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The phakomatoses are a group of genetic and acquired disorders characterized by neurologic, cutaneous, and often ocular manifestations, thus commonly referred to as neurocutaneous syndromes. In several of these conditions the underlying genetic pathophysiology has been elucidated, which will continue to play an important role in advancing therapeutic techniques. This article focuses on several examples of such neurocutaneous syndromes, with special attention to the relevant neurosurgical considerations of these patients.

Purpose The purpose of this study was to investigate associations between the various definitions of adding-on identified in the literature and HRQoL at 10 years following posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) to identify a clinically relevant definition. Methods In this retrospective cohort study, patients with Lenke type 1 or 2 AIS with 10-year follow-up after PSF were identified in a multicenter registry. Adding-on was identified independently using 5 published definitions and expert spine surgeons' visual confirmation. Worsening of HRQoL was defined as a preoperative to 10-year postoperative decrease in SRS-22 domain score ≥ the minimal clinically important difference (MCID). Results One hundred fifty patients were included, and 118 had HRQoL data available for the main analysis. Worsening pain was noted in 49 (41.5%) patients, appearance in 1 (0.8%), and activity in 70 (59.3%) from preoperative to postoperative. 4 of the 5 definitions of adding-on demonstrated no evidence of association with worsening HRQoL. By Cho's definition (either 1. ≥ 5° increase in Cobb angle AND increase in number of vertebrae in curve distally, or 2. ≥ 5° increase in angulation of 1st disc below LIV), adding-on (27 patients, 22.9%) was associated with an increased risk of worsening pain (59.3% vs. 36.3%, p = 0.033) and activity (77.8% vs. 53.8%, p = 0.026). There was no evidence of association with changes in appearance. Risk of worsening pain and activity was increased by 1.75 and 1.51 times (p = 0.023 and p = 0.002), respectively. Conclusion Cho's definition of adding-on was associated with worsening HRQoL at 10 years following PSF for Lenke type 1 and 2 AIS. Consistent use of this definition will allow us to compare studies and obtain meaningful information in this population. Level of evidence Level II.

OBJECTIVE The condylar–C2 sagittal vertical alignment (C-C2SVA) describes the relationship between the occipitoatlantal joint and C2 in patients with Chiari malformation type I (CM-I). It has been suggested that a C-C2SVA ≥ 5 mm is predictive of the need for occipitocervical fusion (OCF) or ventral brainstem decompression (VBD). The authors’ objective was to validate the predictive utility of the C-C2SVA by using a large, multicenter cohort of patients. METHODS This validation study used a cohort of patients derived from the Park-Reeves Syringomyelia Research Consortium; patients < 21 years old with CM-I and syringomyelia treated from June 2011 to May 2016 were identified. The primary outcome was the need for OCF and/or VBD. After patients who required OCF and/or VBD were identified, 10 age- and sex-matched controls served as comparisons for each OCF/VBD patient. The C-C2SVA (defined as the position of a plumb line from the midpoint of the O–C1 joint relative to the posterior aspect of the C2–3 disc space), pBC2 (a line perpendicular to a line from the basion to the posteroinferior aspect of the C2 body), and clival-axial angle (CXA) were measured on sagittal MRI. The secondary outcome was the need for ≥ 2 CM-related operations. RESULTS Of the 206 patients identified, 20 underwent OCF/VBD and 14 underwent repeat posterior fossa decompression. A C-C2SVA ≥ 5 mm was 100% sensitive and 86% specific for requiring OCF/VBD, with a 12.6% misclassification rate, whereas CXA < 125° was 55% sensitive and 99% specific, and pBC2 ≥ 9 was 20% sensitive and 88% specific. Kaplan-Meier analysis demonstrated that there was a significantly shorter time to second decompression in children with C-C2SVA ≥ 5 mm (p = 0.0039). The mean C-C2SVA was greater (6.13 ± 1.28 vs 3.13 ± 1.95 mm, p < 0.0001), CXA was lower (126° ± 15.4° vs 145° ± 10.7°, p < 0.05), and pBC2 was similar (7.65 ± 1.79 vs 7.02 ± 1.26 mm, p = 0.31) among those who underwent OCF/VBD versus decompression only. The intraclass correlation coefficient for the continuous measurement of C-C2SVA was 0.52; the kappa value was 0.47 for the binary categorization of C-C2SVA ≥ 5 mm. CONCLUSIONS These results validated the C-C2SVA using a large, multicenter, external cohort with 100% sensitivity, 86% specificity, and a 12.6% misclassification rate. A C-C2SVA ≥ 5 mm is highly predictive of the need for OCF/VBD in patients with CM-I. The authors recommend that this measurement be considered among the tools to identify the “high-risk” CM-I phenotype.

OBJECTIVE Odontogenic ventral brainstem compression can be a source of significant morbidity in patients with craniocervical disease. The most common methods for odontoidectomy are the transoral and endoscopic endonasal routes. In this study, the authors investigated the use of an institutional protocol for endoscopic transnasal/transoral odontoidectomy in the pediatric population. METHODS From 2007 to 2017, a multidisciplinary institutional protocol was developed and refined for the evaluation and treatment of pediatric patients requiring odontoidectomy. Preoperative assessment included airway evaluation, a sleep study (if indicated), discussion of possible tonsillectomy/adenoidectomy, and thorough imaging review by the neurosurgery and otolaryngology teams. Further preoperative anesthesia consultation was obtained for difficult airways. Intraoperatively, adenoidectomy was performed at the discretion of otolaryngology. The odontoidectomy was performed as a combined procedure. Primary posterior pharyngeal closure was performed by the otolaryngologist. The postoperative protocol called for immediate extubation, advancement to a soft diet at 24 hours, and no postoperative antibiotics. Outcome variables included time to extubation, operative time, estimated blood loss, hospital length of stay, and postoperative complications. RESULTS A total of 13 patients underwent combined endoscopic transoral/transnasal odontoid resection with at least 3 years of follow-up. All patients had stable to improved neurological function in the postoperative setting. All patients were extubated immediately after the procedure. The average operative length was 201 ± 46 minutes, and the average estimated blood loss was 44.6 ± 40.0 ml. Nine of 13 patients underwent simultaneous tonsillectomy and adenoidectomy. The average hospital length of stay was 6.6 ± 5 days. The first patient in the series required revision surgery for removal of a small residual odontoid. One patient experienced pharyngeal flap dehiscence requiring revision. CONCLUSIONS A protocolized, institutional approach for endoscopic transoral/transnasal odontoidectomy is described. The use of a combined, multidisciplinary approach leads to streamlined patient management and favorable outcomes in this complex patient population.

Anterior brainstem compression from odontoid pathology can occur in patients with craniocervical disorders. Occasionally, odontoid resection is required. In adults, odontoid resection has evolved toward transnasal-only endoscopic techniques. Pediatric patients, however, pose special challenges due to abnormal anatomy and smaller working spaces. A combined transnasal/transoral endoscopic odontoid resection (TN/TO EOR) can overcome this limitation. We present a case series with emphasis on otolaryngologic considerations to airway management, endoscopic approach, and management of complications.A single center, retrospective review of patients aged ≤18 undergoing combined transnasal/transoral endoscopic odontoid resection between 2011 and 2022 is presented. Clinical and surgical variables consisting of diagnosis, intubation approach, other airway procedures performed, symptoms, complications, blood loss, and time to extubation, return to oral feeding, and discharge were recorded.19 patients aged 10.7 ± 4.3 (range: 3-18) were included. Diagnoses included congenital syndrome (n = 6), complex Chiari malformation (n = 11), and congenital syndrome with Chiari (n = 2). Patients commonly required indirect videolaryngoscopy for intubation, with or without fiberoptic endoscopic assistance. Seven underwent adenoidectomy, two underwent adenotonsillectomy, and one required adenoidectomy with midline palatal split and inferior turbinate outfracture. Four patients had undergone prior adenotonsillectomy. Presenting symptoms included extremity weakness (n = 9), dysphagia (n = 8), velopharyngeal insufficiency (n = 4), sleep disturbance (n = 5), and headaches (n = 8). Four patients had complications, including one re-operation for residual odontoid, one flap dehiscence, one cerebrospinal fluid (CSF) leak repaired primarily, and one complicated course including temporary spinal cord injury. Blood loss was 50 ± 43 cc (median 30). Time to extubation was 1.1 ± 2.1 days (median 0; one patient underwent tracheotomy for respiratory failure), time to oral intake was 2.9 ± 3.7 days (median 1), and time to discharge was 7.1 ± 7.5 days (median 4).A combined transnasal/transoral approach can be successfully used in pediatric patients to overcome difficult endoscopic access. Although complications exist, early extubation and return to oral intake occurs in the vast majority of cases. For pediatric TN/TO EOR, the otolaryngologist plays a key role in preoperative assessment, airway management, endoscopic exposure, and complication management.

Primary pituitary abscesses are extraordinarily rare lesions, especially in pediatric populations, with very few cases described in the literature. Here we review this rare condition and discuss its management. We report a case of a previously healthy 12-year-old female patient who presented with vomiting and meningismus. We review the literature on pediatric patients presenting with pituitary abscesses and the described treatments. An operative video demonstrating our surgical technique is provided. Magnetic resonance imaging of the brain revealed a rim-enhancing, diffusion-restricting sellar lesion concerning for abscess. No sinus disease or other structural cause of intracranial infection was identified. An endoscopic transsphenoidal approach was used to visualize the endonasal structures, and microscopic guidance was used for evacuation of the abscess. Purulent material was drained immediately upon entry into the lesion. Tissue cultures grew Cutibacterium acnes. Postoperatively, the patient was hypocortisolemic but otherwise endocrinologically normal. She was discharged four days later on oral hydrocortisone and intravenous ceftriaxone without any neurologic deficit. To the best of our knowledge, this is the second report in the literature of a primary pituitary abscess due to C. acnes infection.

Background Hydrocephalus is managed by surgically implanting flow-diversion technologies such as differential pressure valves and antisiphoning devices; however, such hardware is prone to failure. Extensive research has tested them in flow-controlled settings using saline or de-aerated water, yet little has been done to validate their performance in a setting recreating physiologically relevant parameters, including intracranial pressures, cerebrospinal fluid (CSF) protein content, and body position. Objective To more accurately chart the episodic drainage characteristics of flow-diversion technology. A gravity-driven benchtop model of flow was designed and tested continuously during weeks-long trials. Methods Using a hydrostatic pressure gradient as the sole driving force, interval flow rates of 6 valves were examined in parallel with various fluids. Daily trials in the upright and supine positions were run with fluid output collected from distal catheters placed at alternating heights for extended intervals. Results Significant variability in flow rates was observed, both within specific individual valves across different trials and among multiple valves of the same type. These intervalve and intravalve variabilities were greatest during supine trials and with increased protein. None of the valves showed evidence of overt obstruction during 30 d of exposure to CSF containing 5 g/L protein. Conclusion Day-to-day variability of ball-in-cone differential pressure shunt valves may increase overdrainage risk. Narrow-lumen high-resistance flow control devices as tested here under similar conditions appear to achieve more consistent flow rates, suggesting their use may be advantageous, and did not demonstrate any blockage or trend of decreasing flow over the 3 wk of chronic use.

Brainstem cavernous malformations (CMs) often have high hemorrhage rates and significant posthemorrhage morbidity. The authors present two cases in which magnetic resonance thermography-guided laser interstitial therapy was used for treatment of pontine CMs after recurrent hemorrhage. Both patients showed significant symptomatic improvement and were hemorrhage-free at 12- and 6-month follow-up, respectively. Each had radiographic evidence of lesion involution on serial follow-up imaging. These early results demonstrate this treatment modality may be technically safe; however, larger case numbers and longer follow-up are needed to demonstrate efficacy.

Objectives This current systematic review sought to identify and evaluate all current research-based spine surgery applications of AI/ML in optimizing preoperative patient selection, as well as predicting and managing postoperative outcomes and complications. Methods A comprehensive search of publications was conducted through the EMBASE, Medline, and PubMed databases using relevant keywords to maximize the sensitivity of the search. No limits were placed on level of evidence or timing of the study. Findings were reported according to the PRISMA guidelines Results After application of inclusion and exclusion criteria, 41 studies were included in this review. Bayesian networks had the highest average AUC (.80), and neural networks had the best accuracy (83.0%), sensitivity (81.5%), and specificity (71.8%). Preoperative planning/cost prediction models (.89,82.2%) and discharge/length of stay models (.80,78.0%) each reported significantly higher average AUC and accuracy compared to readmissions/reoperation prediction models (.67,70.2%) ( P < .001, P = .005, respectively). Model performance also significantly varied across postoperative management applications for average AUC and accuracy values ( P < .001, P < .027, respectively). Conclusions Generally, authors of the reviewed studies concluded that AI/ML offers a potentially beneficial tool for providers to optimize patient care and improve cost-efficiency. More specifically, AI/ML models performed best, on average, when optimizing preoperative patient selection and planning and predicting costs, hospital discharge, and length of stay. However, models were not as accurate in predicting postoperative complications, adverse events, and readmissions and reoperations. An understanding of AI/ML-based applications is becoming increasingly important, particularly in spine surgery, as the volume of reported literature, technology accessibility, and clinical applications continue to rapidly expand.

To expand on previously described intraoperative aids by developing consensus-based best practice guidelines to optimize the approach to intraoperative neuromonitoring (IONM) events associated with "high-risk" spinal deformity surgery.Consensus was established among a group of experienced spinal deformity surgeons by way of the Delphi method. Through a series of iterative surveys and a final virtual consensus meeting, participants expressed their agreement (strongly agree, agree, disagree, and strongly disagree) with various items. Consensus was defined as ≥ 80% agreement ("strongly agree" or "agree"). Near-consensus was defined as ≥ 60% but 80%. Equipoise was ≥ 20% but 60%, and consensus to exclude was 20%.15 out of 15 (100%) invited surgeons agreed to participate. Final consensus supported inclusion of 105 items (53 in Response Algorithm, 13 in Ongoing Consideration of Etiology, 31 in Real-Time Data Scenarios, 8 in Patterns of IONM Loss), which were organized into a final set of best practice guidelines.Detailed consensus-based best practice guidelines and aids were successfully created with the intention to help organize and direct the surgical team in exploring and responding to neurological complications during high-risk spinal deformity surgery.Level V.

To establish expert consensus on various parameters that constitute elevated risk during spinal deformity surgery and potential preventative strategies that may minimize the risk of intraoperative neuromonitoring (IONM) events and postoperative neurological deficits.Through a series of surveys and a final virtual consensus meeting, the Delphi method was utilized to establish consensus among a group of expert spinal deformity surgeons. During iterative rounds of voting, participants were asked to express their agreement (strongly agree, agree, disagree, strongly disagree) to include items in a final set of guidelines. Consensus was defined as ≥ 80% agreement among participants. Near-consensus was ≥ 60% but 80% agreement, equipoise was ≥ 20% but 60%, and consensus to exclude was 20%.Fifteen of the 15 (100%) invited expert spinal deformity surgeons agreed to participate. There was consensus to include 22 determinants of high-risk (8 patient factors, 8 curve and spinal cord factors, and 6 surgical factors) and 21 preventative strategies (4 preoperative, 14 intraoperative, and 3 postoperative) in the final set of best practice guidelines.A resource highlighting several salient clinical factors found in high-risk spinal deformity patients as well as strategies to prevent neurological events was successfully created through expert consensus. This is intended to serve as a reference for surgeons and other clinicians involved in the care of spinal deformity patients.Level V.

Intramedullary spinal cord tumors (IMSCTs) are rare neoplasms that have limited treatment options and are associated with high rates of morbidity and mortality. To better understand the genetic basis of these tumors we performed whole exome sequencing on 45 tumors and matched germline DNA, including twenty-nine spinal cord ependymomas and sixteen astrocytomas. Though recurrent somatic mutations in IMSCTs were rare, we identified NF2 mutations in 15.7% of tumors (ependymoma, N = 7; astrocytoma, N = 1), RP1 mutations in 5.9% of tumors (ependymoma, N = 3), and ESX1 mutations in 5.9% of tumors (ependymoma, N = 3). We further identified copy number amplifications in CTU1 in 25% of myxopapillary ependymomas. Given the paucity of somatic driver mutations, we further performed whole-genome sequencing of 12 tumors (ependymoma, N = 9; astrocytoma, N = 3). Overall, we observed that IMSCTs with intracranial histologic counterparts (e.g. glioblastoma) did not harbor the canonical mutations associated with their intracranial counterparts. Our findings suggest that the origin of IMSCTs may be distinct from tumors arising within other compartments of the central nervous system and provides the framework to begin more biologically based therapeutic strategies.

OBJECTIVEVentricular shunt infection remains an issue leading to high patient morbidity and cost, warranting further investigation. The authors sought to create an animal model of shunt infection that could be used to evaluate possible catheter modifications and innovations.METHODSThree dogs underwent bilateral ventricular catheter implantation and inoculation with methicillin-sensitive Staphylococcus aureus (S. aureus). In 2 experimental animals, the catheters were modified with a polymer containing chemical “pockets” loaded with vancomycin. In 1 control animal, the catheters were polymer coated but without antibiotics. Animals were monitored for 9 to 11 days, after which the shunts were explanted. MRI was performed after shunt implantation and prior to catheter harvest. The catheters were sonicated prior to microbiological culture and also evaluated by electron microscopy. The animals’ brains were evaluated for histopathology.RESULTSAll animals underwent successful catheter implantation. The animals developed superficial wound infections, but no neurological deficits. Imaging demonstrated ventriculitis and cerebral edema. Harvested catheters from the control animal demonstrated > 104 colony-forming units (CFUs) of S. aureus. In the first experimental animal, one shunt demonstrated > 104 CFUs of S. aureus, but the other demonstrated no growth. In the second experimental animal, one catheter demonstrated no growth, and the other grew trace S. aureus. Brain histopathology revealed acute inflammation and ventriculitis in all animals, which was more severe in the control.CONCLUSIONSThe authors evaluated an animal model of ventricular shunting and reliably induced features of shunt infection that could be microbiologically quantified. With this model, investigation of pathophysiological and imaging correlates of infection and potentially beneficial shunt catheter modifications is possible.

Background: The advancement of endoscopic techniques in the past decade has improved the surgical management of cerebellopontine angle (CPA) tumors. Endoscope-assisted microsurgery improves the ability to evaluate the extent of resection, achieve safe tumor resection and reduce the risk of surgery-related morbidity. Methods: In this study, we used a cadaveric model to demonstrate a step by step endoscope-assisted microsurgery of the retrosigmoid approach to the lateral posterior fossa. Results: Retrosigmoid craniotomies were performed on four latex-injected cadaver heads (eight CPAs). Microsurgical exposures were performed to identify neurovascular structures in each segment. 0° and 30° rigid endoscope lenses were subsequently introduced into each corridor and views were compared in this manner. The endoscopic images were compared with the standard microscopic views to determine the degree of visualization with each technique. In each case, better visualization was provided by both the 0° and 30° endoscope lenses. Endoscopic views frequently clarified neurovascular relationships in obscured anatomic regions. Conclusion: Endoscope-assisted microsurgery could allow better visualization of various regions of the posterior fossa. Surgical planning for posterior fossa lesions should include consideration of this combined approach.

The purpose of this study was to investigate associations between the various definitions of adding-on identified in the literature and HRQoL at 10 years following posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) to identify a clinically relevant definition.In this retrospective cohort study, patients with Lenke type 1 or 2 AIS with 10-year follow-up after PSF were identified in a multicenter registry. Adding-on was identified independently using 5 published definitions and expert spine surgeons' visual confirmation. Worsening of HRQoL was defined as a preoperative to 10-year postoperative decrease in SRS-22 domain score ≥ the minimal clinically important difference (MCID).One hundred fifty patients were included, and 118 had HRQoL data available for the main analysis. Worsening pain was noted in 49 (41.5%) patients, appearance in 1 (0.8%), and activity in 70 (59.3%) from preoperative to postoperative. 4 of the 5 definitions of adding-on demonstrated no evidence of association with worsening HRQoL. By Cho's definition (either 1. ≥ 5° increase in Cobb angle AND increase in number of vertebrae in curve distally, or 2. ≥ 5° increase in angulation of 1st disc below LIV), adding-on (27 patients, 22.9%) was associated with an increased risk of worsening pain (59.3% vs. 36.3%, p = 0.033) and activity (77.8% vs. 53.8%, p = 0.026). There was no evidence of association with changes in appearance. Risk of worsening pain and activity was increased by 1.75 and 1.51 times (p = 0.023 and p = 0.002), respectively.Cho's definition of adding-on was associated with worsening HRQoL at 10 years following PSF for Lenke type 1 and 2 AIS. Consistent use of this definition will allow us to compare studies and obtain meaningful information in this population.Level II.

Efficiency and patient safety are top priorities in any surgical operation. One effective way to achieve these objectives is automating many of the logistical and routine tasks that occur in the operating room. Inspired by smart assistant technology already commonplace in the consumer sector, we engineered the Smart Hospital Assistant (SHA), a smart, voice-controlled virtual assistant that handles natural speech recognition while executing a plurality of functions to aid surgery. Simulated surgeries showed that the SHA reduced operating time, optimized surgical staff resources, and reduced the number of major touch points that can lead to surgical site infections. The SHA not only shows its potential in the operating room, but also in other healthcare environments that may benefit from having virtual smart assistant technology.

Primary pituitary abscesses are extraordinarily rare lesions, especially in pediatric populations, with very few cases described in the literature. Here we review this rare condition and discuss its management.We report a case of a previously healthy 12-year-old female patient who presented with vomiting and meningismus. We review the literature on pediatric patients presenting with pituitary abscesses and the described treatments. An operative video demonstrating our surgical technique is provided.Magnetic resonance imaging of the brain revealed a rim-enhancing, diffusion-restricting sellar lesion concerning for abscess. No sinus disease or other structural cause of intracranial infection was identified. An endoscopic transsphenoidal approach was used to visualize the endonasal structures, and microscopic guidance was used for evacuation of the abscess. Purulent material was drained immediately upon entry into the lesion. Tissue cultures grew Cutibacterium acnes. Postoperatively, the patient was hypocortisolemic but otherwise endocrinologically normal. She was discharged four days later on oral hydrocortisone and intravenous ceftriaxone without any neurologic deficit.To the best of our knowledge, this is the second report in the literature of a primary pituitary abscess due to C. acnes infection.

IntroductionTo improve patient outcomes (eg, reducing blood loss and infection), practitioners have gravitated toward noninvasive and minimally invasive surgeries (MIS), which demand specialized toolkits. Focused ultrasound, for example, facilitates thermal ablation from a distance, thereby reducing injury to surrounding tissue. Focused ultrasound can often be performed noninvasively; however, it is more difficult to carry out in neuro-oncological tumors, as ultrasound is dramatically attenuated while propagating through the skull. This shortcoming has prompted exploration of MIS options for intracranial placement of focused ultrasound probes, such as within the BrainPath™ (NICO Corporation, Indianapolis, IN). Herein, we present the design, development, and in vitro testing of an image-guided, focused ultrasound prototype designed for use in MIS procedures. This probe can ablate neuro-oncological lesions despite its small size.Materials & MethodsPreliminary prototypes were iteratively designed, built, and tested. The final prototype consisted of three 8-mm-diameter therapeutic elements guided by an imaging probe. Probe functionality was validated on a series of tissue-mimicking phantoms.ResultsLesions were created in tissue-mimicking phantoms with average dimensions of 2.5×1.2×6.5mm and 3.4×3.25×9.36mm after 10- and 30-second sonification, respectively. 30s sonification with 118W power at 50% duty cycle generated a peak temperature of 68°C. Each ablation was visualized in real time by the built-in imaging probe.ConclusionWe developed and validated an ultrasound-guided focused ultrasound probe for use in MIS procedures. The dimensional constraints of the prototype were designed to reflect those of BrainPath trocars, which are MIS tools used to create atraumatic access to deep-seated brain pathologies.HIGHLIGHTSAn ultrasound-guided, focused ultrasound prototype was developed and validatedThe therapeutic transducer (1.5MHz) consisted of three 8-mm circular elementsElements were placed on a 9×32mm curved rectangular aperture: 45mm radius curvatureFunctionality was examined on tissue-mimicking phantoms2.5×1.2×6.5mm and 3.4×3.25×9.36mm lesions were seen for 10 and 30s sonification

Wasted time in the operating room results in higher operating costs and greater post-operative complications. One effective way to reduce operation time is automating basic processes that occur during surgery. Given the rise of smart-home devices, implementation of virtual assistants became a feasible solution in many medical settings. With a consumer smart-home device and off-the-shelf components, we engineered a voice-controlled smart surgical bed that adjusts the bed configuration via a voice input. The resulting device is expected to optimize human resources and reduce surgical site infection by eliminating the need of a traditional touch control mechanism. Future work is needed to develop its proprietary hardware and software, and continuous collaboration with medical personnel to bring this device into market.

Background and Study Aims/Objective Deep-seated high-grade gliomas (HGGs) represent a unique surgical challenge because they reside deep to critical cortical and subcortical structures and infiltrate functional areas of the brain. Therefore, accessing and resecting these tumors can often be challenging and associated with significant morbidity. We describe the use of minimally invasive approaches to access deep-seated HGGs to achieve extensive resections while minimizing surgical morbidity. Materials and Methods All patients who underwent resection of a deep-seated intraparenchymal HGG with the use of a tubular retractor with exoscopic visualization from January 2016 to May 2017 were identified prospectively at a single institution. Variables evaluated included tumor location, pre- and postoperative neurologic function, extent of resection, and length of hospital stay. Results Overall, 14 patients underwent resection of an HGG (11 glioblastomas, 3 anaplastic astrocytomas) with a tubular retractor under exoscopic visualization. Seven tumors (50%) involved the thalamus, three (21%) the motor corticospinal tract, two (14%) the inferior frontal occipital fasciculus, one (7%) each the basal ganglia and optic pathway. The median preoperative Karnofsky Performance Score (KPS) was 70 (interquartile range: 55–80), where the major presenting symptom was motor weakness in seven (50%). The average plus or minus the standard error of the mean percentage resection was 97.0 ± 1.2%. The median hospital stay was 4 days (range: 2–7). At 1 month postoperatively, median postoperative KPS (within 30 days) was 87 (range: 77–90), where eight (57%) were improved, five (36%) were stable, and one (7%) was worse postoperatively. Conclusions Deep-seated HGGs can be accessed, visualized, and resected using tubular retractors and exoscopic visualization with minimal morbidity.

Background Surgical treatment of severe cervicothoracic kyphotic deformity may require the use of 3-column osteotomies such as the pedicle subtraction osteotomy and vertebral column resection (VCR), or VCR with anterior longitudinal ligament resection. Such procedures are extensive and are associated with high intra- and perioperative morbidity, in part, due to the need for risky reduction maneuvers. Objective To describe a novel technique utilizing a laterally placed articulating hinge to facilitate kyphotic deformity correction of the cervicothoracic spine. Methods A patient with severe chin-on-chest deformity of the cervicothoracic spine presented for evaluation and a 2-stage VCR with anterior longitudinal ligament resection was planned. To reduce the risk of intraoperative neurological injury and for increased control during reduction maneuvers, lateral instrumentation was placed through the chest wall resection above and below the level of VCR, which was adjoined with an articulating hinge rod apparatus. Results Satisfactory reduction of the kyphosis was achieved utilizing the hinge rod apparatus for controlled deformity correction. The patient remained neurologically intact following this procedure with improvement in their spinal alignment. Conclusion We present a novel technique utilizing a lateral hinge rod apparatus for efficient, controlled correction of severe kyphotic deformity.

Background In children, high rates of occipitocervical (OC) fusion have been demonstrated with the use of rigid instrumentation in combination with harvested autograft, with or without bone morphogenetic protein (BMP). Historically, the use of allograft materials demonstrated inferior OC fusion outcomes compared with autograft. However, autograft harvest harbors an increased risk of patient morbidity, and the use of BMP is costly and controversial in children. Thus, there remains a need for safer, less costly, yet efficacious techniques for OC fusion in the pediatric population. Methods We retrospectively reviewed the charts of patients younger than 21 years of age who underwent OC fusion with structural allograft placement at our institution from 2010 to 2015. Data collected included age, sex, follow-up duration, fusion outcomes, and postoperative complications. Results A total of 19 patients (8 female and 11 male) underwent OC fusion with our surgical technique. Mean age was 8.5 ± 4.3 years. Radiographic follow up data were available for 18 of 19 patients. One patient was lost to clinical follow up but had radiographic confirmation of fusion. Thus, 18 of 18 (100%) of patients with radiographic follow-up achieved successful arthrodesis as determined by computed tomography. Median duration to documented fusion was 4.5 months. Clinical follow-up was available for 17 of 19 patients and was on average 18.8 ± 13.5 months. One patient required reoperation for graft fracture 8 months after radiographic confirmation of successful fusion. There were no vertebral artery injuries or other postoperative complications. Conclusions We demonstrate a modified technique for OC fusion in children with unique structural allograft shaping and affixation, leading to excellent fusion outcomes at follow up. This technique obviates the need for autograft harvest or BMP, and may decrease postoperative morbidity.

Background A subset of patients with skull base cerebrospinal fluid (CSF) leaks are found to have elevated intracranial pressure (ICP). In these patients, elevated ICP is thought to contribute to both the pathophysiology of the leak and postoperative leak recurrences. Current strategies for postoperative ICP control include medical therapy and shunting procedures. The aim of this study is to report the use of venous sinus stenting (VSS) in the management of patients with skull base CSF leaks caused by elevated ICP. Methods We performed a retrospective investigation of 2 patients who underwent surgical repair of skull base CSF leaks and were found to have elevated ICP associated with venous sinus stenosis and subsequently treated with VSS. Results Two patients underwent successful surgical repair of skull base CSF leaks with perioperative ICP monitoring via temporary lumbar catheters. Postoperative CSF pressure measurement demonstrated elevated ICP. Both patients were found to have venous sinus stenosis on further workup and subsequently underwent VSS for treatment of intracranial hypertension. Both patients had improvement in their symptoms with no evidence of recurrent CSF leak at follow-up. Conclusions Patients with skull base CSF leaks of unknown etiology should undergo CSF pressure monitoring postoperatively and, if found to be elevated, be treated for intracranial hypertension. In patients unresponsive to, or intolerant of, medical therapy, VSS can provide an alternative option to medical and surgical shunting procedures for treatment of intracranial hypertension in patients with skull base CSF leaks and venous sinus stenosis.

Cranial vault remodeling surgery for craniosynostosis carries the potential risk of dural venous sinus injury given the extensive bony exposure. Identification of the dural venous sinuses can be challenging in patients with craniosynostosis given the lack of accurate surface-localizing landmarks. Computer-aided design and manufacturing (CAD/CAM) has allowed surgeons to pre-operatively plan these complex procedures in an effort to increase reconstructive efficiency. An added benefit of this technology is the ability to intraoperatively map the dural venous sinuses based on pre-operative imaging. We utilized CAD/CAM technology to intraoperatively map the dural venous sinuses for patients undergoing reconstructive surgery for craniosynostosis in an effort to prevent sinus injury, increase operative efficiency, and enhance patient safety. Here, we describe our experience utilizing this intraoperative technology in pediatric patients with craniosynostosis. We retrospectively reviewed the charts of children undergoing reconstructive surgery for craniosynostosis using CAD/CAM surgical planning guides at our institution between 2012 and 2016. Data collected included the following: age, gender, type of craniosynostosis, estimated blood loss, sagittal sinus deviation from the sagittal suture, peri-operative outcomes, and hospital length of stay. Thirty-two patients underwent reconstructive cranial surgery for craniosynostosis, with a median age of 11 months (range, 7–160). Types of synostosis included metopic (6), unicoronal (6), sagittal (15), lambdoid (1), and multiple suture (4). Sagittal sinus deviation from the sagittal suture was maximal in unicoronal synostosis patients (10.2 ± 0.9 mm). All patients tolerated surgery well, and there were no occurrences of sagittal sinus, transverse sinus, or torcular injury. The use of CAD/CAM technology allows for accurate intraoperative dural venous sinus localization during reconstructive surgery for craniosynostosis and enhances operative efficiency and surgeon confidence while minimizing the risk of patient morbidity.

OBJECTIVEThe authors’ objective was to better understand the anatomical load-bearing relationship between the atlantooccipital joint and the upper cervical spine and its influence on the clinical behavior of patients with Chiari malformation type I (CM-I) and craniocervical pathology.METHODSIn a single-center prospective study of patients younger than 18 years with CM-I from 2015 through 2017 (mean age 9.91 years), the authors measured the occipital condyle–C2 sagittal vertebral alignment (C–C2SVA; defined as the position of a plumb line from the midpoint of the occiput (C0)–C1 joint relative to the posterior aspect of the C2–3 disc space), the pB–C2 (a line perpendicular to a line from the basion to the posteroinferior aspect of the C2 body on sagittal MRI), and the CXA (clivoaxial angle). Control data from 30 patients without CM-I (mean age 8.97 years) were used for comparison. The primary outcome was the need for anterior odontoid resection and/or occipitocervical fusion with or without odontoid reduction. The secondary outcome was the need for two or more Chiari-related operations.RESULTSOf the 60 consecutive patients with CM-I identified, 7 underwent anterior odontoid resection or occipitocervical fusion and 10 underwent ≥ 2 decompressive procedures. The mean C–C2SVA was greater in the overall CM-I group versus controls (3.68 vs 0.13 mm, p < 0.0001), as was the pB–C2 (7.7 vs 6.4 mm, p = 0.0092); the CXA was smaller (136° vs 148°, p < 0.0001). A C–C2SVA ≥ 5 mm was found in 35% of CM-I children and 3.3% of controls (p = 0.0006). The sensitivities and specificities for requiring ventral decompression/occipitocervical fusion were 100% and 74%, respectively, for C–C2SVA ≥ 5 mm; 71% and 94%, respectively, for CXA < 125°; and 71% and 75%, respectively, for pB–C2 ≥ 9 mm. The sensitivities and specificities for the need for ≥ 2 decompressive procedures were 60% and 70%, respectively, for C–C2SVA ≥ 5 mm; 50% and 94%, respectively, for CXA < 125°; and 60% and 76%, respectively, for pB–C2 ≥ 9 mm. The log-rank test demonstrated significant differences between C–C2SVA groups (p = 0.0007) for the primary outcome. A kappa value of 0.73 for C–C2SVA between raters indicated substantial agreement.CONCLUSIONSA novel screening measurement for craniocervical bony relationships, the C–C2SVA, is described. A significant difference in C–C2SVA between CM-I patients and controls was found. A C–C2SVA ≥ 5 mm is highly predictive of the need for occipitocervical fusion/ventral decompression in patients with CM-I. Further validation of this screening measurement is needed.

Neurosurgery typically requires craniectomy and meticulous dissection to achieve sufficient exposure for subsequent surgical intervention. This highly invasive process requires hours of operating time, long recovery periods and leaves patients with visible surgical scars. Non-invasive high-intensity focused ultrasound (HIFU) has shown some promise yet remains challenged by the attenuation of ultrasonic waves while passing through the skull. Consequently, the clinical impact of this technology remains limited, particularly in the treatment of neuro-oncology. In order to compensate for acoustic attenuation, excessive use of power for HIFU devices has been investigated, although it is undesirable from a regulatory and patient safety standpoint. Here, we report the design and development of a novel HIFU device prototype for neurologic lesion ablation. This device concept is envisioned to access the ventricular space via a minimally invasive ventriculostomy, allowing ultrasound to reach targets deep in the brain, while eliminating the need for high power to penetrate the skull.

OBJECTIVE Augmented reality (AR) has the potential to improve the accuracy and efficiency of instrumentation placement in spinal fusion surgery, increasing patient safety and outcomes, optimizing ergonomics in the surgical suite, and ultimately lowering procedural costs. The authors sought to describe the use of a commercial prototype Spine AR platform (SpineAR) that provides a commercial AR head-mounted display (ARHMD) user interface for navigation-guided spine surgery incorporating real-time navigation images from intraoperative imaging with a 3D-reconstructed model in the surgeon's field of view, and to assess screw placement accuracy via this method. METHODS Pedicle screw placement accuracy was assessed and compared with literature-reported data of the freehand (FH) technique. Accuracy with SpineAR was also compared between participants of varying spine surgical experience. Eleven operators without prior experience with AR-assisted pedicle screw placement took part in the study: 5 attending neurosurgeons and 6 trainees (1 neurosurgical fellow, 1 senior orthopedic resident, 3 neurosurgical residents, and 1 medical student). Commercially available 3D-printed lumbar spine models were utilized as surrogates of human anatomy. Among the operators, a total of 192 screws were instrumented bilaterally from L2–5 using SpineAR in 24 lumbar spine models. All but one trainee also inserted 8 screws using the FH method. In addition to accuracy scoring using the Gertzbein-Robbins grading scale, axial trajectory was assessed, and user feedback on experience with SpineAR was collected. RESULTS Based on the Gertzbein-Robbins grading scale, the overall screw placement accuracy using SpineAR among all users was 98.4% (192 screws). Accuracy for attendings and trainees was 99.1% (112 screws) and 97.5% (80 screws), respectively. Accuracy rates were higher compared with literature-reported lumbar screw placement accuracy using FH for attendings (99.1% vs 94.32%; p = 0.0212) and all users (98.4% vs 94.32%; p = 0.0099). The percentage of total inserted screws with a minimum of 5° medial angulation was 100%. No differences were observed between attendings and trainees or between the two methods. User feedback on SpineAR was generally positive. CONCLUSIONS Screw placement was feasible and accurate using SpineAR, an ARHMD platform with real-time navigation guidance that provided a favorable surgeon-user experience.

Brainstem gliomas comprise 10-20% of all pediatric central nervous system (CNS) tumors and diffuse intrinsic pontine gliomas (DIPGs) account for the majority of these lesions. DIPG is a rapidly progressive disease with almost universally fatal outcomes and a median survival less than 12 months. Current standard-of-care treatment for DIPG includes radiation therapy, but its long-term survival effects are still under debate. Clinical trials investigating the efficacy of systemic administration of various therapeutic agents have been associated with disappointing outcomes. Recent efforts have focused on improvements in chemotherapeutic agents employed and in methods of localized and targeted drug delivery. This review provides an update on current preclinical and clinical studies investigating treatment options for brainstem gliomas.

Introduction To improve patient outcomes (eg, reducing blood loss and infection), practitioners have gravitated toward noninvasive and minimally invasive surgeries (MIS), which demand specialized toolkits. Focused ultrasound, for example, facilitates thermal ablation from a distance, thereby reducing injury to surrounding tissue. Focused ultrasound can often be performed noninvasively; however, it is more difficult to carry out in neuro-oncological tumors, as ultrasound is dramatically attenuated while propagating through the skull. This shortcoming has prompted exploration of MIS options for intracranial placement of focused ultrasound probes, such as within the BrainPath™ (NICO Corporation, Indianapolis, IN). Herein, we present the design, development, and in vitro testing of an image-guided, focused ultrasound prototype designed for use in MIS procedures. This probe can ablate neuro-oncological lesions despite its small size. Materials & Methods Preliminary prototypes were iteratively designed, built, and tested. The final prototype consisted of three 8-mm-diameter therapeutic elements guided by an imaging probe. Probe functionality was validated on a series of tissue-mimicking phantoms. Results Lesions were created in tissue-mimicking phantoms with average dimensions of 2.5 × 1.2 × 6.5 mm and 3.4 × 3.25 × 9.36 mm after 10- and 30-second sonification, respectively. 30 s sonification with 118 W power at 50% duty cycle generated a peak temperature of 68 °C. Each ablation was visualized in real time by the built-in imaging probe. Conclusion We developed and validated an ultrasound-guided focused ultrasound probe for use in MIS procedures. The dimensional constraints of the prototype were designed to reflect those of BrainPath trocars, which are MIS tools used to create atraumatic access to deep-seated brain pathologies.

This case involved a 6-year-old boy with Down syndrome, left C1 lateral mass hypertrophy, C1–2 rotatory subluxation, and spinal cord compression. He presented after falling down some stairs at his home. Torticollis, dysphagia, and speech delay were noted on examination. Vascular imaging showed impingement on the left vertebral artery by the anomalous C1 lateral mass. Through a posterior approach, the hypertrophic C1 lateral mass was resected, and an occiput–C2 fusion was performed. Postoperatively, his torticollis and brainstem symptoms were resolved.The video can be found here: https://youtu.be/1U0GLdw6c70

Intraventricular tumors are rare and comprise a variety of histological entities. They may be found throughout the ventricular system, which is comprised broadly of the lateral ventricles, third ventricle, cerebral aqueduct, and fourth ventricle. Given the widespread and varied topography of the ventricular system, its presence in the supratentorial and infratentorial compartments, as well as its diverse cellular constituency, it is not surprising that tumors arising from this complex are quite heterogeneous in location, clinical consequence, and biological behavior. The diverse nature of intraventricular tumors makes their classification more challenging than the standard intra-axial versus extra-axial categorization applied to many central nervous system lesions.

Introduction: Posterior fossa decompression (PFD) is the treatment of choice for symptomatic Chiari I malformation (CIM) patients; however, controversy exists regarding specific surgical techniques needed to accomplish this goal. While a variety of methods have been described, an ongoing central debate exists regarding the need for duraplasty (PFDD) in addition to bony posterior fossa decompression for CIM.Materials and Methods: We performed a systematic review, including articles from 2000 to 2018, following the PRISMA statement, to study outcomes of CIM patients who underwent PFD with or without duraplasty. Information on demographic data, the type of procedure, study design, the presence of a syrinx, follow-up duration, and various clinical outcomes were collected. We conducted a meta-analysis to assess outcomes, such as clinical improvement, syringomyelia resolution, complications and reoperation rate.Results: Nine studies (including 212 and 315 patients undergoing PFD and PFDD, respectively) were included in the meta-analysis. Regarding clinical outcomes, PFDD showed higher clinical and syrinx improvement rates compared to PFD (p>0.05

Background Pedicle screws (PSs) are routinely used for stabilization to enhance fusion in a variety of spinal diseases. Although the accuracy of different PS placement methods has been previously reported, most of these studies have been limited to 1 or 2 techniques. The purpose was to determine the current accuracy of PS placement among 4 modalities of PS insertion (freehand [FH], fluoroscopy-assisted [FA], computed tomography navigation–guided [CTNav], and robot-assisted [RA]) and analyze variables associated with screw misplacement. Methods A systematic review was performed of peer-reviewed articles reporting PS accuracy of 1 technique from January 1990 to June 2018. Accuracy of PS placement, PS insertion technique, and pedicle breach (PB) data were collected. A meta-analysis was performed to estimate the overall pooled (OP) rates of PS accuracy as a primary outcome, stratified by screw insertion techniques. Potential determinants were analyzed via meta-regression analyses. Results Seventy-eight studies with 7858 patients, 51,161 PSs, and 3614 cortical PBs were included. CTNav showed the highest PS placement accuracy compared with other techniques: OP accuracy rates were 95.5%, 93.1%, 91.5%, and 90.5%, via CTNav, FH, FA, and RA techniques, respectively. RA and CTNav were associated with the highest PS accuracy in the thoracic spine, compared with FH. Conclusions The OP data show that CTNav has the highest PS accuracy rates. Thoracic PSs were associated with lower accuracy rates; however, RA showed fewer breaches in the thoracic spine compared with FH and FA. Given the heterogeneity among studies, further standardized and comparative investigations are required to confirm our findings.

Intramedullary spinal cord tumors (IMSCTs) are rare central nervous system lesions with heterogeneous patient presentations that often include pain and neurological dysfunction. In adults, ependymomas are the most common IMSCT, and in children, pilocytic astrocytomas are most frequent. The mainstay of treatment for IMSCTs is maximal safe surgical resection. Careful surgical planning and technique are critical for successful outcomes. This includes the choice of appropriate spinal levels for surgery, interpretation of real-time intraoperative neurophysiological monitoring, maintenance of meticulous hemostasis, careful microsurgical dissection, and watertight dural closure. Close attention to intraoperative neuromonitoring can allow for maximal tumor resection without permanent postoperative deficits in experienced hands. Transient postoperative deficits often improve with time. Aggressive resection often leads to excellent outcomes and should be attempted regardless of patient age.

Recent work has yielded a method for automatic labeling of vertebrae in intraoperative radiographs as an assistant to manual level counting. The method, called LevelCheck, previously demonstrated promise in phantom studies and retrospective studies. This study aims to: (#1) Analyze the effect of LevelCheck on accuracy and confidence of localization in two modes: (a) Independent Check (labels displayed after the surgeon’s decision) and (b) Active Assistant (labels presented before the surgeon’s decision). (#2) Assess the feasibility and utility of LevelCheck in the operating room. Two studies were conducted: a laboratory study investigating these two workflow implementations in a simulated operating environment with 5 surgeons, reviewing 62 cases selected from a dataset of radiographs exhibiting a challenge to vertebral localization; and a clinical study involving 20 patients undergoing spine surgery. In Study #1, the median localization error without assistance was 30.4% (IQR = 5.2%) due to the challenging nature of the cases. LevelCheck reduced the median error to 2.4% for both the Independent Check and Active Assistant modes (p

This chapter examines the preoperative and operative considerations that should influence the management of neoplasms affecting the spinal column. The three broad categories of spinal tumors (extradural, intradural extramedullary, and intramedullary) are described. The patient and tumor subtype factors that can create challenges in the treatment of these patients are discussed. This chapter also covers scoring systems that can help physicians in counseling and management of patients diagnosed with tumors affecting the spinal column. A multidisciplinary approach should be used to ensure that patients with spinal neoplasms receive holistic, comprehensive care.

OBJECTIVEInfants with severe hydrocephalus and extreme macrocephaly typically undergo CSF diversion early in life, which can result in significant cranial deformity due to CSF overdrainage. In this scenario, overlap of the cranial plates can precede the development of secondary synostosis and/or severe, permanent cranial deformity. As a result, extensive cranial vault remodeling is sometimes undertaken later in life, which is often challenging and has been associated with mortality and a high morbidity rate. The authors have previously described a technique for early postnatal cranial vault reduction and fixation (CVRF), in which the calvarial bones are stabilized using absorbable fixation plates in the neonatal period, in an attempt to facilitate patient positioning, simplify hydrocephalus management, and improve cosmesis. Here, the authors describe their institutional experience managing patients with extreme neonatal hydrocephalus with CSF diversion, with and without CVRF, over the past 12 years.METHODSThe authors retrospectively reviewed the charts of infants with extreme hydrocephalus (head circumference > 49 cm) treated at their children’s hospital with ventriculoperitoneal shunting, with or without CVRF, between 2005 and 2017. Data collected included age, sex, etiology of hydrocephalus, type of CVRF performed (anterior, posterior, or combined), follow-up duration, orbitofrontal circumference, craniometric measurements, intraoperative blood loss, operative duration, and postoperative complications. Developmental data were collected using the third edition of the Ages and Stages Questionnaire. Photographic imaging was used to demonstrate esthetic outcomes, and family questionnaires were used to evaluate satisfaction with the esthetic outcome.RESULTSEleven patients with extreme neonatal hydrocephalus underwent CSF shunting; 5 underwent shunting alone and 6 patients underwent shunting and CVRF. For patients who underwent shunting and CVRF, the median age at CVRF was 6 days and the median interval between shunt placement and CVRF was 2.5 days. The mean extent of calvarial vault volume reduction was 44.5% (± 3.9%). The mean duration of the CVRF procedure was 108 minutes, and 5 of 6 patients required intraoperative transfusion. Of the 5 patients who underwent shunting alone, 3 developed severe cranial deformities. Of 6 patients who underwent shunting and CVRF, 1 had a poor cosmetic outcome. In the shunting-alone group, 2 patients died and 1 required extensive cranial vault correction at 10 years of age. One patient in the shunting and CVRF group also died.CONCLUSIONSCVRF in combination with CSF shunting in the neonatal period can simplify the treatment of the rare case of severe hydrocephalic macrocephaly and leads to cosmetic outcomes that are considered good by their families.

OBJECTIVEMany infants with sagittal craniosynostosis undergo effective surgical correction with endoscopic strip craniectomy (ESC) and postoperative helmet therapy (PHT). While PHT is essential to achieving optimal cosmesis following ESC, there has been little comprehensive analysis of the ideal PHT duration needed to attain this goal.METHODSThe authors retrospectively reviewed the charts of infants undergoing ESC and PHT for sagittal synostosis at our institution between 2008 and 2015. Data collected included age at surgery, follow-up duration, and PHT duration. Cephalic index (CI) was evaluated preoperatively (CIpre), at its peak level (CImax), at termination of helmet therapy (CIoff), and at last follow-up (CIfinal). A multivariate regression analysis was performed to determine factors influencing CIfinal.RESULTSThirty-one patients (27 male, 4 female) were treated in the studied time period. The median age at surgery was 2.7 months (range 1.6 to 3.2) and the median duration of PHT was 10.4 months (range 8.4 to 14.4). The mean CImax was 0.83 (SD 0.01), which was attained an average of 8.4 months (SD 1.2) following PHT initiation. At last follow-up, there was an average retraction of CIfinal among all patients to 0.78 (SD 0.01). Longer helmet duration after achieving CImax did not correlate with higher CIfinal values. While CImax was a significant predictor of CIfinal, neither age at surgery nor CIpre were found to be predictive of final outcome.CONCLUSIONSPatients undergoing ESC and PHT for sagittal synostosis reach a peak CI around 7 to 9 months after surgery. PHT beyond CImax does not improve final anthropometric outcomes. CIfinal is significantly dependent on CImax, but not on age, nor CIpre. These results imply that helmet removal at CImax may be appropriate for ESC patients, while helmeting beyond the peak does not change final outcome.