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Craniosynostosis may lead to elevated intracranial pressure, which may be implicated with impaired neurocognitive development. However, accurately measuring intracranial pressure is challenging, and patterns in craniosynostosis patients are poorly characterized. Spectral-domain optical coherence tomography may enable noninvasive assessment of intracranial pressure in pediatric patients with craniosynostosis.Pediatric patients with craniosynostosis undergoing surgical intervention between 2014 and 2019 prospectively underwent optical coherence tomographic evaluation. Intracranial pressure was directly measured intraoperatively in a subset of cases. Optical coherence tomographic parameters were compared to directly measured intracranial pressure and used for pattern assessment.Optical coherence tomography was performed in 158 subjects, among which 42 underwent direct intracranial pressure measurement during an initial cranial procedure. Maximal retinal nerve fiber layer thickness, maximal retinal thickness, and maximal anterior projection optical coherence tomographic parameters were positively correlated with intracranial pressure (p ≤ 0.001), with all parameters showing significantly higher values in patients with intracranial pressure thresholds of 15 mmHg (p0.001) and 20 mmHg (p ≤ 0.007). Patients with maximal retinal nerve fiber layer thickness and maximal anterior projection exceeding set thresholds in optical coherence tomography of either eye demonstrated 77.3 percent sensitivity and 95.0 percent specificity for detecting intracranial pressure above 15 mmHg, and 90.0 percent sensitivity and 81.3 percent specificity for detecting intracranial pressure above 20 mmHg. Patients with associated syndromes or multiple suture involvement and patients aged 9 months or older were significantly more likely to have elevated intracranial pressure above 15 mmHg (p ≤ 0.030) and above 20 mmHg (p ≤ 0.035).Spectral-domain optical coherence tomography can noninvasively detect elevated intracranial pressure in patients with craniosynostosis with reliable sensitivity and specificity. This technology may help guide decisions about the appropriate type and timing of surgical treatment.Diagnostic, I.

OBJECTIVE Children with multiple prematurely fused cranial sutures and those undergoing surgical correction later in life appear to experience worse neurocognitive outcomes, but it is unclear whether higher intracranial pressure (ICP) is implicated in this process. The purpose of this study was to elucidate the effect of age at intervention and number of involved cranial sutures on ICP, as well as to assess which cranial suture closure may be more associated with elevated ICP. METHODS The prospective craniofacial database at the authors’ institution was queried for patients undergoing initial corrective surgery for craniosynostosis in whom intraoperative measurement of ICP was obtained prior to craniectomy. Age, involved sutures, and syndromic status were analyzed in the context of measured ICP by using multiple linear regression. RESULTS Fifty patients met the inclusion criteria. Age at procedure (p = 0.028, β = +0.060 mm Hg/month) and multiple-suture involvement (p = 0.010, β = +4.175 mm Hg if multisuture) were both significantly implicated in elevated ICP. The actual number of major sutures involved was significantly correlated to ICP (p = 0.001; β = +1.687 mm Hg/suture). Among patients with single-suture involvement, there was an overall significant difference of median ICP across the suture types (p = 0.008), with metopic having the lowest (12.5 mm Hg) and sagittal having the highest (16.0 mm Hg). Patients with multiple-suture involvement had significantly higher ICP (p = 0.003; 18.5 mm Hg). Patients with craniofacial syndromes were 79.3 times more likely to have multiple-suture involvement (p < 0.001). Corrective surgery for craniosynostosis demonstrated significant intraoperative reduction of elevated ICP (all p < 0.050). CONCLUSIONS Syndromic status, older age at intervention for craniosynostosis, and multiple premature fusion of cranial sutures were associated with significantly higher ICP.

OBJECTIVE Endoscopic strip craniectomy (ESC) and spring-mediated cranioplasty (SMC) are two minimally invasive techniques for treating sagittal craniosynostosis in early infancy. Data comparing the perioperative outcomes of these two techniques are sparse. Here, the authors hypothesized that outcomes would be similar between patients undergoing SMC and those undergoing ESC and conducted a study using the multicenter Pediatric Craniofacial Surgery Perioperative Registry (PCSPR). METHODS The PCSPR was queried for infants under the age of 6 months who had undergone SMC or ESC for sagittal synostosis. SMC patients were propensity score matched 1:2 with ESC patients on age and weight. Primary outcomes were transfusion-free hospital course, intensive care unit (ICU) admission, ICU length of stay (LOS), and hospital length of stay (HLOS). The authors also obtained data points regarding spring removal. Comparisons of outcomes between matched groups were performed with multivariable regression models. RESULTS The query returned data from 676 infants who had undergone procedures from June 2012 through September 2019, comprising 580 ESC infants from 32 centers and 96 SMC infants from 5 centers. Ninety-six SMC patients were matched to 192 ESC patients. There was no difference in transfusion-free hospital course between the two groups (adjusted odds ratio [aOR] 0.78, 95% CI 0.45–1.35). SMC patients were more likely to be admitted to the ICU (aOR 7.50, 95% CI 3.75–14.99) and had longer ICU LOSs (incident rate ratio [IRR] 1.42, 95% CI 1.37–1.48) and HLOSs (IRR 1.28, 95% CI 1.17–1.39). CONCLUSIONS In this multicenter study of ESC and SMC, the authors found similar transfusion-free hospital courses; however, SMC infants had longer ICU LOSs and HLOSs. A trial comparing longer-term outcomes in SMC versus ESC would further define the roles of these two approaches in the management of sagittal craniosynostosis.

The purpose of this study was to compare perioperative safety and efficacy of posterior vault distraction osteogenesis (PVDO) in patients with primary nonsynostotic cephalo-cranial disproportion, namely slit ventricle syndrome and idiopathic intracranial hypertension (IIH), to a cohort of patients with craniosynostosis (CS). A retrospective review of patients undergoing PVDO from 2009 to 2019 at our institution was performed. Craniosynostosis patients were matched by sex and age at PVDO to the nonsynostotic cohort. Operative details, perioperative outcomes, and distraction patterns were analyzed with appropriate statistics. Nine patients met inclusion criteria for the non-CS cohort. Six patients (67%) underwent PVDO for slit ventricle and the remaining 3 patients (33%) underwent PVDO for IIH. The majority of CS patients were syndromic (n = 6, 67%) and had multisuture synostosis (n = 7, 78%). The non-CS cohort underwent PVDO at a median 56.1 months old [Q1 41.1, Q3 86.6] versus the CS cohort at 55.7 months [Q1 39.6, Q3 76.0] (P = 0.931). Total hospital length of stay was longer in the non-CS patients (median days 5 [Q1 4, Q3 6] versus 3 [Q1 3, Q3 4], P = 0.021). Non-CS patients with ventriculoperitoneal shunts had significantly less shunt operations for ICP concerns post-PVDO (median rate: 1.74/year [Q1 1.30, Q3 3.00] versus median: 0.18/year [Q1 0.0, Q3 0.7]; P = 0.046). In this pilot study using PVDO to treat slit ventricle syndrome and IIH, safety appears similar to PVDO in the synostotic setting. The cohort lacks adequate follow-up to assess long term efficacy, although short-midterm follow-up demonstrates promising results with less need for shunt revision and symptomatic relief. Future studies are warranted to identify the preferred surgical approach in these complex patients.

A larger volume cranial vault expansion is likely facilitated by a low posterior cranial osteotomy beneath the torcula; however, this may impart an increased risk of venous bleeding. The authors compared the safety of infratorcular versus supratorcular osteotomy in patients undergoing posterior vault reconstruction or posterior vault distraction osteogenesis and analyzed volumetric changes.Patients undergoing initial posterior vault reconstruction or distraction osteogenesis between 2009 and 2021 at the authors' institution were grouped by occipital osteotomy location and analyzed retrospectively. Craniometric analysis was performed if patients had high-resolution computed tomography scans available within 180 days preoperatively and postoperatively.A total of 187 patients were included: 106 (57 percent) who underwent posterior vault distraction osteogenesis and 81 (43 percent) who underwent posterior vault reconstruction. Infratorcular osteotomy was more common in reconstruction [ n = 65 (80 percent)] than in distraction osteogenesis [ n = 61 (58 percent); p0.002]. Blood transfused was similar between low and high osteotomy cohorts in the distraction osteogenesis ( p = 0.285) and reconstruction ( p = 0.342) groups. However, median transfused blood volume per kilogram of patient weight was greater in the low versus high osteotomy distraction osteogenesis ( p = 0.010) and reconstruction ( p = 0.041) cohorts. Intraoperative venous sinus injury was rare. In the distraction osteogenesis cohort, there was increased median intracranial volumetric gain in the low (263 ml) compared with the high osteotomy cohort (127 ml; p = 0.043); however, when controlled for distraction distance, only a trend was observed ( p = 0.221). Patients undergoing distraction osteogenesis showed a larger median intracranial volume increase (168 ml) compared with those undergoing reconstruction (73 ml; p0.001).Infratorcular osteotomy can be performed safely in most patients undergoing cranial vault remodeling and does not appear to be associated with greater hemodynamic instability or sinus injury.Therapeutic, III.

Background: Brachial plexus region tumors are rare. In this study, we reviewed our experience with resection of tumors involving or adjacent to the brachial plexus to identify patterns in presentation and outcome. Methods: We report a retrospective case series of brachial plexus tumors operated on by a single surgeon at a single institution over 15 years. Outcome data were recorded from the most recent follow-up office visit. Findings were compared to a prior internal series and comparable series in the literature. Results: From 2001 to 2016, 103 consecutive brachial plexus tumors in 98 patients met inclusion criteria. Ninety percent of patients presented with a palpable mass, and 81% had deficits in sensation, motor function, or both. Mean follow-up time was 10 months. Serious complications were infrequent. For patients with a preoperative motor deficit, the rate of postoperative motor decline was 10%. For patients without a preoperative motor deficit, the rate of postoperative motor decline was 35%, which decreased to 27% at 6 months. There were no differences in motor outcome based on extent of resection, tumor pathology, or age. Conclusion: We present one of the largest recent series of tumors of the brachial plexus region. Although the rate of worsened postoperative motor function was higher in those without preoperative weakness, the motor deficit improves over time and is no worse than antigravity strength in the majority of cases. Our findings help guide patient counseling in regard to postoperative motor function.

OBJECTIVE Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM. METHODS The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft. RESULTS A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain. CONCLUSIONS In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.

Anterior cranial vault fronto-orbital distraction osteogenesis is a promising treatment modality for children with unicoronal craniosynostosis. A minimally invasive, endoscope-assisted approach offers the additional potential benefits of less scalp scarring, decreased blood loss, and decreased scalp dissection. In this article, the authors present a novel technique for minimally invasive, endoscope-assisted fronto-orbital distraction osteogenesis.

BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P

Corpus callosotomy is a palliative surgical option for patients with refractory epilepsy and frequent drop attacks, decreasing seizure frequency and severity by disconnecting the cerebral hemispheres. Though often successful, open surgery is not without risk. Corpus callosotomy by MRI-guided laser interstitial thermal therapy (MRgLITT) is a minimally invasive alternative to the standard open procedure. This report aims to present an illustrative case and systematically review the current literature on the surgical technique. A systematic review of the English literature through January 2019 was performed. Articles were searched by title utilizing the following key word combinations: “laser” and “callosotomy”, “callosotomy” and “ablation”, “laser” and “corpus”, “callosotomy” and “thermal”, and “stereotactic” and callosotomy”. The articles recovered were then classified by level of evidence and summarized. Fifteen papers were reviewed, of which 6 met inclusion and exclusion criteria. All included studies were classified as level IV evidence. There was a total of 13 patients ranging from 13 months to 44 years old (mean 23.5 years old). The number of laser fibers utilized ranged from 1 to 3. Engel class I was achieved in 5 patients, II in 4, III in 2, IV in 1. Reported patient follow up was 4–39.7 months (mean 15.43). Corpus callosotomy utilizing MRI-guided laser interstitial thermal therapy results in improvement in seizure frequency and severity with minimal complications. Prospective trials are needed to compare its seizure control and long-term outcomes to that of standard open callosotomy.

BACKGROUND Thermal flow evaluation (TFE) is a non-invasive method to assess ventriculoperitoneal shunt function. Flow detected by TFE is a negative predictor of the need for revision surgery. Further optimization of testing protocols, evaluation in multiple centers, and integration with clinical and imaging impressions prompted the current study. OBJECTIVE To compare the diagnostic accuracy of 2 TFE protocols, with micropumper (TFE+MP) or without (TFE-only), to neuro-imaging in patients emergently presenting with symptoms concerning for shunt malfunction. METHODS We performed a prospective multicenter operator-blinded trial of a consecutive series of patients who underwent evaluation for shunt malfunction. TFE was performed, and preimaging clinician impressions and imaging results were recorded. The primary outcome was shunt obstruction requiring neurosurgical revision within 7 d. Non-inferiority of the sensitivity of TFE vs neuro-imaging for detecting shunt obstruction was tested using a prospectively determined a priori margin of −2.5%. RESULTS We enrolled 406 patients at 10 centers. Of these, 68/348 (20%) evaluated with TFE+MP and 30/215 (14%) with TFE-only had shunt obstruction. The sensitivity for detecting obstruction was 100% (95% CI: 88%-100%) for TFE-only, 90% (95% CI: 80%-96%) for TFE+MP, 76% (95% CI: 65%-86%) for imaging in TFE+MP cohort, and 77% (95% CI: 58%-90%) for imaging in the TFE-only cohort. Difference in sensitivities between TFE methods and imaging did not exceed the non-inferiority margin. CONCLUSION TFE is non-inferior to imaging in ruling out shunt malfunction and may help avoid imaging and other steps. For this purpose, TFE only is favored over TFE+MP.

Placement of ventriculoperitoneal shunts (VPS) can be challenging in children with previous VPS, previous abdominal surgery, or complex abdominal pathology. Laparoscopy can help identify intra-abdominal adhesions and determine the optimal site for insertion of the distal VPS catheter. We analyzed the feasibility and safety of laparoscopy assisted VPS placement (lapVPS) in children.A retrospective review from January/2015 to December/2018 was performed. Laparoscopy was performed via a 5 mm trans-umbilical port. Once the optimal entry site was identified, the distal end of the VPS was inserted by Seldinger technique under direct laparoscopic guidance.One hundred ten lapVPS procedures were reviewed. Median age was 1 (IQR 0.3-9.37) year. Fifty-five (50%) patients were1 year, and 15 (13.6%) were ≤28 days old. The mean age of the neonates was 14.7 (SD 7.6, range 4-28) days, and the mean weight was 3 (SD 0.39, range 2.4-3.7) kg. Thirty-one (28.2%) lapVPS were initial VPS placements, whereas 79 (71.8%) were subsequent VPS placements. Thirty-nine (35.5%) patients had a history of abdominal surgery or complex abdominal pathology. Median operative time was 36 (IQR 28-56) minutes. One hundred seven (97.3%) patients underwent successful lapVPS. Two (1.8%) patients underwent diagnostic laparoscopy, and lapVPS was deemed infeasible due to intraabdominal adhesions. One (0.9%) patient had an intestinal perforation from trocar placement. Three patients developed 5 postoperative complications that required a reoperation.Laparoscopy is a valuable tool to assess the suitability of the peritoneal cavity to accommodate a VPS. LapVPS is safe and can be performed in children of all ages.Level IV.

OBJECTIVES Spring-mediated cranial vault expansion (SMC) may enable less invasive treatment of sagittal craniosynostosis than conventional methods. The influence of spring characteristics such as force, length, and quantity on cranial vault outcomes are not well understood. Using in vivo and ex vivo models, we evaluate the interaction between spring force, length, and quantity on correction of scaphocephalic deformity in patients undergoing SMC. METHODS The authors retrospectively studied subjects with isolated sagittal craniosynostosis who underwent SMC between 2011 and 2019. The primary outcome measure of in vivo analysis was head shape determined by cephalic index (CI). Ex vivo experimentation analyzed the impact of spring length, bend, and thickness on resultant force. RESULTS Eighty-nine subjects underwent SMC at median 3.4 months with median preoperative CI 69% (interquartile range: 66, 71%). Twenty-six and 63 subjects underwent SMC with 2 and 3 springs, with mean total force 20.1 and 27.6 N, respectively (P < 0.001).Postoperative CI increased from 71% to 74% and 68% to 77% in subjects undergoing 2- and 3-spring cranioplasty at the 6-month timepoint, respectively (P < 0.001). Total spring force correlated to increased change in CI (P < 0.002). Spring length was inversely related to transverse cranial expansion at Postoperative day 1, however, directly related at 1 and 3 months (P < 0.001). Ex vivo modeling of spring length was inversely related to spring force regardless of spring number (P < 0.0001). Ex vivo analysis demonstrated greater resultant force when utilizing wider, thicker springs independent of spring arm length and degree of compression. CONCLUSIONS A dynamic relationship among spring characteristics including length, bend, thickness, and quantity appear to influence SMC outcomes.

Introduction: Ultrasound (US)-based indexes such as fronto-occipital ratio (FOR) can be used to obtain an acceptable estimation of ventricular volume. Patients with colpocephaly present a unique challenge due to the shape of their ventricles. In the present study, we aimed to evaluate the validity and reproducibility of the modified US-FOR index in children with Chiari II-related ventriculomegaly. Methods: In this retrospective study, we evaluated Chiari II patients younger than 1 year who underwent head US and MR or CT scans for ventriculomegaly evaluation. MR/CT-based FOR was measured in the axial plane by identifying the widest diameter of frontal horns, occipital horns, and the interparietal diameter (IPD). US-based FOR (US-FOR) was measured using the largest diameter based on the following landmarks: frontal horn and IPD in the coronal plane at the level of the foramen of Monro, IPD just superior to the Sylvian fissures, and occipital horn posterior to the thalami and inferior to the superior margins of the thalami. Intraclass correlation coefficients (ICCs) were used to evaluate inter-rater reliability, and Pearson correlation coefficients and Bland-Altman plots were applied to assess agreement between US and other two modalities. Results: Sixty-three paired US and MR/CT exams were assessed for agreement between US-FOR and MR/CT-FOR measurements. ICC showed an excellent inter-rater reliability for US-FOR (ICC = 0.99, p < 0.001) and MR/CT-FOR (ICC = 0.99, p < 0.001) measurements. The mean (range) values based on US-FOR showed a slight overestimation in comparison with MR/CT-FOR (0.51 [0.36–0.68] vs. 0.46 [0.34–0.64]). The Pearson correlation coefficient showed high cross-modality agreement for the FOR index (r = 0.83, p < 0.001). The Bland-Altman plot showed excellent concordance between US-FOR and MR/CT-FOR with a bias of 0.05 (95% CI: −0.03 to 0.13). Conclusion: US-FOR in the coronal plane is a comparable tool for evaluating ventriculomegaly in Chiari II patients when compared with MR/CT-FOR, even in the context of colpocephaly.

BACKGROUND The Management of Myelomeningocele Study (MOMS) demonstrated that fetal myelomeningocele (fMMC) closure results in improved hydrocephalus and hindbrain herniation when compared to postnatal closure. OBJECTIVE To report on the outcomes of a single institution's experience in the post-MOMS era, with regard to hydrocephalus absence and hindbrain herniation resolution. METHODS A single-center retrospective study of a subset of post-MOMS patients who underwent fetal/postnatal myelomeningocele closure was performed. Primary outcomes included cerebrospinal fluid (CSF) diversion status and hindbrain herniation resolution. Families were contacted via telephone for outcome information if care was transitioned to outside institutions. Univariate/multivariable analyses were performed using several prenatal and postnatal variables. RESULTS From January 2011 to May 2016, data were reviewed from families of 62 postnatal and 119 fMMC closure patients. In the postnatal group, 80.6% required CSF diversion compared to 38.7% fetal cases (P

Conjoined twins who are classified as craniopagus (joined at the cranium) have a rare congenital anomaly. Despite advances in surgical techniques and critical care, the rate of complications and death is still high among twins with total fusion in which the superior sagittal sinus is shared. Here, we describe total-fusion craniopagus twins who at 10 months of age underwent successful surgical separation performed by a multidisciplinary team. Computer-aided design and modeling with a three-dimensional printer, custom-designed cranial distraction and constriction devices, and intraoperative navigation techniques were used. These techniques allowed for separation of the twins at an early age and harnessed the regenerative capacity of their young brains.

OBJECTIVEEvaluation of increased intracranial pressure (ICP) in the pediatric craniosynostosis population based solely on ophthalmological, clinical, and radiographic data is subjective, insensitive, and inconsistent. The aim of this study was to examine the intraoperative ICP before and after craniectomy in this patient population.METHODSThe authors measured the ICP before and after craniectomy using a subdural ICP monitor in 45 children. They regulated end-tidal carbon dioxide and the monitoring site under general anesthesia to record consistent ICP readings.RESULTSThe average age of the patient population was 29 months (range 3.8–180.5 months). Thirty-seven patients (82.2%) were undergoing initial craniosynostosis procedures. All craniosynostosis procedures were categorized as one of the following: frontoorbital advancement (n = 24), frontoorbital advancement with distraction osteogenesis (n = 1), posterior vault distraction osteogenesis (n = 10), and posterior vault reconstruction (n = 10). Nineteen of 45 patients (42.2%) had syndromic or multisuture craniosynostosis. The mean postcraniectomy ICP (8.8 mm Hg, range 2–18 mm Hg) was significantly lower than the precraniectomy ICP (16.5 mm Hg, range 6–35 mm Hg) (p < 0.001). Twenty-four patients (53%) had elevated ICP prior to craniectomy, defined as ≥ 15 mm Hg. Only 4 (8.9%) children had papilledema on preoperative funduscopic examination (sensitivity 17%, specificity 100%, negative predictive value 51%, and positive predictive value 100%). There were no significant differences in elevated precraniectomy ICP based on type of craniosynostosis (syndromic/multisuture or nonsyndromic) or age at the time of surgery. Patients undergoing initial surgery in the first 12 months of life were significantly less likely to have elevated precraniectomy ICP compared with patients older than 12 months (26.3% vs 73.1%, p = 0.005).CONCLUSIONSIn this study, the authors report the largest cohort of syndromic and nonsyndromic craniosynostosis patients (n = 45) who underwent precraniectomy and postcraniectomy ICP evaluation. A craniectomy or completed craniotomy cuts for distractors effectively reduced ICP in 43/45 patients. The authors’ findings support the notion that papilledema on funduscopy is a highly specific, however poorly sensitive, indicator of increased ICP, and thus is not a reliable screening method. These findings indicate that even nonsyndromic patients with craniosynostosis are at risk for increased ICP. Furthermore, patients who present prior to 12 months of age appear less likely to have elevated ICP on presentation. Further studies with other noninvasive imaging of the retina may be useful as an adjunct tool for determining elevated ICP.

Neurogenic thoracic outlet syndrome (nTOS) describes a set of disorders related to symptomatic compression of the brachial plexus as it passes through the thoracic outlet into the arm. In children, nTOS is a rare and frequently delayed diagnosis due to the difficulty of communicating symptoms with adults and healthcare providers and decreased prevalence of the syndrome compared to adult nTOS. Additionally, nTOS is an uncommon variant of pediatric thoracic outlet syndrome, which is predominantly arterial or vascular in nature. In this chapter we review the presentation, associated radiographic and exam findings, and management for pediatric nTOS. We summarize the outcomes for pediatric nTOS reported in the literature to date, and also present our institutional experience managing these challenging and uncommon patients.

BACKGROUND Patients undergoing cranial expansion including spring-mediated cranioplasty (SMC) and cranial vault remodeling (CVR) receive costly and high acuity post-operative intensive care (ICU) given concerns over neurologic and hemodynamic vulnerability. The authors analyzed perioperative and post-operative events for patients presenting with sagittal craniosynostosis (CS) undergoing SMC and CVR in order to compare complication profiles. METHODS The authors performed a single center retrospective cohort study of patients undergoing SMC and CVR for the treatment of nonsyndromic, isolated sagittal CS from 2011 to 2018. Perioperative and post-operative factors were collected, focusing on hemodynamic instability and events necessitating ICU care. Mann-Whitney U and Fisher exact tests were used to compare data with significance defined as P

OBJECTIVE Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

Study design This is a level IV retrospective descriptive study at a single institution. Objective The objective of the study was to determine the preoperative signs or symptoms prompting cervicomedullary imaging in Jeune syndrome. Summary of background data Jeune syndrome is a rare autosomal recessive disorder that results in pulmonary compromise from abnormal development of the thorax. Multiple medical comorbidities complicate timely diagnosis of cervicomedullary stenosis, which neurologically jeopardizes this patient population with regards to improper cervical manipulation. Currently, explicit screening of the cervicomedullary junction is not advocated in national guidelines. Methods The User Reporting Workbench and Center for Thoracic Insufficiency Syndrome (CTIS) Safety Registry was queried for patients with Jeune syndrome under the age of 18 with cervicomedullary stenosis with or without suboccipital craniectomy/craniotomy evaluated at the authors' institution from January 1, 2007 to August 21, 2018. The primary outcome was the clinical reason for cervicomedullary screening. Secondary outcomes were: age at time of surgery, preoperative myelopathy (spasticity, urinary retention), hydrocephalus, postoperative deficits (respiratory, motor, swallowing difficulty), and need for cervical fusion. Results Of 32 patients with Jeune syndrome, four (12.5%) had cervicomedullary stenosis requiring decompression. The average age at surgery was 5.25 months (2-9 mo). Two patients underwent imaging due to desaturation events while the other two patients were diagnosed with cervical stenosis as an incidental finding. No patients exhibited clinical myelopathy. Two patients had baseline preoperative swallowing difficulties. None of the patients postoperatively required cervical fusions, nor did they exhibit respiratory deficits, motor deficits, or worsening swallowing difficulties. Conclusion Jeune patients should be routinely screened for cervicomedullary stenosis and undergo subsequent prophylactic decompression to minimize or eliminate the development of irreversible neurologic compromise. Level of evidence 4.

OBJECTIVENeurogenic thoracic outlet syndrome (nTOS) is an uncommon compression syndrome of the brachial plexus that presents with pain, sensory changes, and motor weakness in the affected limb. The authors reviewed the clinical presentations and outcomes in their series of pediatric patients with surgically treated nTOS over a 6-year period.METHODSCases of nTOS in patients age 18 years or younger were extracted for analysis from a prospective database of peripheral nerve operations. Baseline patient characteristics, imaging and neurophysiological data, operative findings, and outcomes and complications were assessed.RESULTSTwelve patients with 14 cases of nTOS surgically treated between April 2010 and December 2016 were identified. One-third of the patients were male, and 2 male patients underwent staged, bilateral procedures. Disabling pain (both local and radiating) was the most common presenting symptom (100%), followed by numbness (35.7%), then tingling (28.6%). The mean duration of symptoms prior to surgery was 15.8 ± 6.6 months (mean ± SD). Sports-related onset of symptoms was seen in 78.6% of cases. Imaging revealed cervical ribs in 4 cases, prominent C-7 transverse processes in 4 cases, abnormal first thoracic ribs in 2 cases, and absence of bony anomalies in 4 cases. Neurophysiological testing results were normal in 85.7% of cases. Conservative management failed in all patients, with 5 patients reporting minimal improvement in symptoms with physical therapy. With a mean follow-up after surgery of 22 ± 18.3 months (mean ± SD), pain relief was excellent (> 90%) in 8 cases (57.1%), and good (improved > 50%) in 6 cases (42.9%). On univariate analysis, patients who reported excellent pain resolution following surgery at long-term follow-up were found to be significantly younger, and to have suffered a shorter duration of preoperative symptoms than patients who had worse outcomes. Lack of significant trauma or previous surgery to the affected arm was also associated with excellent outcomes. There were 4 minor complications in 3 patients within 30 days of surgery: 1 patient developed a small pneumothorax that resolved spontaneously; 1 patient suffered a transient increase in pain requiring consultation, followed by hiccups for a period of 3 hours that resolved spontaneously; and 1 patient fell at home, with transient increased pain in the surgically treated extremity. There were no new neurological deficits, wound infections, deep vein thromboses, or readmissions.CONCLUSIONPediatric nTOS commonly presents with disabling pain and is more frequently associated with bony anomalies compared with adult nTOS. In carefully selected patients, surgical decompression of the brachial plexus results in excellent pain relief, which is more likely to be seen in younger patients who present for early surgical evaluation.

Open spina bifida or myelomeningocele (MMC) is one of the most common serious congenital malformations. Historically, this condition has been treated with closure of the MMC defect shortly after birth. The goal of postnatal closure is to cover the exposed spinal cord and prevent infection. However, postnatal surgery does not reverse or prevent the neurologic injury seen in MMC, reverse hindbrain herniation, or prevent hydrocephalus. The neurologic defects result from primary incomplete neurulation and secondary chronic prenatal damage to the exposed neural elements through mechanical and chemical trauma. In a hope to reduce the numerous surgical procedures, medical complications, and lifelong disabilities associated with MMC, the proposal of prenatal closure was put forth more than two decades ago. After promising results in animal models and some clinical series, a randomized controlled trial, the Management of Myelomeningocele Study (MOMS), was conducted. The MOMS trial demonstrated that closure during the prenatal period could be performed relatively safely and can result in significant benefit to the child. Specifically, prenatal closure results in improved motor function, reduced hindbrain herniation, and reduced need for a cerebral spinal fluid diversion. Long-term outcomes of the patients in the MOMS trial continues in the MOMS 2 study as these children grow. Additionally, investigations are underway on modifications to the open fetal MMC closure techniques.

Chiari malformation type-1 (CM-1) may be treated by intradural (ID) or extradural (ED) posterior fossa decompression, although the optimal approach is debated. The Chiari Severity Index (CSI) is a pre-operative metric to predict patient-defined improvement after CM-1 surgery. In this study, we evaluate the results of ID versus ED decompression and assess the external validity of the CSI. We performed a retrospective cohort study of pediatric CM-1 patients undergoing decompression at a single academic children’s hospital. Characteristics of headache, syrinx, and myelopathy were collected to derive CSI grade. The primary outcome measure was pre-operative symptom resolution. The proportion of patients with favorable outcome was tabulated for each of the three CSI grades and compared to previously published results. From 2004 to 2014, 189 patients underwent ID (48%) or ED (52%) decompression at the Children’s Hospital of Philadelphia (CHOP). Follow-up ranged from 1 to 75 months. Rates of symptom resolution (58–64%) and reoperation (8%) were similar regardless of surgical approach. Although proportions of favorable outcomes differed between the CHOP and Washington University (WU) cohorts, the difference was not related to CSI grade (p = 0.63). Furthermore, there was no difference in the proportion of favorable outcomes between the two cohorts regardless of ID (p = 0.26) or ED approach (p = 0.11). Equivalent rates of symptom resolution and reoperation following ID and ED decompression support the ED approach as a first-line surgical option for pediatric CM-1 patients. In addition, our findings provide preliminary evidence supporting the generalizability of the CSI and its use in future comparative trials.

OBJECTIVE To determine whether the presence of a myelomeningocele (MMC) sac and sac size correlate with compromised lower-extremity function in fetuses with open spinal dysraphism. METHODS A radiology database search was performed to identify cases of MMC and myeloschisis (MS) diagnosed prenatally in a single center from 2013 to 2017. All cases were evaluated between 18 and 25 weeks. Ultrasound reports were reviewed for talipes and impaired lower-extremity motion. In MMC cases, sac volume was calculated from ultrasound measurements. Magnetic resonance imaging reports were reviewed for hindbrain herniation. The association of presence of a MMC sac and sac size with talipes and impaired lower-extremity motion was assessed. Post-hoc analysis of data from the multicenter Management of Myelomeningocele Study (MOMS) randomized controlled trial was performed to confirm the study findings. RESULTS In total, 283 MMC and 121 MS cases were identified. MMC was associated with a lower incidence of hindbrain herniation than was MS (80.9% vs 100%; P < 0.001). Compared with MS cases, MMC cases with hindbrain herniation had a higher rate of talipes (28.4% vs 16.5%, P = 0.02) and of talipes or lower-extremity impairment (34.9% vs 19.0%, P = 0.002). Although there was a higher rate of impaired lower-extremity motion alone in MMC cases with hindbrain herniation than in MS cases, the difference was not statistically significant (6.6% vs 2.5%; P = 0.13). Among MMC cases with hindbrain herniation, mean sac volume was higher in those associated with talipes compared with those without talipes (4.7 ± 4.2 vs 3.0 ± 2.6 mL; P = 0.002). Review of the MOMS data demonstrated similar findings; cases with a sac on baseline imaging had a higher incidence of talipes than did those without a sac (28.2% vs 7.5%; P = 0.007). CONCLUSIONS In fetuses with open spinal dysraphism, the presence of a MMC sac was associated with fetal talipes, and this effect was correlated with sac size. The presence of a larger sac in fetuses with open spinal dysraphism may result in additional injury through mechanical stretching of the nerves, suggesting another acquired mechanism of injury to the exposed spinal tissue. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Children with myelomeningocele (MMC) are at increased risk of developing neuromuscular scoliosis and spinal cord re-tethering (Childs Nerv Syst 12:748-754, 1996; Neurosurg Focus 16:2, 2004; Neurosurg Focus 29:1, 2010). Some centers perform prophylactic untethering on asymptomatic MMC patients prior to scoliosis surgery because of concern that additional traction on the cord may place the patient at greater risk of neurologic deterioration peri-operatively. However, prophylactic untethering may not be justified if it carries increased surgical risks. The purpose of this study was to determine if prophylactic untethering is necessary in asymptomatic children with MMC undergoing scoliosis surgery. A multidisciplinary, retrospective cohort study from seven children’s hospitals was performed including asymptomatic children with MMC

There is no clear consensus for the optimal treatment of sagittal craniosynostosis; however, recent studies suggest that improved neurocognitive outcomes may be obtained when surgical intervention imparts active cranial expansion or remodeling and is performed before 6 months of age. The authors consider spring-mediated cranioplasty (SMC) to optimally address these imperatives, and this is an investigation of how helmet orthoses before or after SMC affect aesthetic outcomes.The authors retrospectively evaluated patients treated with SMC and adjunct helmeting for sagittal synostosis. Patients were stratified into 4 cohorts based on helmet usage: preop, postop, both, and neither. The cephalic index (CI) was used to assess head shape changes and outcomes. Twenty-six patients met inclusion criteria: 6 (23%) had preop, 11 (42%) had postop, 4 (15%) had preop and postop, and 5 (19%) had no helmeting. Average age at surgery was 3.6 months. Overall, CI improved from a mean 69.8 to 77.9 during an average 7-month course of care. Mean preoperative change in CI showed greater improvement with preop helmet (1.3) versus not (0.0), (P = 0.029), despite similar initial CI in these cohorts (70.4 and 69.6 respectively, P = 0.69). Nonetheless, all patient cohorts regardless of helmeting status achieved similar final CIs (range 76.4-80.4; P = 0.72).In summary, preoperative molding helmet therapy leads to improved CI at the time of spring-mediated cranioplasty. However, this benefit does not necessarily translate into overall improved CI after surgery and in follow-up, calling into question the benefits of molding helmet therapy in this setting.

There are a variety of imaging modalities for evaluation of peripheral nerves. Of these, ultrasonography (US) is often underused. There are several advantages of this imaging modality, including its cost-effectiveness, time-efficient assessment of long segments of peripheral nerves, ability to perform dynamic maneuvers, lack of contraindications, portability, and noninvasiveness. It can provide diagnostic information that cannot be obtained by electrophysiologic or, in some cases, magnetic resonance imaging studies. Ideally, the neurosurgeon can use US as a diagnostic adjunct in the preoperative assessment of a patient with traumatic, neoplastic, infective, or compressive nerve injury. Perhaps its most unique use is in intraoperative surgical planning. In this article, a brief description of normal US nerve anatomy is presented followed by a description of the US appearance of peripheral nerve disease caused by trauma, tumor, infection, and entrapment.

OBJECT Mutations in the sodium channel alpha 1 subunit gene (SCN1A) have been associated with a wide range of epilepsy phenotypes including Dravet syndrome. There currently exist few histopathological and surgical outcome reports in patients with this disease. In this case series, the authors describe the clinical features, surgical pathology, and outcomes in 6 patients with SCN1A mutations and refractory epilepsy who underwent focal cortical resection prior to uncovering the genetic basis of their epilepsy. METHODS Medical records of SCN1A mutation-positive children with treatment-resistant epilepsy who had undergone resective epilepsy surgery were reviewed retrospectively. Surgical pathology specimens were reviewed. RESULTS All 6 patients identified carried diagnoses of intractable epilepsy with mixed seizure types. Age at surgery ranged from 18 months to 20 years. Seizures were refractory to surgery in every case. Surgical histopathology showed evidence of subtle cortical dysplasia in 4 of 6 patients, with more neurons in the molecular layer of the cortex and white matter. CONCLUSIONS Cortical resection is unlikely to be beneficial in these children due to the genetic defect and the unexpected neuropathological finding of mild diffuse malformations of cortical development. Together, these findings suggest a diffuse pathophysiological mechanism of the patients’ epilepsy which will not respond to focal resective surgery.

BACKGROUND The effect of modifications in fetal myelomeningocele (fMMC) closure techniques has not been extensively studied. OBJECTIVE To study the effect of a modified closure technique on fMMC postnatal patient outcomes: hydrocephalus, hindbrain herniation, and cyst development. METHODS We performed single-center retrospective study of a subset of post-MOMS (Management of Myelomeningocele Study) trial patients who underwent fMMC closure. After January 2015, the fetal myofascial closure technique was modified. Needlepoint monopolar cautery was used to raise dural lined myofascial flaps to create a more robust closure. Outcomes between the pre- and postmodification groups were compared with regard to hindbrain herniation, hydrocephalus, and cyst development. Families who transitioned care to local institutions were contacted via telephone for outcome information. RESULTS From January 2011 to May 2016, data were reviewed from 119 fMMC closure patients. Patients without full follow-up data were excluded from the final analysis. Cerebrospinal fluid diversion was seen in 32 of 74 patients with the standard technique compared to 14 of 45 with the modified closure and was significantly decreased in postmodification when compared to that of the MOMS trial (P = .01). Hindbrain herniation resolution was significantly decreased in both the pre- and postmodification groups compared to that of the MOMS trial (P

OBJECTIVEThe Management of Myelomeningocele Study (MOMS) compared prenatal with postnatal surgery for myelomeningocele (MMC). The present study sought to determine how MOMS influenced the clinical recommendations of pediatric neurosurgeons, how surgeons’ risk tolerance affected their views, how their views compare to those of their colleagues in other specialties, and how their management of hydrocephalus compares to the guidelines used in the MOMS trial.METHODSA cross-sectional survey was sent to all 154 pediatric neurosurgeons in the American Society of Pediatric Neurosurgeons. The effect of surgeons’ risk tolerance on opinions and counseling of prenatal closure was determined by using ordered logistic regression.RESULTSCompared to postnatal closure, 71% of responding pediatric neurosurgeons viewed prenatal closure as either “very favorable” or “somewhat favorable,” and 51% reported being more likely to recommend prenatal surgery in light of MOMS. Compared to pediatric surgeons, neonatologists, and maternal-fetal medicine specialists, pediatric neurosurgeons viewed prenatal MMC repair less favorably (p < 0.001). Responders who believed the surgical risks were high were less likely to view prenatal surgery favorably and were also less likely to recommend prenatal surgery (p < 0.001). The management of hydrocephalus was variable, with 60% of responders using endoscopic third ventriculostomy in addition to ventriculoperitoneal shunts.CONCLUSIONSThe majority of pediatric neurosurgeons have a favorable view of prenatal surgery for MMC following MOMS, although less so than in other specialties. The reported acceptability of surgical risks was strongly predictive of prenatal counseling. Variation in the management of hydrocephalus may impact outcomes following prenatal closure.

This paper describes some of the more common patterns in neurobehavioral deficits and their underlying neuroanatomical basis in myelomeningocele (MMC). Patients with MMC can face a lifetime of specific organ system dysfunction, chief among them spinal cord malformations, orthopedic issues, hydrocephalus, and urological disabilities. In addition, patients can experience specific patterns of neurobehavioral difficulties due to the changes in neuroanatomy associated with the open spinal defect. Although there is variability in these patterns, some trends have been described among MMC patients. It is thought that early recognition of these potential neurobehavioral deficits by treating neurosurgeons and other members of the treatment team could lead to earlier intervention and positively impact the overall outcome for patients. Neurodevelopmental and neurobehavioral follow-up assessments are recommended to help guide planning for relevant treatments or accommodations.

Myelomeningocele (MMC) affects approximately 2000 infants in the US yearly and causes long-term damage to the genitourinary system. There is a wide spectrum of effects on the bladder and urethral sphincter that do not correlate with the level of the spinal cord defect. The bladder changes can provide a safe storage pressure, often at the expense of continence, or can create a hostile environment leading to increased pressure on the kidneys and eventually to chronic kidney disease. The ultimate goals in management are to maintain a safe system that prevents renal deterioration, while providing social continence and independence in the long term. This review intends to provide an overview of the effects of MMC on the genitourinary system, regardless of intervention, in order to understand the pathophysiology of the disease.

OBJECTIVEComplications after complex tethered spinal cord (cTSC) surgery include infections and cerebrospinal fluid (CSF) leaks. With little empirical evidence to guide management, there is variability in the interventions undertaken to limit complications. Expert-based best practices may improve the care of patients undergoing cTSC surgery. Here, authors conducted a study to identify consensus-driven best practices.METHODSThe Delphi method was employed to identify consensual best practices. A literature review regarding cTSC surgery together with a survey of current practices was distributed to 17 board-certified pediatric neurosurgeons. Thirty statements were then formulated and distributed to the group. Results of the second survey were discussed during an in-person meeting leading to further consensus, which was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree).RESULTSSeventeen consensus-driven best practices were identified, with all participants willing to incorporate them into their practice. There were four preoperative interventions: (1, 2) asymptomatic AND symptomatic patients should be referred to urology preoperatively, (3, 4) routine preoperative urine cultures are not necessary for asymptomatic AND symptomatic patients. There were nine intraoperative interventions: (5) patients should receive perioperative cefazolin or an equivalent alternative in the event of allergy, (6) chlorhexidine-based skin preparation is the preferred regimen, (7) saline irrigation should be used intermittently throughout the case, (8) antibiotic-containing irrigation should be used following dural closure, (9) a nonlocking running suture technique should be used for dural closure, (10) dural graft overlay should be used when unable to obtain primary dural closure, (11) an expansile dural graft should be incorporated in cases of lipomyelomeningocele in which primary dural closure does not permit free flow of CSF, (12) paraxial muscles should be closed as a layer separate from the fascia, (13) routine placement of postoperative drains is not necessary. There were three postoperative interventions: (14) postoperative antibiotics are an option and, if given, should be discontinued within 24 hours; (15) patients should remain flat for at least 24 hours postoperatively; (16) routine use of abdominal binders or other compressive devices postoperatively is not necessary. One intervention was prioritized for additional study: (17) further study of additional gram-negative perioperative coverage is needed.CONCLUSIONSA modified Delphi technique was used to develop consensus-driven best practices for decreasing wound complications after cTSC surgery. Further study is required to determine if implementation of these practices will lead to reduced complications. Discussion through the course of this study resulted in the initiation of a multicenter study of gram-negative surgical site infections in cTSC surgery.

Myelomeningocele is one of the most common congenital malformations. A randomized controlled trial, known as the Management of Myelomeningocele Study (MOMS), demonstrated that closure during the fetal period can be performed relatively safely and be of significant benefit to patients. However, postnatally, patients can develop resultant symptoms from a tethered cord and inclusion cysts; this often requires surgical treatment. Repeat surgery in this population can be challenging due to the age of the patients, the extent of surgical exposure needed and the need for resection of dermal and epidermal tissues in the midline. We describe our approach for closure of these complex defects using lateral fasciocutaneous flaps with relaxing incisions made in the posterior axillary line, in order to minimize tension and maximize soft tissue coverage of the midline.

BACKGROUND: Ventricular shunt complications in children can be severe and life-threatening if not identified and treated in a timely manner. Evaluation for shunt obstruction is not without risk, including lifetime cumulative radiation as patients routinely receive computed tomography (CT) scans of the brain and shunt series (multiple radiographs of the skull, neck, chest, and abdomen). METHODS: A multidisciplinary team collaborated to develop a clinical pathway with the goal of standardizing the evaluation and management of patients with suspected shunt complication. The team implemented a low-dose CT scan, specifically tailored for the detection of hydrocephalus and discouraged routine use of shunt series with single-view radiographs used only when specifically indicated. RESULTS: There was a reduction in the average CT effective dose (millisievert) per emergency department (ED) encounter of 50.6% (confidence interval, 46.0–54.9; P ≤ .001) during the intervention period. There was a significant reduction in the number of shunt surveys obtained per ED encounter, from 62.4% to 5.32% (P < .01). There was no significant change in the 72-hour ED revisit rate or CT scan utilization rate after hospital admission. There were no reports of inadequate patient evaluations or serious medical events. CONCLUSIONS: A new clinical pathway has rapidly reduced radiation exposure, both by reducing the radiation dose of CT scans and eliminating or reducing the number of radiographs obtained in the evaluation of patients with ventricular shunts without compromising clinical care.

The International Subarachnoid Aneurysm Trial heralded a paradigm shift in the treatment of intracranial aneurysms. During this same time frame, neurosurgical training programs increased in size and scope. The present study examines the impact of trends in surgical clipping and the endovascular treatment of intracranial aneurysms, over one decade, and the neurosurgical resident complement on the resident teaching environment using the Nationwide Inpatient Sample (NIS).The NIS was used to estimate the number of aneurysms treated with either surgical clipping and endovascular methods from 2002 through 2011 at teaching institutions. Teaching opportunities per year per resident or chief resident were calculated as the ratio of the number of specified cases to the average number of neurosurgical trainees by year. Annualized trends were assessed.Over the study period, the percent change in odds of occurrence of a clipped ruptured aneurysm was -15.6% per year (P0.001) and of ruptured aneurysms undergoing endovascular treatment was 18.7% per year (P 0.001) within teaching institutions. This corresponded to a decline in teaching opportunities for clipped ruptured aneurysms for both residents and chief residents (P 0.001). In contrast, teaching opportunities for endovascular treatment of both ruptured and unruptured aneurysms increased dramatically over the study period.There has been a significant decrease in opportunity for operative exposure to craniotomy for ruptured aneurysm clipping over the past decade, whereas the volume of endovascular procedures for aneurysms has dramatically increased, highlighting the need for a shift in training strategy for those neurosurgeons graduating from residency desiring to subspecialize in neurovascular neurosurgery.

Myelomeningocele (MMC) is one of the most common serious congenital malformations. Typically this condition has been treated with closure of the MMC defect shortly after birth. In general, surgery for MMC aims to provide a multilayered closure to provide protection to the neural elements, prevent leakage of spinal fluid and reduce infection risks. A randomized controlled trial, the Management of Myelomeningocele Study (MOMS), has shown that closure during the fetal period can be performed relatively safely and can result in significant benefit to the child. Whereas the surgical technique of prenatal closure of an MMC defect is similar to a postnatal closure, there are some important technical differences. The goal of this paper is to describe the technique of fetal closure of MMC defects, highlight the unique steps that are needed for this surgery and delineate some potential pitfalls.

Object Fetal myelomeningocele closure has been shown to be advantageous in a number of areas. In this study, the authors report on neural function in patients who had previously undergone fetal myelomeningocele repair and returned to the authors' institution for further surgery that included intraoperative neurophysiological monitoring. Methods The authors retrospectively reviewed data obtained in 6 cases involving patients who underwent fetal myelomeningocele repair and later returned to their institution for spinal cord untethering. (In 4 of the 6 cases, the patients also underwent removal of a dermoid cyst [3 cases] or removal of an epidermoid cyst [1 case] during the untethering procedure.) Records and imaging studies were reviewed to identify the anatomical level of the myelomeningocele as well as the functional status of each patient. Stimulated electromyography (EMG) and transcranial motor evoked potential (tcMEP) recordings obtained during surgery were reviewed to assess the functional integrity of the nerve roots and spinal cord. Results During reexploration, all patients had reproducible signals at or below their anatomical level on stimulated EMG and tcMEP recordings. Corresponding to these findings, prior to tethering, all patients had antigravity muscle function below their anatomical level. Conclusions All 6 patients had lower-extremity function and neurophysiological monitoring recording signals at or below their anatomical level. These cases provide direct evidence of spinal cord and nerve root conductivity and functionality below the anatomical level of the myelomeningocele, further supporting that neurological status improves with fetal repair.

Object Neonatal brachial plexus palsy (NBPP) represents a significant health problem with potentially devastating consequences. The most common form of NBPP involves the upper trunk roots. Currently, primary surgical repair is performed if clinical improvement is lacking. There has been increasing interest in “early” surgical repair of NBPPs, occurring within 3–6 months of life. However, early treatment recommendations ignore spontaneous recovery in cases of Erb's palsy. This study was undertaken to evaluate the optimal timing of surgical repair in this group with respect to quality of life. Methods The authors formulated a decision analytical model to compare 4 treatment strategies (no repair or repair at 3, 6, or 12 months of life) for infants with persistent NBPPs. The model derives data from a critical review of published studies and projects health-related quality of life and quality-adjusted life years over a lifetime. Results When evaluating the quality of life of infants with NBPP, improved outcomes are seen with delayed surgical repair at 12 months, compared with no repair or repair at early and intermediate time points, at 3 and 6 months, respectively. ANOVA showed that the differences among the 4 groups are highly significant (F = 8369; p < 0.0001). Pairwise post hoc comparisons revealed that there are highly significant differences between each pair of strategies (p < 0.0001). Meta-regression showed no evidence of improved outcomes with more recent treatment dates, compared with older ones, for either nonsurgical or for surgical treatment (p = 0.767 and p = 0.865, respectively). Conclusions These data support a delayed approach of primary surgical reconstruction to optimize quality of life. Early surgery for NBPPs may be an overly aggressive strategy for infants who would otherwise demonstrate spontaneous recovery of function by 12 months. A randomized, controlled trial would be necessary to fully elucidate the natural history of NBPP and determine the optimal time point for surgical intervention.