children.

BACKGROUND: Children treated with cerebrospinal fluid (CSF) shunts to manage hydrocephalus frequently develop shunt failure and/or infections, conditions that present with overlapping symptoms. The potential life-threatening nature of shunt infections requires rapid diagnosis; however, traditional microbiology is time consuming, expensive, and potentially unreliable. We set out to identify a biomarker that would identify shunt infection. METHODS: CSF was assayed for the soluble membrane attack complex (sMAC) by ELISA in patients with suspected shunt failure or infection. CSF was obtained at the time of initial surgical intervention. Statistical analysis was performed to assess the diagnostic potential of sMAC in pyogenic-infected versus noninfected patients. RESULTS: Children with pyogenic shunt infection had significantly increased sMAC levels compared with noninfected patients (3,211 ± 1,111 ng/ml vs. 26 ± 3.8 ng/ml, P = 0.0001). In infected patients undergoing serial CSF draws, sMAC levels were prognostic for both positive and negative clinical outcomes. Children with delayed, broth-only growth of commensal organisms (P. acnes, S. epidermidis, etc.) had the lowest sMAC levels (7.96 ± 1.7 ng/ml), suggesting contamination rather than shunt infection. CONCLUSION: Elevated CSF sMAC levels are both sensitive and specific for diagnosing pyogenic shunt infection and may serve as a useful prognostic biomarker during recovery from infection. FUNDING: This work was supported in part by the Impact Fund of Children's of Alabama.

Radiographic markers of clinical outcomes after endoscopic third ventriculostomy with choroid plexus cauterization: cerebrospinal fluid turbulence and choroid plexus visualization.

OBJECTIVE Endoscopic third ventriculostomy (ETV) with choroid plexus (CP) cauterization (CPC) represents a viable treatment option for congenital hydrocephalus in infants younger than 2 years. Imaging studies complement clinical data in the evaluation of treatment success or failure. The objectives of this study were to investigate novel radiographic markers-cerebrospinal fluid (CSF) turbulence and CP visualization-and their ability to reflect or predict clinical outcomes following ETV/CPC. METHODS Hydrocephalic patients younger than 2 years who were initially treated by ETV/CPC at the senior authors' institution between March 2013 and February 2014 were retrospectively reviewed. Clinical data, as well as the visualization of CSF turbulence and CP on pre- and postoperative fast-sequence MRI, were recorded. Radiographic images were reviewed by a blinded observer based on specific criteria for the visualization of CSF turbulence and CP. Data were collected and analyzed using descriptive statistics, including Fisher's exact test for comparisons. The research team obtained appropriate institutional review board approval for this study, without the need for informed consent. RESULTS Among the 32 patients (53% male and 47% female) studied, 18 of 32 (56%) responded favorably to initial or repeat ETV/CPC, with 13 of 32 (41%) patients requiring 1 surgery. Of the 19 (59%) patients whose initial ETV/CPC failed, 8 of 19 (42%) patients underwent repeat ETV/CPC, with 5 of 8 (63%) patients responding favorably. Radiographic CSF turbulence appeared more frequently following ETV/CPC failure than after ETV/CPC success (55% vs 18%, respectively; p = 0.02). The sensitivity and specificity of CSF turbulence as a radiographic marker for ETV/CPC failure were 80% and 58%, respectively. The radiographic depiction of CP disappearance following ETV/CPC from pre- to postoperative imaging occurred in 20 of 30 patients (67%). Among the patients who responded unsuccessfully to ETV/CPC and ultimately required secondary shunt insertion, 71% (10 of 14 patients) demonstrated CP persistence on postoperative imaging. In contrast, 6% (1 of 18) of patients who were treated successfully by ETV/CPC demonstrated the presence of CP on follow-up imaging. This difference reached statistical significance (p = 0.0001). The visualization of CP persistence despite ETV/CPC reflected treatment failure with 91% sensitivity and 81% specificity. The sensitivity of either or both radiographic markers to suggest ETV/CPC failure was 77%, while their specificity (both markers absent, thereby indicating ETV/CPC success) was 81%. CONCLUSIONS Radiographic markers correlate with clinical outcomes following the treatment of infantile hydrocephalus with ETV/CPC. Specifically, CSF turbulence may indicate ongoing pathological CSF flow dynamics, while CP absence following ETV/CPC may predict shunt independence. Future studies that incorporate prospective review and formal intra- and interobserver reliability estimates may help corroborate the utility of these radiographic markers.

Assessing health-related quality of life in children with spina bifida.

OBJECT: The purpose of this study is to explore various aspects of health-related quality of life (HRQOL) in children with spinal dysraphism. METHODS: The authors enrolled a prospective cohort of 159 patients from the multidisciplinary spina bifida clinic. Surveys were distributed to caregivers of patients with spina bifida who were 5 years old and older. Data were collected using the Health Utilities Index Mark 3 focusing on vision, speech, hearing, dexterity, ambulation, cognition, emotions, and pain. Each participant received an overall HRQOL utility score and individual domain subscores. These were correlated with demographic and treatment variables. Analysis was done using SPSS statistics (version 21). RESULTS: There were 125 patients with myelomeningocele, 25 with lipomyelomeningocele, and 9 with other dysraphisms. Among patients with myelomeningocele, 107 (86%) had CSF shunts in place, 14 (11%) had undergone Chiari malformation Type II decompression, 59 (47%) were community ambulators, and 45 (36%) were nonambulatory. Patients with myelomeningocele had significantly lower overall HRQOL scores than patients with closed spinal dysraphism. Among patients with myelomeningocele, younger patients had higher HRQOL scores. Patients with impaired bowel continence had lower overall HRQOL scores. History of a ventriculoperitoneal shunt was associated with worse HRQOL (overall score, ambulation, and cognition subscores). History of Chiari malformation Type II decompression was associated with worse overall, speech, and cognition scores. Patients who could ambulate in the community had higher overall and ambulation scores. A history of tethered cord release was correlated with lower pain subscore. No association was found between sex, race, insurance type, or bladder continence and HRQOL. CONCLUSIONS: Patients with myelomeningocele have significantly lower HRQOL scores than those with other spinal dysraphisms. History of shunt treatment and Chiari decompression correlate with lower HRQOL scores.