Search

Background: Neurenteric cysts are rare congenital lesions from heterotopic endodermal tissue, often presenting with radiculopathy or myelopathy in young adults. Gross-total resection is curative; however, the surgical approach remains widely debated for cervicothoracic cases. While the posterior approach is common, the anterior approach has had success in adults. The authors present the first pediatric case of anterior corpectomy with gross-total resection of a cervicothoracic neurenteric cyst alongside an extensive literature review., Observations: A 10-year-old male, who had undergone a previous cyst resection via a posterior approach at an outside institution, presented with back pain, paraplegia, and urinary incontinence. Magnetic resonance imaging of the spine revealed a ventral hyperintense cyst at C7-T1 consistent with a neurenteric cyst. An anterior approach involving C7 and T1 corpectomies was performed, including intradural exploration leading to complete cyst resection. This was followed by the placement of an expandable cage and anterior and posterior fixation with arthrodesis for stabilization. The patient's symptoms completely resolved after surgery, and there has been no recurrence., Lessons: The anterior approach is a viable option for cervicothoracic neurenteric cyst resection in the pediatric population and can aid in gross-total resection by providing better lesion visualization. More studies are needed on long-term outcomes of the anterior approach in pediatric populations. https://thejns.org/doi/10.3171/CASE24120.

Scoliosis is a common complication of neuromuscular disorders. These patients are frequently recalcitrant to nonoperative treatment. When treated surgically, they have the highest risk of complications of all forms of scoliosis. While recent studies have shown an improvement in the rate of complications, they still remain high ranging from 6.3 to 75% depending upon the underlying etiology and the treatment center (Mohamad et al. in J Pediatr Orthop 27:392-397, 2007; McElroy et al. in Spine, 2012; Toll et al. in J Neurosurg Pediatr 22:207-213, 2018; Cognetti et al. in Neurosurg Focus 43:E10, 2017). For those patients who are able to recover from the perioperative period without major complications, several recent studies have shown decreased long-term mortality and improved health-related quality of life in neuromuscular patients who have undergone spine fusion (Bohtz et al. in J Pediatr Orthop 31:668-673, 2011; Ahonen et al. in Neurology 101:e1787-e1792, 2023; Jain et al. in JBJS 98:1821-1828, 2016). It is critically important to optimize patients preoperatively to minimize the risk of post-operative complications and maximize long-term outcomes. In order to do so, one must familiarize themselves with the common complications and their treatment. The most common complications are pulmonary in nature. With reported rates as high as 23-29%, pre-operative optimization should be employed for these patients to minimize the risk of post-operative complications (Sharma et al. in Eur Spine J 22:1230-1249, 2013; Rumalla et al. in J Neurosurg Spine 25:500-508, 2016). The next most common cause of complications are implant related, with 13-23% of patients experiencing an implant-related complication that may require a second procedure (Toll et al. in J Neurosurg Pediatr 22:207-213, 2018; Sharma et al. in Eur Spine J 22:1230-1249, 2013) Therefore optimization of bone quality prior to surgical intervention is important to help minimize the risk of instrumentation failure. Optimization of muscle tone and spasticity may help to decrease the risk of instrumentation complications, but may also contribute to the progression of scoliosis. While only 3% of patients have neurologic complication, significant equipoise remains regarding whether or not patients should undergo prophylactic detethering procedures to minimize those risks (Sharma et al. in Eur Spine J 22:1230-1249, 2013). Although only 1.8% of complications are classified as cardiac related, they can be among the most devastating (Rumalla et al. in J Neurosurg Spine 25:500-508, 2016). Simply understanding the underlying etiology and the potential risks associated with each condition (i.e., conduction abnormalities in a patient with Rett syndrome or cardiomyopathies patients with muscular dystrophy) can be lifesaving. The following article is a summation of the half day course on neuromuscular scoliosis from the 58th annual SRS annual meeting, summarizing the recommendations from some of the world's experts on medical considerations in surgical treatment of neuromuscular scoliosis., (© 2024. The Author(s), under exclusive licence to Scoliosis Research Society.)

Objective: Selective dorsal rhizotomy (SDR) is a neurosurgical procedure to reduce spasticity in children with cerebral palsy and spastic diplegia. The authors developed a procedure called focal SDR for children with spasticity predominantly in the L5 or S1 motor distribution, which can be combined with orthopedic correction of fixed soft-tissue or bony deformity. The authors describe in detail the technique of minimally invasive focal SDR and propose selection criteria., Methods: The authors conducted a retrospective study of patients who underwent focal SDR at their institution and underwent baseline and 1-year postoperative 3D gait analysis. Modified Ashworth scale (MAS) and Gait Deviation Index (GDI) scores were the primary outcome measures., Results: Ten patients met the study criteria, all with an underlying diagnosis of cerebral palsy. All underwent focal SDR at the unilateral or bilateral S1 level, and 4 additionally underwent focal SDR at the L5 level unilaterally or bilaterally. All but 1 patient underwent concurrent orthopedic surgery. The improvement in spasticity of the plantar flexors, as measured by the MAS score, was 2.2 (p < 0.001). In the patients who underwent L5 focal SDR, there was an improvement in the hamstring MAS score of 1.4 (p = 0.004). The mean improvement in the GDI score following focal SDR was 11 (range -6 to 29, p < 0.001)., Conclusions: Focally impairing spasticity in the gastrocsoleus complex and/or hamstrings muscle group in the setting of less functionally impactful proximal tone is extremely common in cerebral palsy. The novel technique of focal SDR, combined with orthopedic intervention, improves spasticity scores and overall gait mechanics. Further investigation is warranted to define the ideal candidacy and outcomes.

Objective: The objective was to describe the indications, technique, and initial outcomes of autologous rib graft with recombinant human bone morphogenetic protein (rhBMP) in pediatric patients undergoing posterior cervical fusion., Methods: A retrospective study was performed of all pediatric patients who underwent autologous rib grafting with extra-small rhBMP-2 for posterior craniocervical or cervical arthrodesis at a single institution between May 2020 and July 2023. Patients with less than 3 months of postoperative follow-up and no postoperative CT data were excluded. Primary outcomes included presence of fusion on CT, 30-day perioperative complications, and rib harvest complications., Results: Twenty-eight sequential patients met inclusion criteria. Thirteen were male, 15 were female, and the average age was 9 years. There were no surgical site infections or instances of postoperative seroma or unplanned return to the operating room. All patients had solid fusion on postoperative CT at 3 months. The average follow-up was 14.5 months, with a range of 4 months to 3 years. There were no complications associated with the rib harvest, including no instances of harvest site pain, and all patient incisions healed well., Conclusions: The authors' preliminary results demonstrate that autologous rib graft with extra-small rhBMP-2 is an effective strategy to achieve a high rate of fusion in pediatric patients undergoing posterior instrumented craniocervical or cervical fusion. In this series, the authors found an acceptable safety profile, without seroma, surgical site infection, unplanned return to the operating room, or rib harvest complications.

Purpose: Hirayama disease, a rare cervical myelopathy in children and young adults, leads to progressive upper limb weakness and muscle loss. Non-invasive external cervical orthosis has been shown to prevent further neurologic decline; however, this treatment modality has not been successful at restoring neurologic and motor function, especially in long standing cases with significant weakness. The pathophysiology remains not entirely understood, complicating standardized operative guidelines; however, some studies report favorable outcomes with internal fixation. We report a successful surgically treated case of pediatric Hirayama disease, supplemented by a systematic review and collation of reported cases in the literature., Methods: A review of the literature was performed by searching PubMed, Embase, and Web of Science. Full-length articles were included if they reported clinical data regarding the treatment of at least one patient with Hirayama disease and the neurologic outcome of that treatment. Articles were excluded if they did not provide information on treatment outcomes, were abstract-only publications, or were published in languages other than English., Results: Of the fifteen articles reviewed, 63 patients were described, with 59 undergoing surgery. This encompassed both anterior and posterior spinal procedures and 1 hand tendon transfer. Fifty-five patients, including one from our institution, showed improvement post-treatment. Eleven of these patients were under 18 years old., Conclusion: Hirayama disease is an infrequent yet impactful cervical myelopathy with limited high-quality evidence available for optimal treatment. The current literature supports surgical decompression and stabilization as promising interventions. However, comprehensive research is crucial for evolving diagnosis and treatment paradigms., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF)., Methods: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio., Results: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95)., Conclusions: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Objectives: To report pediatric intensivists' and pediatric neurosurgeons' responses to case-based scenarios about plasma and platelet transfusions before intracranial pressure (ICP) monitor placement in children with severe traumatic brain injury (TBI)., Design: Cross-sectional, electronic survey to evaluate reported plasma and platelet transfusion decisions in eight scenarios of TBI in which ICP monitor placement was indicated., Setting: Survey administered through the Pediatric Acute Lung Injury and Sepsis Investigators and the American Association of Neurologic Surgeons., Subjects: Pediatric intensivists and pediatric neurosurgeons., Interventions: None., Measurements and Main Results: A total of 184 participants responded (85 identified as pediatric intensivists and 54 as pediatric neurosurgeons). In all eight scenarios, the majority of respondents reported that they would base their decision-making about plasma transfusion on international normalized ratio (INR) alone (60-69%), or platelet transfusion on platelet count alone (83-86%). Pediatric intensivists, as opposed to pediatric neurosurgeons, more frequently reported that they would have used viscoelastic testing in their consideration of plasma transfusion (32% vs. 7%, p < 0.001), as well as to guide platelet transfusions (29 vs. 8%, p < 0.001), for the case-based scenarios. For all relevant case-based scenarios, pediatric neurosurgeons in comparison with pediatric reported that they would use a lower median (interquartile range [IQR]) INR threshold for plasma transfusion (1.5 [IQR 1.4-1.7] vs. 2.0 [IQR 1.5-2.0], p < 0.001). Overall, in all respondents, the reported median platelet count threshold for platelet transfusion in the case-based scenario was 100 (IQR 50-100) ×10 9 /L, with no difference between specialties., Conclusions: Despite little evidence showing efficacy, when we tested specialists' decision-making, we found that they reported using INR and platelet count in pediatric case-based scenarios of TBI undergoing ICP monitor placement. We also found that pediatric intensivists and pediatric neurosurgeons had differences in decision-making about the scenarios., Competing Interests: The authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2024 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.)

Background: Anterior cervicothoracic myelomeningoceles are a rare pathology. In reported cases, treatment has included shunting, isolated resection and repair without deformity correction, or isolated deformity correction without meningocele repair. The authors describe a pediatric patient with an anterior cervicothoracic myelomeningocele presenting with progressive neurological decline, who underwent simultaneous treatment of the myelomeningocele to detether the spinal cord and achieve major correction of the scoliotic deformity., Observations: A 15-year-old girl was born with C7-T1-T2 hemivertebrae and anterior cervical myelomeningocele at C7-T1. She developed progressive cervical thoracic scoliosis, left hemiparesis initially, and additional right hemiparesis eventually. She underwent surgical repair via C7, T1, and T2 corpectomies with intradural detethering of the spinal cord. The scoliosis was treated with C7-T2 Ponte osteotomies and C2-T5 posterior fixation, followed by anterior reconstruction with a titanium cage and anterior plate from C6 to T3. The myelomeningocele was adequately treated with good correction of the patient's deformity. The patient had postoperative improvement in her strength and solid arthrodesis on postoperative imaging., Lessons: The authors describe the successful treatment of an anterior cervicothoracic myelomeningocele and associated scoliosis in a child. This is a unique report of a combined strategy to achieve both deformity correction and detethering of the spinal cord.

Background and Objectives: The purpose of this guideline is to update the 2010 American Academy of Neurology (AAN) brain death/death by neurologic criteria (BD/DNC) guideline for adults and the 2011 American Academy of Pediatrics, Child Neurology Society, and Society of Critical Care Medicine guideline for infants and children and to clarify the BD/DNC determination process by integrating guidance for adults and children into a single guideline. Updates in this guideline include guidance related to conducting the BD/DNC evaluation in the context of extracorporeal membrane oxygenation, targeted temperature management, and primary infratentorial injury., Methods: A panel of experts from multiple medical societies developed BD/DNC recommendations. Because of the lack of high-quality evidence on the subject, a novel, evidence-informed formal consensus process was used. This process relied on the panel experts' review and detailed knowledge of the literature surrounding BD/DNC to guide the development of preliminary recommendations. Recommendations were formulated and voted on, using a modified Delphi process, according to the 2017 AAN Clinical Practice Guideline Process Manual., Major Recommendations: Eighty-five recommendations were developed on the following: (1) general principles for the BD/DNC evaluation, (2) qualifications to perform BD/DNC evaluations, (3) prerequisites for BD/DNC determination, (4) components of the BD/DNC neurologic examination, (5) apnea testing as part of the BD/DNC evaluation, (6) ancillary testing as part of the BD/DNC evaluation, and (7) special considerations for BD/DNC determination.

Background: The cancellation of clinical rotations (CRs) and implementation of virtual interviews (VIs) profoundly affected the residency selection process leading up to the 2021 NRMP Match. The authors investigated how these changes influenced the caliber of applicants taken by general surgery (GS) residency programs from the perspectives of program directors (PDs)., Methods: A 14 question, web-based electronic survey was emailed to PDs of ACGME-accredited GS residency programs. Questions sought program characteristics and PDs' perspectives regarding potential differences in subjective characteristics and clinical skills demonstrated by their 2021 Match class relative to previous resident classes., Results: A total of 75 PDs (27.2%) responded to our survey. Most respondents observed no changes in residents' fit with their program (72.0%), communication skills (68.0%), responsiveness to clinical instruction and feedback (73.3%), work ethic (73.3%), and rotation evaluations (68.0%). Only 21.3% of PDs believed that VIs negatively impacted their ability to accurately assess applicant intangibles. Conversely, 56.0% of PDs reported that the cancellation of CRs in 2020 negatively affected residents' clinical competency at the start of residency. At 1-year following the 2021 NRMP Match, 30.7% of PDs reported that the clinical skills exhibited by their 2021 Match class were poorer than previous resident classes., Discussion: Our findings suggest that VIs limited selection committees' ability to accurately assess applicant's subjective characteristics to a lesser degree than previously described in the literature. Canceled CRs adversely affected the 2021 Match Class's clinical skills at the start of residency and at 1 year following the 2021 NRMP Match., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Chiari I malformation (CIM) is characterized by descent of the cerebellar tonsils through the foramen magnum, potentially causing symptoms from compression or obstruction of the flow of cerebrospinal fluid. Diagnosis and treatment of CIM is varied, and guidelines produced through systematic review may be helpful for clinicians., Objective: To perform a systematic review of the medical literature to answer specific questions on the diagnosis and treatment of CIM., Methods: PubMed and Embase were queried between 1946 and January 23, 2021, using the search strategies provided in Appendix I of the full guidelines., Results: The literature search yielded 567 abstracts, of which 151 were selected for full-text review, 109 were then rejected for not meeting the inclusion criteria or for being off-topic, and 42 were included in this systematic review., Conclusion: Three Grade C recommendations were made based on Level III evidence. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/1-imaging ., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Background: Chiari malformation type I (CIM) diagnoses have increased in recent years. Controversy regarding the best operative management prompted a review of the literature to offer guidance on surgical interventions., Objective: To assess the literature to determine (1) whether posterior fossa decompression or posterior fossa decompression with duraplasty is more effective in preoperative symptom resolution; (2) whether there is benefit from cerebellar tonsillar resection/reduction; (3) the role of intraoperative neuromonitoring; (4) in patients with a syrinx, how long should a syrinx be observed for improvement before additional surgery is performed; and 5) what is the optimal duration of follow-up care after preoperative symptom resolution., Methods: A systematic review was performed using the National Library of Medicine/PubMed and Embase databases for studies on CIM in children and adults. The most appropriate surgical interventions, the use of neuromonitoring, and clinical improvement during follow-up were reviewed for studies published between 1946 and January 23, 2021., Results: A total of 80 studies met inclusion criteria. Posterior fossa decompression with or without duraplasty or cerebellar tonsil reduction all appeared to show some benefit for symptom relief and syrinx reduction. There was insufficient evidence to determine whether duraplasty or cerebellar tonsil reduction was needed for specific patient groups. There was no strong correlation between symptom relief and syringomyelia resolution. Many surgeons follow patients for 6-12 months before considering reoperation for persistent syringomyelia. No benefit or harm was seen with the use of neuromonitoring., Conclusion: This evidence-based clinical guidelines for the treatment of CIM provide 1 Class II and 4 Class III recommendations. In patients with CIM with or without syringomyelia, treatment options include bone decompression with or without duraplasty or cerebellar tonsil reduction. Improved syrinx resolution may potentially be seen with dural patch grafting. Symptom resolution and syrinx resolution did not correlate directly. Reoperation for a persistent syrinx was potentially beneficial if the syrinx had not improved 6 to 12 months after the initial operation. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/3-surgical-interventions ., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Background: Chiari I malformation (CIM) is characterized by descent of the cerebellar tonsils through the foramen magnum, potentially causing symptoms from compression or obstruction of the flow of cerebrospinal fluid. Diagnosis and treatment of CIM is varied, and guidelines produced through systematic review may be helpful for clinicians., Objective: To perform a systematic review of the medical literature to answer specific questions on the diagnosis and treatment of CIM., Methods: PubMed and Embase were queried between 1946 and January 23, 2021, using the search strategies provided in Appendix I of the full guidelines., Results: The literature search yielded 430 abstracts, of which 79 were selected for full-text review, 44 were then rejected for not meeting the inclusion criteria or for being off-topic, and 35 were included in this systematic review., Conclusion: Four Grade C recommendations were made based on Class III evidence, and 1 question had insufficient evidence. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/2-symptoms ., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Background Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder that predominantly affects cardiovascular, skeletal, and craniofacial structures. Associated thoracolumbar scoliosis in LDS can be challenging to manage, though other etiologies of pediatric scoliosis have better-defined management guidelines. We examined our institutional experience regarding the treatment of pediatric patients with LDS and scoliosis. Methodology In this retrospective study, all patients seen at our pediatric tertiary care center from 2004 through 2018 with a diagnosis of LDS were reviewed, and those with radiographic diagnoses of scoliosis (full-length scoliosis X-rays) were included. Demographic, clinical, and radiographic parameters were collected, and management strategies were reported. Results A total of 39 LDS patients whose ages ranged between seven and 13 years were identified. A total of nine patients were radiographically diagnosed with scoliosis, but three patients were excluded due to incomplete medical records, leaving six patients. The median age at scoliosis diagnosis was 11.5 years, with a median follow-up of 51 months. Two patients were successfully managed with observation (average initial Cobb angle (CA): 14°, average final CA: 20.5°). Two were braced, one successfully (initial CA: 15°, final CA: 30°) and one with a progressive disease requiring surgery (initial CA: 40°, final CA: 58°). Of the two who were offered surgical correction, one underwent surgery with a durable correction of spinal deformity (CA: 33° to 19°). One patient underwent a recent correction of aortic root dilatation and was not a candidate for scoliosis surgery. Conclusions Principles of adolescent idiopathic scoliosis management such as bracing for CA of 20-50° and surgery for CA of >50° can be applied to LDS patients with good outcomes. This augments our understanding of the treatment algorithm for pediatric patients with LDS., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, LoPresti et al.)

Primary spinal cord high-grade gliomas, including those histologically identified as glioblastoma (GBM), are a rare entity in the pediatric population but should be considered in the differential diagnosis of intramedullary lesions. Pediatric spinal cord high-grade gliomas have an aggressive course with poor prognosis. The aim of this case report is to present a 15-year-old female adolescent with histopathologically confirmed spinal cord GBM with H3F3A K27 M mutation consistent with a diffuse midline glioma (DMG), H3 K27-altered, CNS WHO grade 4 with leptomeningeal seeding on initial presentation. As imaging features of H3 K27-altered DMGs are non-specific and may mimic more frequently encountered neoplastic diseases as well as demyelinating disorders, severe neurological deficits at presentation with short duration, rapid progression, and early leptomeningeal seeding should however raise the suspicion for a pediatric-type diffuse high-grade glioma like DMG, H3 K27-altered.

Accurate quantification of perfusion is crucial for diagnosis and monitoring of kidney function. Arterial spin labeling (ASL), a completely non-invasive magnetic resonance imaging technique, is a promising method for this application. However, differences in acquisition (e.g., ASL parameters, readout) and processing (e.g., registration, segmentation) between studies impede the comparison of results. To alleviate challenges arising solely from differences in processing pipelines, synthetic data are of great value. In this work, synthetic renal ASL data were generated using body models from the XCAT phantom and perfusion was added using the general kinetic model. Our in-house developed processing pipeline was then evaluated in terms of registration, quantification, and segmentation using the synthetic data. Registration performance was evaluated qualitatively with line profiles and quantitatively with mean structural similarity index measures (MSSIMs). Perfusion values obtained from the pipeline were compared to the values assumed when generating the synthetic data. Segmentation masks obtained by semi-automated procedure of the processing pipeline were compared to the original XCAT organ masks using the Dice index. Overall, the pipeline evaluation yielded good results. After registration, line profiles were smoother and, on average, MSSIMs increased by 25%. Mean perfusion values for cortex and medulla were close to the assumed perfusion of 250 mL/100 g/min and 50 mL/100 g/min, respectively. Dice indices ranged 0.80-0.93, 0.78-0.89, and 0.64-0.84 for whole kidney, cortex, and medulla, respectively. The generation of synthetic ASL data allows flexible choice of parameters and the generated data are well suited for evaluation of processing pipelines.

Objective: In utero repair of fetal posterior cephaloceles (meningocele and encephalocele) is being performed based on the premise that fetal surgery prevents progressive herniation of neural tissue and brain damage during pregnancy. However, the extent to which progressive herniation occurs during pregnancy, specifically from prenatal diagnosis to after delivery, is not well known. The objective of this study was to describe the natural history of patients with fetal cephaloceles focusing on the incidence of progressive herniation., Methods: The authors conducted a retrospective cohort study of all patients referred to their center for posterior fetal cephalocele between 2006 and 2021. All patients underwent prenatal and postnatal MRI. Progressive herniation (primary outcome) was defined as an increase in the absolute volume of neural tissue within the cephalocele of > 5% or new herniation of a critical structure into the cephalocele. Total brain and cephalocele volumes were calculated to determine herniation progression from prenatal to postnatal MRI. Information on the presence of hydrocephalus, epilepsy, and developmental delay (secondary outcomes) was collected at 1 year of age., Results: Twenty patients met all study criteria. Ten patients (50%; 95% CI 0.27-0.73) demonstrated progressive herniation from prenatal to postnatal MRI. Three patients with progressive herniation were diagnosed with a meningocele prenatally and had an encephalocele postnatally. Two patients without progression had meningocele identified prenatally that regressed and became atretic by birth. Both prenatal hindbrain herniation (p = 0.03) and prenatal microcephaly (p = 0.05) were predictive of progressive herniation. The rates of hydrocephalus (44%), epilepsy (44%), and developmental delay (63%) were not associated with the occurrence of progressive herniation in this study., Conclusions: In this study, progressive herniation was not a rare event (50%). Fetal hindbrain herniation and fetal microcephaly were associated with the occurrence of progressive herniation. These results support further investigations into why progressive herniation occurs in utero and if progressive cerebral herniation in utero plays a significant role in determining clinical outcome.

Purpose: The liver is a common site for metastatic disease, which is a challenging and life-threatening condition with a grim prognosis and outcome. We propose a standardized workflow for the diagnosis of oligometastatic disease (OMD), as a gold standard workflow has not been established yet. The envisioned workflow comprises the acquisition of a multimodal image data set, novel image processing techniques, and cone beam computed tomography (CBCT)-guided biopsy for subsequent molecular subtyping. By combining morphological, molecular, and functional information about the tumor, a patient-specific treatment planning is possible. We designed and manufactured an abdominal liver phantom that we used to demonstrate multimodal image acquisition, image processing, and biopsy of the OMD diagnosis workflow., Methods: The anthropomorphic abdominal phantom contains a rib cage, a portal vein, lungs, a liver with six lesions, and a hepatic vessel tree. This phantom incorporates three different lesion types with varying visibility under computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography CT (PET-CT), which reflects clinical reality. The phantom is puncturable and the size of the corpus and the organs is comparable to those of a real human abdomen. By using several modern additive manufacturing techniques, the manufacturing process is reproducible and allows to incorporate patient-specific anatomies. As a first step of the OMD diagnosis workflow, a preinterventional CT, MRI, and PET-CT data set of the phantom was acquired. The image information was fused using image registration and organ information was extracted via image segmentation. A CBCT-guided needle puncture experiment was performed, where all six liver lesions were punctured with coaxial biopsy needles., Results: Qualitative observation of the image data and quantitative evaluation using contrast-to-noise ratio (CNR) confirms that one lesion type is visible only in MRI and not CT. The other two lesion types are visible in CT and MRI. The CBCT-guided needle placement was performed for all six lesions, including those visible only in MRI and not CBCT. This was possible by successfully merging multimodal preinterventional image data. Lungs, bones, and liver vessels serve as realistic inhibitions during needle path planning., Conclusions: We have developed a reusable abdominal phantom that has been used to validate a standardized OMD diagnosis workflow. Utilizing the phantom, we have been able to show that a multimodal imaging pipeline is advantageous for a comprehensive detection of liver lesions. In a CBCT-guided needle placement experiment we have punctured lesions that are invisible in CBCT using registered preinterventional MRI scans for needle path planning., (© 2022 The Authors. Medical Physics published by Wiley Periodicals LLC on behalf of American Association of Physicists in Medicine.)

Objective: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM)., Methods: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups., Results: A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD., Conclusions: PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

Purpose: To design and manufacture a pelvis phantom for magnetic resonance (MR)-guided prostate interventions, such as MRGB (MR-guided biopsy) or brachytherapy seed placement., Methods: The phantom was designed to mimic the human pelvis incorporating bones, bladder, prostate with four lesions, urethra, arteries, veins, and six lymph nodes embedded in ballistic gelatin. A hollow rectum enables transrectal access to the prostate. To demonstrate the feasibility of the phantom for minimal invasive MRI-guided interventions, a targeted inbore MRGB was performed. The needle probe was rectally inserted and guided using an MRI-compatible remote controlled manipulator (RCM)., Results: The presented pelvis phantom has realistic imaging properties for MR imaging (MRI), computed tomography (CT) and ultrasound (US). In the targeted inbore MRGB, a prostate lesion was successfully hit with an accuracy of 3.5 mm. The experiment demonstrates that the limited size of the rectum represents a realistic impairment for needle placements., Conclusion: The phantom provides a valuable platform for evaluating the performance of MRGB systems. Interventionalists can use the phantom to learn how to deal with challenging situations, without risking harm to patients., (© 2021 The Authors. Magnetic Resonance in Medicine published by Wiley Periodicals LLC on behalf of International Society for Magnetic Resonance in Medicine.)

Cone-beam CT (CBCT) with non-circular acquisition orbits has the potential to improve image quality, increase the field-of view, and facilitate minimal interference within an interventional imaging setting. Because time is of the essence in interventional imaging scenarios, rapid reconstruction methods are advantageous. Model-Based Iterative Reconstruction (MBIR) techniques implicitly handle arbitrary geometries; however, the computational burden for these approaches is particularly high. The aim of this work is to extend a previously proposed framework for fast reconstruction of non-circular CBCT trajectories. The pipeline combines a deconvolution operation on the backprojected measurements using an approximate, shift-invariant system response prior to processing with a Convolutional Neural Network (CNN). We trained and evaluated the CNN for this approach using 1800 randomized arbitrary orbits. Noisy projection data were formed from 1000 procedurally generated tetrahedral phantoms as well as anthropomorphic data in the form of 800 CT and CBCT images from the Lung Image Database Consortium Image Collection (LIDC). Using this proposed reconstruction pipeline, computation time was reduced by 90% as compared to MBIR with only minor differences in performance. Quantitative comparisons of nRMSE, FSIM and SSIM are reported. Performance was consistent for projection data simulated with acquisition orbits the network has not previously been trained on. These results suggest the potential for fast processing of arbitrary CBCT trajectory data with reconstruction times that are clinically relevant and applicable - facilitating the application of non-circular orbits in CT image-guided interventions and intraoperative imaging.

Abstract: The authors provide the case of a 6-year-old male who presented late with multi-suture craniosynostosis and chronically elevated intracranial pressures (ICPs). He was surgically managed with frontal orbital advancement. This particular case illustrates the significant bleeding and unique bony pathology that can occur in patients with high ICP with concomitant venous collateralization. At 1-month follow-up, he demonstrated significant improvement with maintained expansion and no signs of elevated ICP despite delayed intervention. Frontal orbital advancement serves as an effective method for cranial vault expansion and correction of frontal deformities caused by craniosynostosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Purpose of Review: This review provides guidance for acute spinal cord injury (SCI) management through an analytical assessment of the most recent evidence on therapies available for treating SCI, including newer therapies under investigation. We present an approach to the SCI patient starting at presentation to acute rehabilitation and prognostication, with additional emphasis on the pediatric population when evidence is available., Recent Findings: Further studies since the Surgical Timing in Acute Spinal Cord Injury Study (STASCIS) demonstrated a potential functional outcome benefit with ultra-early surgical intervention ≤ 8 h post-SCI. Subsequent analysis of the National Acute Spinal Cord Injury Study (NASCIS) II and NASCIS III trials have demonstrated potentially serious complications from intravenous methylprednisolone with limited benefit. Newer therapies actively being studied have demonstrated limited or no benefit in preclinical and clinical trials with insufficient evidence to support use in acute SCI treatment., Summary: Care for SCI patients requires a multi-disciplinary team. Immediate evaluation and management are focused on preventing additional injury and restoring perfusion to the affected cord. Rapid assessment and intervention involve focused neurological examination, targeted imaging, and surgical intervention when indicated. There are currently no evidence-based recommendations for pathomechanistically targeted therapies., Competing Interests: Conflict of InterestThe authors did not receive support from any organization for the submitted work. The authors have no relevant financial or non-financial interests to disclose. Open access of this publication was supported by the Texas Children’s Hospital Young Investigator Endowment., (© The Author(s) 2022.)

Objectives: Critically ill children frequently receive plasma and platelet transfusions. We sought to determine evidence-based recommendations, and when evidence was insufficient, we developed expert-based consensus statements about decision-making for plasma and platelet transfusions in critically ill pediatric patients., Design: Systematic review and consensus conference series involving multidisciplinary international experts in hemostasis, and plasma/platelet transfusion in critically ill infants and children (Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding [TAXI-CAB])., Setting: Not applicable., Patients: Children admitted to a PICU at risk of bleeding and receipt of plasma and/or platelet transfusions., Interventions: None., Measurements and Main Results: A panel of 29 experts in methodology, transfusion, and implementation science from five countries and nine pediatric subspecialties completed a systematic review and participated in a virtual consensus conference series to develop recommendations. The search included MEDLINE, EMBASE, and Cochrane Library databases, from inception to December 2020, using a combination of subject heading terms and text words for concepts of plasma and platelet transfusion in critically ill children. Four graded recommendations and 49 consensus expert statements were developed using modified Research and Development/UCLA and Grading of Recommendations, Assessment, Development, and Evaluation methodology. We focused on eight subpopulations of critical illness (1, severe trauma, intracranial hemorrhage, or traumatic brain injury; 2, cardiopulmonary bypass surgery; 3, extracorporeal membrane oxygenation; 4, oncologic diagnosis or hematopoietic stem cell transplantation; 5, acute liver failure or liver transplantation; 6, noncardiac surgery; 7, invasive procedures outside the operating room; 8, sepsis and/or disseminated intravascular coagulation) as well as laboratory assays and selection/processing of plasma and platelet components. In total, we came to consensus on four recommendations, five good practice statements, and 44 consensus-based statements. These results were further developed into consensus-based clinical decision trees for plasma and platelet transfusion in critically ill pediatric patients., Conclusions: The TAXI-CAB program provides expert-based consensus for pediatric intensivists for the administration of plasma and/or platelet transfusions in critically ill pediatric patients. There is a pressing need for primary research to provide more evidence to guide practitioners., Competing Interests: Drs. Nellis, Bateman, Bembea, and Russell received support for article research from the National Institutes of Health. Dr. Bembea’s institution received funding from the National Institute of Neurologic Disorders and Stroke (R01NS106292), the National Institute of Child Health and Human Development, and Grifols Investigator Sponsored Research Grant. Dr. Nishijima received funding from Bristol Myers Squibb. Dr. Emani received funding from Cheisi Pharmaceuticals. Dr. Haas received funding from Octapharma. Dr. Goel received funding from the National Heart, Lung, and Blood Institute and Rigel Pharmaceuticals. Dr. Crighton disclosed that she is employed by Royal Children’s Hospital of Melbourne, Australia and that she was the Australian and New Zealand Society of Blood Transfusion President. The remaining authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2021 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.)

Objectives: To present consensus statements and supporting literature for plasma and platelet transfusions in critically ill children with severe trauma, traumatic brain injury, and/or intracranial hemorrhage from the Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding., Design: Systematic review and consensus conference of international, multidisciplinary experts in platelet and plasma transfusion management of critically ill children., Setting: Not applicable., Patients: Critically ill neonates and children with severe trauma, traumatic brain injury, and/or intracranial hemorrhage., Interventions: None., Measurements and Main Results: A panel of eight experts developed expert-based statements for plasma and platelet transfusions in critically ill neonates and children with severe trauma, traumatic brain injury, and/or intracranial hemorrhage. These statements were reviewed and ratified by the 29 Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding experts. A systematic review was conducted using MEDLINE, EMBASE, and Cochrane Library databases, from inception to December 2020. Consensus was obtained using the Research and Development/University of California, Los Angeles Appropriateness Method. Results were summarized using the Grading of Recommendations Assessment, Development, and Evaluation method. We developed one good practice statement and six expert consensus statements., Conclusions: The lack of evidence precludes proposing recommendations on monitoring of the coagulation system and on plasma and platelets transfusion in critically ill pediatric patients with severe trauma, severe traumatic brain injury, or nontraumatic intracranial hemorrhage., Competing Interests: Drs. Russell and Nellis received support for article research from the National Institutes of Health. Dr. Haas is a consultant for Octapharma. Dr. Nishijima is a consultant for Bristol Myers Squibb (BMS). Dr. Haas received funding from Octapharma. Dr. Nishijima received funding from BMS. The remaining authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2022 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.)

Abdominal aortic aneurysms (AAA) may remain clinically silent until they enlarge and patients present with a potentially lethal rupture. This necessitates early detection and elective treatment. The goal of this study was to develop an easy-to-train algorithm which is capable of automated AAA screening in CT scans and can be applied to an intra-hospital environment. Three deep convolutional neural networks (ResNet, VGG-16 and AlexNet) were adapted for 3D classification and applied to a dataset consisting of 187 heterogenous CT scans. The 3D ResNet outperformed both other networks. Across the five folds of the first training dataset it achieved an accuracy of 0.856 and an area under the curve (AUC) of 0.926. Subsequently, the algorithms performance was verified on a second data set containing 106 scans, where it ran fully automated and resulted in an accuracy of 0.953 and an AUC of 0.971. A layer-wise relevance propagation (LRP) made the decision process interpretable and showed that the network correctly focused on the aortic lumen. In conclusion, the deep learning-based screening proved to be robust and showed high performance even on a heterogeneous multi-center data set. Integration into hospital workflow and its effect on aneurysm management would be an exciting topic of future research.

Chari 1 malformation, a radiologic finding of caudal cerebellar tonsillar displacement, has a clinical course that can range from benign to complications involving life-threatening hydrocephalus. While the pathophysiologic processes underlying this variation in outcome remain a matter of scientific debate, the clinical realities and decision-making conundrums that these patients pose require a coherent approach to this entity. In this review, we seek to highlight the various processes underlying the development of hydrocephalus in patients with Chiari 1 malformations. Hydrocephalus may occur as a cause, consequence, or in parallel with the development of Chiari 1 malformation, and understanding the etiology of such hydrocephalus is critical to the treatment of Chiari 1 malformations with associated hydrocephalus. We further discuss the literature pertaining to the management of these patients and unify the current scientific thinking on Chiari 1 malformations with the extant data on operative management of Chiari 1 to develop a structured and pragmatic approach to the diagnosis and management of patients with Chiari 1-associated hydrocephalus., Competing Interests: None

Extensive multifocal intradural lesions in children present a formidable challenge. This surgical video illustrates our management of a 14-yr=old boy with two intradural mass lesions on magnetic resonance imaging (MRI): one at T2-5 and the other from T12 through the sacral cul-de-sac. In a single procedure, we performed a T2-5 laminectomy and laminoplasty and T12-sacrum laminectomy for tumor resection. For reconstruction, we performed complete laminoplasty at all levels with supplementation at the thoracolumbar junction via T11-L2 posterior spinal fixation and allograft placement for fusion. In this video, we illustrate the microsurgical challenges of intradural tumor resection in both the thoracic cord and amidst the cauda equina. In young patients, prevention of postsurgical spinal deformity is of paramount concern. We discuss considerations for long-segment spinal stabilization in an adolescent and describe our decision-making to perform stabilization at the thoracolumbar junction to supplement laminoplasty while preserving function. The patient and their family consented to the procedure.  Image of the article at 0:51 is from McGirt et al, Short-term progressive spinal deformity following laminoplasty versus laminectomy for resection of intradural spinal tumors: analysis of 239 patients, Neurosurgery, 2010, 66(5), 1005-1012, by permission of the Congress of Neurological Surgeons., (© Congress of Neurological Surgeons 2021.)

Clinical/methodological Issue: Multiparametric magnetic resonance imaging (mpMRI) of the prostate plays a crucial role in the diagnosis and local staging of primary prostate cancer., Standard Radiological Methods: Image-guided biopsy techniques such as MRI-ultrasound fusion not only allow guidance for targeted tissue sampling of index lesions for diagnostic confirmation, but also improve the detection of clinically significant prostate cancer., Methodological Innovations: Minimally invasive, focal therapies of localized prostate cancer complement the treatment spectrum, especially for low- and intermediate-risk patients., Performance: In patients of low and intermediate risk, MR-guided, minimally invasive therapies could enable local tumor control, improved functional outcomes and possible subsequent therapy escalation. Further study results related to multimodal approaches and the application of artificial intelligence (AI) by machine and deep learning algorithms will help to leverage the full potential of focal therapies for prostate cancer in the upcoming era of precision medicine., Achievements: Completion of ongoing randomized trials comparing each minimally invasive therapy approach with established whole-gland procedures is needed before minimally invasive therapies can be implemented into existing treatment guidelines., Practical Recommendations: This review article highlights minimally invasive therapies of prostate cancer and the key role of mpMRI for planning and conducting these therapies., (© 2021. Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Movement disorders in a pediatric population represent a spectrum of secondary functional deficits affecting ease of care, ambulation, and activities of daily living. Cerebral palsy represents the most common form of movement disorder seen in the pediatric population. Several medical and surgical options exist in the treatment of pediatric spasticity and dystonia, which can have profound effects on the functionality of these patients. Given the complex medical and surgical problems in these patients, children are well served by a multidisciplinary team of practitioners, including physical therapists, physical medicine and rehabilitation physicians, and surgeons., Competing Interests: Disclosure No sources of funding or financial support were used in the production of this article. The authors have no conflicts of interest related to this article., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: Sparsity of annotated data is a major limitation in medical image processing tasks such as registration. Registered multimodal image data are essential for the diagnosis of medical conditions and the success of interventional medical procedures. To overcome the shortage of data, we present a method that allows the generation of annotated multimodal 4D datasets., Methods: We use a CycleGAN network architecture to generate multimodal synthetic data from the 4D extended cardiac-torso (XCAT) phantom and real patient data. Organ masks are provided by the XCAT phantom; therefore, the generated dataset can serve as ground truth for image segmentation and registration. Realistic simulation of respiration and heartbeat is possible within the XCAT framework. To underline the usability as a registration ground truth, a proof of principle registration is performed., Results: Compared to real patient data, the synthetic data showed good agreement regarding the image voxel intensity distribution and the noise characteristics. The generated T1-weighted magnetic resonance imaging, computed tomography (CT), and cone beam CT images are inherently co-registered. Thus, the synthetic dataset allowed us to optimize registration parameters of a multimodal non-rigid registration, utilizing liver organ masks for evaluation., Conclusion: Our proposed framework provides not only annotated but also multimodal synthetic data which can serve as a ground truth for various tasks in medical imaging processing. We demonstrated the applicability of synthetic data for the development of multimodal medical image registration algorithms., (© 2021. The Author(s).)

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.

Texas Children's Hospital opened its doors in 1954, and since that time the institution has remained dedicated to a three-part mission: patient care, education, and research. Dr. William R. Cheek developed an early interest in pediatric neurosurgery, which led to his efforts in building and developing a service at Texas Children's Hospital at a time when the field was just emerging. His work with other early pioneers in the field led to the establishment of organized societies, educational texts, and governing bodies that have led to significant advances in the field over the past 50 years.

Objective: Three-dimensional (3D) blood vessel structure information is important for diagnosis and treatment in various clinical scenarios. We present a fully automatic method for the extraction and differentiation of the arterial and venous vessel trees from abdominal contrast enhanced computed tomography (CE-CT) volumes using convolutional neural networks (CNNs)., Methods: We used a novel ratio-based sampling method to train 2D and 3D versions of the U-Net, the V-Net and the DeepVesselNet. Networks were trained with a combination of the Dice and cross entropy loss. Performance was evaluated on 20 IRCAD subjects. Best performing networks were combined into an ensemble. We investigated seven different weighting schemes. Trained networks were additionally applied to 26 BTCV cases to validate the generalizability., Results: Based on our experiments, the optimal configuration is an equally weighted ensemble of 2D and 3D U- and V-Nets. Our method achieved Dice similarity coefficients of 0.758 ± 0.050 (veins) and 0.838 ± 0.074 (arteries) on the IRCAD data set. Application to the BTCV data set showed a high transfer ability., Conclusion: Abdominal vascular structures can be segmented more accurately using ensembles than individual CNNs. 2D and 3D networks have complementary strengths and weaknesses. Our ensemble of 2D and 3D U-Nets and V-Nets in combination with ratio-based sampling achieves a high agreement with manual annotations for both artery and vein segmentation. Our results surpass other state-of-the-art methods., Significance: Our segmentation pipeline can provide valuable information for the planning of living donor organ transplantations.

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.

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 < .001) were increased within the OCF group, whereas only basilar invagination (1/4, P < .001) was increased in the OCF/VD group. Clivo-axial angle (CXA) was significantly lower for both OCF (128.8 ± 15.3°, P = .008) and OCF/VD (115.0 ± 11.6°, P = .025) groups when compared to PFD-only group (145.3 ± 12.7°). pB-C2 did not differ among groups., Conclusion: Although PFD alone is adequate for treating the vast majority of CM-1/SM patients, OCF or OCF/VD may be occasionally utilized. Cranial base and spine pathologies and CXA may provide insight into the need for OCF and/or OCF/VD., (Copyright © 2020 by the Congress of Neurological Surgeons.)

Background: The Congress of Neurological Surgeons reviews its guidelines according to the Institute of Medicine's recommended best practice of reviewing guidelines every 5 yrs. The authors performed a planned 5-yr review of the medical literature used to develop the "Pediatric hydrocephalus: systematic literature review and evidence-based guidelines" and determined the need for an update to the original guideline based on new available evidence., Objective: To perform an update to include the current medical literature for the "Pediatric hydrocephalus: systematic literature review and evidence-based guidelines", originally published in 2014., Methods: The Guidelines Task Force used the search terms and strategies consistent with the original guidelines to search PubMed and Cochrane Central for relevant literature published between March 2012 and November 2019. The same inclusion/exclusion criteria were also used to screen abstracts and to perform the full-text review. Full text articles were then reviewed and when appropriate, included as evidence and recommendations were added or changed accordingly., Results: A total of 41 studies yielded by the updated search met inclusion criteria and were included in this update., Conclusion: New literature resulting from the update yielded a new recommendation in Part 2, which states that neuro-endoscopic lavage is a feasible and safe option for the removal of intraventricular clots and may lower the rate of shunt placement (Level III). Additionally a recommendation in part 7 of the guideline now states that antibiotic-impregnated shunt tubing reduces the risk of shunt infection compared with conventional silicone hardware and should be used for children who require placement of a shunt (Level I). ., (Copyright © 2020 by the Congress of Neurological Surgeons.)

Objective: Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established., Methods: Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length., Results: The authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16-0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34-1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12-0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02-2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31-3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67-5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate., Conclusions: These data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.

Background: Craniosynostosis has a known association with rickets. Because of abnormal bone development and a tendency for patients with rickets to present at an older age than most patients with craniosynostosis, repair may be complicated by inadequate cranial expansion and greater risk of sutural re-fusion. We present 2 cases of rickets-associated sagittal craniosynostosis and show the effectiveness of distraction osteogenesis in the surgical management of this condition., Case Description: Two 3-year-old boys with rickets presented with sagittal synostosis and marked scaphocephalic deformity. Cranial osteotomies were performed followed by placement of 2 internal distractors. The devices were activated daily for 30 days to achieve a target transverse cranial distraction of 30 mm. A subsequent period of 10-12 weeks allowed for adequate bone consolidation. At the time of device removal, the final breadth of distraction and the quality of new bone formed was assessed. Distraction distance in both cases was verified intraoperatively to be 30 mm. Clinical examination confirmed that the distraction gaps were bridged by solid bone. The treatment protocol resulted in a significant improvement in cranial proportion in both patients, which was maintained at long-term follow-up. Estimated blood loss was high in both cases, presumably related to the hyperemic nature of rickets bone., Conclusions: Distraction osteogenesis promotes bone growth and cranial remodeling in patients with craniosynostosis caused by rickets. The technique allows for continuous incremental expansion of both bone and scalp tissue. We recommend consideration of distraction osteogenesis in the treatment of older children with severe deformity related to craniosynostosis, including those with rickets., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: The potential of medical image analysis with neural networks is limited by the restricted availability of extensive data sets. The incorporation of synthetic training data is one approach to bypass this shortcoming, as synthetic data offer accurate annotations and unlimited data size., Methods: We evaluated eleven CycleGAN for the synthesis of computed tomography (CT) images based on XCAT body phantoms. The image quality was assessed in terms of anatomical accuracy and realistic noise properties. We performed two studies exploring various network and training configurations as well as a task-based adaption of the corresponding loss function., Results: The CycleGAN using the Res-Net architecture and three XCAT input slices achieved the best overall performance in the configuration study. In the task-based study, the anatomical accuracy of the generated synthetic CTs remained high ([Formula: see text] and [Formula: see text]). At the same time, the generated noise texture was close to real data with a noise power spectrum correlation coefficient of [Formula: see text]. Simultaneously, we observed an improvement in annotation accuracy of 65% when using the dedicated loss function. The feasibility of a combined training on both real and synthetic data was demonstrated in a blood vessel segmentation task (dice similarity coefficient [Formula: see text])., Conclusion: CT synthesis using CycleGAN is a feasible approach to generate realistic images from simulated XCAT phantoms. Synthetic CTs generated with a task-based loss function can be used in addition to real data to improve the performance of segmentation networks.

Background: The incidence of spina bifida (SB) is higher in the developing world as compared to the United States because of folic acid deficiency during pregnancy. Advances in technology have made prenatal repair of myelomeningocele (MM) possible., Objective: The objective of this guideline was to determine if there is a difference in the rate of development of tethered cord syndrome (TCS) in infants who had prenatal closure compared to infants who had MM repair after birth., Methods: The Guidelines Task Force developed search terms and strategies to search PubMed and Embase for the relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used. Full text articles were reviewed and, when appropriate, included as evidence., Results: A total of 261 abstracts were reviewed. Fifty-four full-text articles were selected for further analysis. Three studies met inclusion criteria., Conclusion: There was Class II evidence from 1 study and Class III evidence from another 2 studies demonstrating that TCS develops in infants with prenatal MM closure at an equal or higher rate than with postnatal closure. There was an increased risk of development of inclusion cysts in infants who underwent in utero closure. Continued surveillance for TCS and/or the development of inclusion cysts in children with prenatal and postnatal closure of MM is indicated (Level II). Differences between prenatal and postnatal repair with respect to the development of TCS and/or inclusion cysts should be considered alongside other relevant maternal and fetal outcomes when deciding upon a preferred method for MM closure.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-6., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Background: Myelomeningocele (MM) is an open neural tube defect treated by pediatric neurosurgeons with prenatal or postnatal closure., Objective: The objective of this systematic review was to answer the question: What is the evidence for the effectiveness of prenatal vs postnatal closure of MM regarding short and long-term ambulatory status? Treatment recommendations were provided based on the available evidence., Methods: The National Library of Medicine PubMed database and Embase were queried using MeSH headings and keywords relevant to ambulatory status after prenatal or postnatal closure of MM. Abstracts were reviewed to identify which studies met the inclusion criteria. An evidence table was assembled summarizing the studies and the quality of evidence (Classes I-III). Based on the quality of the literature, a recommendation was rendered (Level I, II, or III)., Results: One randomized controlled trial (Class II) and 3 retrospective cohort studies (Class III) were included as evidence. Initial ambulatory status depended on anatomic level of the neural tube defect. In the short term, prenatal closure may improve ambulatory status compared to postnatal closure. Spinal cord tethering or dermoid inclusion cyst has been associated with neurologic deterioration in infants closed in utero and after birth. Ambulation may cease in both groups over time. No long-term studies evaluated whether there is a difference in the ability to ambulate upon reaching adulthood., Conclusion: Prenatal closure of MM may improve ambulatory status in the short term (Level II). Spinal cord tethering in both groups caused deterioration in the ability to walk. Evaluation and treatment of spinal cord tethering may help maintain ambulatory status (Level III). No studies evaluate whether prenatal or postnatal repair provides improved ability to ambulate upon reaching adulthood.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-3., (Copyright © 2019 by the Congress of Neurological Surgeons.)