Disparities in indications and outcomes reporting for pediatric tethered cord surgery: The need for a standardized outcome assessment tool.

PURPOSE: Tethered cord syndrome (TCS) is characterized by abnormal attachment of the spinal cord neural elements to surrounding tissues. The most common symptoms include pain, motor or sensory dysfunction, and urologic deficits. Although TCS is common in children, there is a significant heterogeneity in outcomes reporting. We systematically reviewed surgical indications and postoperative outcomes to assess the need for a grading/classification system. METHODS: PubMed and EMBASE searches identified pediatric TCS literature published between 1950 and 2023. Studies reporting surgical interventions, ≥ 6-month follow-up, and ≥ 5 patients were included. RESULTS: Fifty-five studies representing 3798 patients were included. The most commonly reported non-urologic symptoms were nonspecific lower-extremity motor disturbances (36.4% of studies), lower-extremity/back pain (32.7%), nonspecific lower-extremity sensory disturbances (29.1%), gait abnormalities (29.1%), and nonspecific bowel dysfunction/fecal incontinence (25.5%). Urologic symptoms were most commonly reported as nonspecific complaints (40.0%). After detethering surgery, retethering was the most widely reported non-urologic outcome (40.0%), followed by other nonspecific findings: motor deficits (32.7%), lower-extremity/back/perianal pain (18.2%), gait/ambulation function (18.2%), sensory deficits (12.7%), and bowel deficits/fecal incontinence (12.7%). Commonly reported urologic outcomes included nonspecific bladder/urinary deficits (27.3%), bladder capacity (20.0%), bladder compliance (18.2%), urinary incontinence/enuresis/neurogenic bladder (18.2%), and nonspecific urodynamics/urodynamics score change (16.4%). CONCLUSION: TCS surgical literature is highly variable regarding surgical indications and reporting of postsurgical outcomes. The lack of common data elements and consistent quantitative measures inhibits higher-level analysis. The development and validation of a standardized outcomes measurement tool-ideally encompassing both patient-reported outcome and objective measures-would significantly benefit future TCS research and surgical management.

Primary pituitary abscess in an adolescent female patient: case report, literature review, and operative video.

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: To the best of our knowledge, this is the second report in the literature of a primary pituitary abscess due to C. acnes infection.

Real-time navigation guidance with intraoperative CT imaging for pedicle screw placement using an augmented reality head-mounted display: a proof-of-concept study.

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.

Endoscopic third ventriculostomy for pediatric tumor-associated hydrocephalus.

OBJECTIVE: Surgical options for managing hydrocephalus secondary to CNS tumors have traditionally included ventriculoperitoneal shunting (VPS) when tumor resection or medical management alone are ineffective. Endoscopic third ventriculostomy (ETV) has emerged as an attractive treatment strategy for tumor-associated hydrocephalus because it offers a lower risk of infection and hardware-related complications; however, relatively little has been written on the topic of ETV specifically for the treatment of tumor-associated hydrocephalus. Here, the authors reviewed the existing literature on the use of ETV in the treatment of tumor-associated hydrocephalus, focusing on the frequency of ETV use and the failure rates in patients with hydrocephalus secondary to CNS tumor. METHODS: The authors queried PubMed for the following terms: "endoscopic third ventriculostomy," "tumor," and "pediatric." Papers with only adult populations, case reports, and papers published before the year 2000 were excluded. The authors analyzed the etiology of hydrocephalus and failure rates after ETV, and they compared failure rates of ETV with those of VPS where reported. RESULTS: Thirty-two studies with data on pediatric patients undergoing ETV for tumor-related hydrocephalus were analyzed. Tumors, particularly in the posterior fossa, were reported as the etiology of hydrocephalus in 38.6% of all ETVs performed (984 of 2547 ETVs, range 29%-55%). The ETV failure rate in tumor-related hydrocephalus ranged from 6% to 38.6%, and in the largest studies analyzed (> 100 patients), the ETV failure rate ranged from 10% to 38.6%. The pooled ETV failure rate was 18.3% (199 failures after 1087 procedures). The mean or median follow-up for ETV failure assessment ranged from 6 months to 8 years in these studies. Only 5 studies directly compared ETV with VPS for tumor-associated hydrocephalus, and they reported mixed results in regard to failure rate and time to failure. Overall failure rates appear similar for ETV and VPS over time, and the risk of infection appears to be lower in those patients undergoing ETV. The literature is mixed regarding the need for routine ETV before resection for posterior fossa tumors with associated hydrocephalus. CONCLUSIONS: Treatment of tumor-related hydrocephalus with ETV is common and is warranted in select pediatric patient populations. Failure rates are overall similar to those of VPS for tumor-associated hydrocephalus.

Stereotactic laser interstitial thermal therapy for brainstem cavernous malformations: two preliminary cases.

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.

Use of computer-assisted design and manufacturing to localize dural venous sinuses during reconstructive surgery for craniosynostosis.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Single incision endoscope-assisted surgery for sagittal craniosynostosis.

OBJECTIVE: The objective of this study is to present the novel technique and associated results of a single-incision endoscope-assisted procedure for the treatment of sagittal craniosynostosis. METHODS: We retrospectively reviewed the charts of infants who underwent single-incision endoscope-assisted sagittal craniectomy for craniosynostosis at our institution. Demographic data collected included patient age, blood loss, operative time, pre- and post-operative hemoglobin, pre- and post-operative cephalic index (CI), and hospital length of stay. RESULTS: Seven consecutive infants underwent surgery for sagittal craniosynostosis using a single-incision endoscopic technique. Average operative time was 87 (±10.5) minutes. Average blood loss was 32 (±13.5) cubic centimeters (cc). Post-operative hemoglobin was an average of 7.1 (±0.2) g/dL. No patients required a blood transfusion intra-operatively or in the post-operative setting. Dural tears were encountered in one patient. The average hospital length of stay was 1.4 (±1.1) days. Difference between pre- and post-operative CI was 8.4 % (±3.5; p < 0.05). CONCLUSIONS: We demonstrate the novel use of a single-incision technique for endoscope-assisted sagittal craniosynostosis correction that improves upon the classically described surgical procedure by decreasing invasiveness, while allowing for excellent clinical outcomes.

Instrumented fusion for spinal deformity after laminectomy or laminoplasty for resection of intramedullary spinal cord tumors in pediatric patients.

OBJECTIVE Spinal deformity has become a well-recognized complication of intramedullary spinal cord tumor (IMSCT) resection. In particular, laminectomy can result in biomechanical instability caused by loss of the posterior tension band. Therefore, laminoplasty has been proposed as an alternative to laminectomy. Here, the authors describe the largest current series of pediatric patients who have undergone laminoplasty for IMSCT resection and investigate the need for surgical fusion after both laminectomy and laminoplasty. METHODS The medical records of pediatric patients who underwent resection of an IMSCT at a single institution between November 2003 and May 2014 were reviewed retrospectively. Demographic, clinical, radiological, surgical, histopathological, and follow-up data were collected. RESULTS Sixty-six consecutive patients underwent resection of an IMSCT during the study period. Forty-three (65%) patients were male. The patients had a median age of 12.9 years (interquartile range [IQR] 7.2-16.5 years) at the time of surgery. Patients typically presented with a tumor that involved the cervical and/or thoracic spine. Nineteen (29%) patients underwent laminectomy, and 47 (71%) patients underwent laminoplasty. Patients in each cohort had a similar rate of postoperative deformity. Overall, 10 (15%) patients required instrumented spinal fusion for spinal deformity. Four patients required revision of the primary fusion. CONCLUSIONS These findings show that among pediatric patients with an IMSCT, postoperative surgical fusion rates remain high, even after laminoplasty. Known risk factors, such as the age of the patient, location of the tumor, and the number of involved levels, might play a larger role than replacement of the laminae in determining the rate of surgical fusion after IMSCT resection.

ß-Catenin signaling initiates the activation of astrocytes and its dysregulation contributes to the pathogenesis of astrocytomas.

Astrocytes are the most abundant cell of the CNS and demonstrate contact inhibition in which a nonproliferative, nonmotile cellular state is achieved once stable intercellular contacts are formed between mature cells. Cellular injury disrupts these intercellular contacts, causing a loss of contact inhibition and the rapid initiation of healing. Dysregulation of the molecular pathways involved in this process is thought to lead to an aggressive cellular state associated with neoplasia. We investigated whether a comparable correlation exists between the response of astrocytes to injury and the malignant phenotype of astrocytomas. We discovered that the loss of contact inhibition plays a critical role in the initiation and regulation of reactive astrocytes in the healing of wounds. In particular, injury of the astrocytes interrupts and destabilizes the cadherin-catenin complexes at the cell membrane leading to nuclear translocation of ß-catenin and characteristic changes associated with the activation of astrocytes. Similar signaling pathways are found to be active--but dysregulated--in astrocytomas. Inhibition of ß-catenin signaling diminished both the response of astrocytes to injury and induction of the malignant phenotype of astrocytomas. The findings shed light on a unique mechanism associated with the pathogenesis of astrocytomas and provide a model for the loss of contact inhibition that may broadly apply to understanding the mechanisms of tissue repair and tumorigenesis in the brain.

Missense mutations in the NF2 gene result in the quantitative loss of merlin protein and minimally affect protein intrinsic function.

Neurofibromatosis type 2 (NF2) is a multiple neoplasia syndrome and is caused by a mutation of the NF2 tumor suppressor gene that encodes for the tumor suppressor protein merlin. Biallelic NF2 gene inactivation results in the development of central nervous system tumors, including schwannomas, meningiomas, ependymomas, and astrocytomas. Although a wide variety of missense germline mutations in the coding sequences of the NF2 gene can cause loss of merlin function, the mechanism of this functional loss is unknown. To gain insight into the mechanisms underlying loss of merlin function in NF2, we investigated mutated merlin homeostasis and function in NF2-associated tumors and cell lines. Quantitative protein and RT-PCR analysis revealed that whereas merlin protein expression was significantly reduced in NF2-associated tumors, mRNA expression levels were unchanged. Transfection of genetic constructs of common NF2 missense mutations into NF2 gene-deficient meningioma cell lines revealed that merlin loss of function is due to a reduction in mutant protein half-life and increased protein degradation. Transfection analysis also demonstrated that recovery of tumor suppressor protein function is possible, indicating that these mutants maintain intrinsic functional capacity. Further, increased expression of mutant protein is possible after treatment with specific proteostasis regulators, implicating protein quality control systems in the degradative fate of mutant tumor suppressor proteins. These findings provide direct insight into protein function and tumorigenesis in NF2 and indicate a unique treatment paradigm for this disorder.

Pre-op considerations in neuromuscular scoliosis deformity surgery: proceedings of the half day course at the 58th annual meeting of the Scoliosis Research Society.

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.

Disparities in Indications and Outcomes Reporting for Spinal Column Shortening for Tethered Cord Syndrome: The Need for a Standardized Approach.

STUDY DESIGN: Systematic review. OBJECTIVE: To identify commonly reported indications and outcomes in spinal column shortening (SCS) procedures. SUMMARY OF BACKGROUND DATA: SCS is a surgical procedure used in patients with tethered cord syndrome (TCS)-characterized by abnormal attachment of neural components to surrounding tissues-to shorten the vertebral column, release tension on the spinal cord/neural elements, and alleviate associated symptoms. METHODS: PubMed and EMBASE searches captured SCS literature published between 1950 and 2023. Prospective/retrospective cohort studies and case series were included without age limit or required follow-up period. Review articles without new patient presentations, meta-analyses, systematic reviews, conference abstracts, and letters were excluded. Studies included adult and pediatric patients. RESULTS: The 29 identified studies represented 278 patients (age 5-76 y). In 24.1% of studies, patients underwent primary TCS intervention via SCS. In 41.4% of studies, patients underwent SCS after failed previous primary detethering (24.1% of studies were mixed and 10.3% were unspecified). The most commonly reported non-genitourinary/bowel surgical indications were back pain (55.2%), lower-extremity pain (48.3%), lower-extremity weakness (48.3%), lower-extremity numbness (34.5%), and lower-extremity motor dysfunction (34.5%). Genitourinary/bowel symptoms were most often described as nonspecific bladder dysfunction (58.6%), bladder incontinence (34.5%), and bowel dysfunction (31.0%). After SCS, non-genitourinary/bowel outcomes included lower-extremity pain (44.8%), back pain (31.0%), and lower-extremity sensory and motor function (both 31.0%). Bladder dysfunction (79.3%), bowel dysfunction (34.5%), and bladder incontinence (13.8%) were commonly reported genitourinary/bowel outcomes. In total, 40 presenting surgical indication categories and 33 unique outcome measures were reported across studies. Seventeen of the 278 patients (6.1%) experienced a complication. CONCLUSION: The SCS surgical literature displays variability in operative indications and postoperative outcomes. The lack of common reporting mechanisms impedes higher-level analysis. A standardized outcomes measurement tool, encompassing both patient-reported outcome measures and objective metrics, is necessary. LEVEL OF EVIDENCE: Level 4.

Frequency and predictors of complication clustering within 30 days of spinal fusion surgery: a study of children with neuromuscular scoliosis.

PURPOSE: There is limited information on the clustering or co-occurrence of complications after spinal fusion surgery for neuromuscular disease in children. We aimed to identify the frequency and predictive factors of co-occurring perioperative complications in these children. METHODS: In this retrospective database cohort study, we identified children (ages 10-18 years) with neuromuscular scoliosis who underwent elective spinal fusion in 2012-2020 from the National Surgical Quality Improvement Program-Pediatric database. The rates of co-occurring complications within 30 days were calculated, and associated factors were identified by logistic regression analysis. Correlation between a number of complications and outcomes was assessed. RESULTS: Approximately 11% (709/6677 children with neuromuscular scoliosis undergoing spinal fusion had co-occurring complications: 7% experienced two complications and 4% experienced ≥ 3. The most common complication was bleeding/transfusion (80%), which most frequently co-occurred with pneumonia (24%) and reintubation (18%). Surgical time ≥ 400 min (odds ratio (OR) 1.49 [95% confidence interval (CI) 1.25-1.75]), fusion ≥ 13 levels (1.42 [1.13-1.79]), and pelvic fixation (OR 1.21 [1.01, 1.44]) were identified as procedural factors that independently predicted concurrent complications. Clinical risk factors for co-occurring complications included an American Society of Anesthesiologist physical status classification ≥ 3 (1.73 [1.27-2.37]), structural pulmonary/airway abnormalities (1.24 [1.01-1.52]), impaired cognitive status (1.80 [1.41-2.30]), seizure disorder (1.36 [1.12-1.67]), hematologic disorder (1.40 [1.03-1.91], preoperative nutritional support (1.34 [1.08-1.72]), and congenital malformations (1.20 [1.01-1.44]). Preoperative tracheostomy was protective against concurrent complications (0.62 [0.43-0.89]). Significant correlations were found between number of complications and length of stay, non-home discharge, readmissions, and death. CONCLUSION: Longer surgical time (≥ 400 min), fusion ≥ 13 levels and pelvic fixation are surgical risk factors independently associated with co-occurring complications, which were associated with poorer patient outcomes. Recognizing identified nonmodifiable risk factors might also be important for preoperative planning and risk stratification of children with neuromuscular scoliosis requiring spinal fusion. LEVEL OF EVIDENCE: Level IV evidence.

Morphological and ultrastructural investigation of the posterior atlanto-occipital membrane: Comparing children with Chiari malformation type I and controls.

INTRODUCTION: The fibrous posterior atlanto-occipital membrane (PAOM) at the craniocervical junction is typically removed during decompression surgery for Chiari malformation type I (CM-I); however, its importance and ultrastructural architecture have not been investigated in children. We hypothesized that there are structural differences in the PAOM of patients with CM-I and those without. METHODS: In this prospective study, blinded pathological analysis was performed on PAOM specimens from children who had surgery for CM-I and children who had surgery for posterior fossa tumors (controls). Clinical and radiographic data were collected. Statistical analysis included comparisons between the CM-I and control cohorts and correlations with imaging measures. RESULTS: A total of 35 children (mean age at surgery 10.7 years; 94.3% white) with viable specimens for evaluation were enrolled: 24 with CM-I and 11 controls. There were no statistical demographic differences between the two cohorts. Four children had a family history of CM-I and five had a syndromic condition. The cohorts had similar measurements of tonsillar descent, syringomyelia, basion to C2, and condylar-to-C2 vertical axis (all p>0.05). The clival-axial angle was lower in patients with CM-I (138.1 vs. 149.3 degrees, p = 0.016). Morphologically, the PAOM demonstrated statistically higher proportions of disorganized architecture in patients with CM-I (75.0% vs. 36.4%, p = 0.012). There were no differences in PAOM fat, elastin, or collagen percentages overall and no differences in imaging or ultrastructural findings between male and female patients. Posterior fossa volume was lower in children with CM-I (163,234 mm3 vs. 218,305 mm3, p<0.001), a difference that persisted after normalizing for patient height (129.9 vs. 160.9, p = 0.028). CONCLUSIONS: In patients with CM-I, the PAOM demonstrates disorganized architecture compared with that of control patients. This likely represents an anatomic adaptation in the presence of CM-I rather than a pathologic contribution.

Timing of intraoperative neurophysiological monitoring (IONM) recovery and clinical recovery after termination of pediatric spinal deformity surgery due to loss of IONM signals.

BACKGROUND CONTEXT: Intraoperative neurophysiological monitoring (IONM) is used to reduce the risk of spinal cord injury during pediatric spinal deformity surgery. Significant reduction and/or loss of IONM signals without immediate recovery may lead the surgeon to acutely abort the case. The timing of when monitorable signals return remains largely unknown. PURPOSE: The goal of this study was to investigate the correlation between IONM signal loss, clinical examination, and subsequent normalization of IONM signals after aborted pediatric spinal deformity surgery to help determine when it is safe to return to the operating room. STUDY DESIGN/SETTING: This is a multicenter, multidisciplinary, retrospective study of pediatric patients (<18 years old) undergoing spinal deformity surgery whose surgery was aborted due to a significant reduction or loss of IONM potentials. PATIENT SAMPLE: Sixty-six patients less than 18 years old who underwent spinal deformity surgery that was aborted due to IONM signal loss were enrolled into the study. OUTCOME MEASURES: IONM data, operative reports, and clinical examinations were investigated to determine the relationship between IONM loss, clinical examination, recovery of IONM signals, and clinical outcome. METHODS: Information regarding patient demographics, deformity type, clinical history, neurologic and ambulation status, operative details, IONM information (eg, quality of loss [SSEPs, MEPs], laterality, any recovery of signals, etc.), intraoperative wake-up test, postoperative neurologic exam, postoperative imaging, and time to return to the operating were all collected. All factors were analyzed and compared with univariate and multivariate analysis using appropriate statistical analysis. RESULTS: Sixty-six patients were enrolled with a median age of 13 years [IQR 11-14], and the most common sex was female (42/66, 63.6%). Most patients had idiopathic scoliosis (33/66, 50%). The most common causes of IONM loss were screw placement (27/66, 40.9%) followed by rod correction (19/66, 28.8%). All patients had either complete bilateral (39/66, 59.0%), partial bilateral (10/66, 15.2%) or unilateral (17/66, 25.8%) MEP loss leading to termination of the case. Overall, when patients were returned to the operating room 2 weeks postoperatively, nearly 75% (40/55) had monitorable IONM signals. Univariate analysis demonstrated that bilateral SSEP loss (p=.019), bilateral SSEP and MEP loss (p=.022) and delayed clinical neurologic recovery (p=.008) were significantly associated with having unmonitorable IONM signals at repeat surgery. Multivariate regression analysis demonstrated that delayed clinical neurologic recovery (> 72 hours) was significantly associated with unmonitorable IONM signals when returned to the operating room (p=.006). All patients ultimately made a full neurologic recovery. CONCLUSIONS: In children whose spinal deformity surgery was aborted due to intraoperative IONM loss, there was a strong correlation between combined intraoperative SSEP/MEP loss, the magnitude of IONM loss, the timing of clinical recovery, and the time of electrophysiological IONM recovery. The highest likelihood of having a prolonged postoperative neurological deficit and undetectable IONM signals upon return to the OR occurs with bilateral complete loss of SSEPs and MEPs.

The Sensitivity of Limited-Sequence MRI in Identifying Pediatric Cervical Spine Injury: A Western Pediatric Surgery Research Consortium Multicenter Retrospective Cohort Study.

INTRODUCTION: Clinical clearance of a child's cervical spine after trauma is often challenging due to impaired mental status or an unreliable neurologic examination. Magnetic resonance imaging (MRI) is the gold standard for excluding ligamentous injury in children but is constrained by long image acquisition times and frequent need for anesthesia. Limited-sequence MRI (LSMRI) is used in evaluating the evolution of traumatic brain injury and may also be useful for cervical spine clearance while potentially avoiding the need for anesthesia. The purpose of this study was to assess the sensitivity and negative predictive value of LSMRI as compared to gold standard full-sequence MRI as a screening tool to rule out clinically significant ligamentous cervical spine injury. METHODS: We conducted a ten-center, five-year retrospective cohort study (2017-2021) of all children (0-18y) with a cervical spine MRI after blunt trauma. MRI images were re-reviewed by a study pediatric radiologist at each site to determine if the presence of an injury could be identified on limited sequences alone. Unstable cervical spine injury was determined by study neurosurgeon review at each site. RESULTS: We identified 2,663 children less than 18 years of age who underwent an MRI of the cervical spine with 1,008 injuries detected on full-sequence studies. The sensitivity and negative predictive value of LSMRI were both >99% for detecting any injury and 100% for detecting any unstable injury. Young children (age < 5 years) were more likely to be electively intubated or sedated for cervical spine MRI. CONCLUSION: LSMRI is reliably detects clinically significant ligamentous injury in children after blunt trauma. To decrease anesthesia use and minimize MRI time, trauma centers should develop LSMRI screening protocols for children without a reliable neurologic exam. LEVEL OF EVIDENCE: 2 (Diagnostic Tests or Criteria).

Decreased glucocerebrosidase activity in Gaucher disease parallels quantitative enzyme loss due to abnormal interaction with TCP1 and c-Cbl.

Gaucher disease (GD), the most common lysosomal storage disorder of humans, is caused by mutations in the gene coding for the enzyme glucocerebrosidase (GCase). Clinical manifestations vary among patients with the three types of GD, and phenotypic heterogeneity occurs even among patients with identical mutations. To gain insight into why phenotypic heterogeneity occurs in GD, we investigated mechanisms underlying the net loss of GCase catalytic activity in cultured skin fibroblasts derived from patients with the three types of GD. The findings indicate that the loss of catalytic activity of GCase correlates with its quantitative reduction, rather than a decrease in functional capacity of mutant enzyme. Use of a proteasome inhibitor, lactacystin, resulted in increased expression of GCase, suggesting a mechanism of protein degradation in GD. Furthermore, reduced binding of GCase to TCP1 ring complex (TRiC), a regulator of correct protein folding, may result in defective maturation of nascent GCase in GD cells. Additionally, increased interaction between GCase and c-Cbl, an E3 ubiquitin ligase, may be involved in the degradation and loss of GCase in GD. The findings suggest that specific molecular mediators involved in GCase maturation and degradation could be responsible for phenotypic variation among patients with the same genotypes and that these mediators could be therapeutically targeted to increase GCase activity in patients with GD.

Combined transnasal/transoral endoscopic odontoid resection in pediatric patients: Otolaryngologic considerations to airway management, endoscopic exposure, and complication management.

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Younger age at spinal cord detethering is potentially associated with a reduced risk of curve progression in children with early onset scoliosis.

PURPOSE: In children with early onset scoliosis (EOS) who have tethered spinal cord (TSC), spinal cord detethering is commonly performed prior to spinal deformity correction (SDC). The purpose of this study was to investigate whether age or curve magnitude at the time of detethering is associated with curve progression at a follow-up of at least 2 years. It was hypothesized that patients who undergo detethering at a younger age, or those with a smaller curve magnitude, would experience a reduced rate of curve progression when compared with those who are older or with larger curves. METHODS: Patients with EOS who underwent detethering at least 2 years prior to SDC were identified in a multicenter international registry. Radiographs were assessed just prior to the detethering procedure (pre-detether) and at the most recent visit prior to SDC (most recent post-detether). The rate of curve progression > 10° was examined. Owing to unequal follow-up in individual patients, Cox regression was used to investigate associations between primary variables (age and magnitude of major coronal curve) and rate of curve progression. RESULTS: 37 patients met inclusion criteria and 18 (mean age: 3.7 ± 2.9 years, 66.7% female, mean follow-up: 3.4 ± 1.3 years) had radiographic data available for analysis. Pre-detether and most recent post-detether major coronal curves were 44.8° ± 18.5° and 47.6° ± 23.9°, respectively. 5 (27.8%) patients had curve progression > 10° at a follow-up of 3.2 ± 1.2 years. Patients with progression > 10° were older at the time of detethering when compared with those without (5.6 ± 2.8 vs. 3 ± 2.7 years, p = 0.084). Regression analysis demonstrated that as age at detethering increased by 1 year, the rate of curve progression > 10° increased by 28.6% [95% confidence interval (CI) 0.899; 1.839, p = 0.169]. There was no evidence of an association between pre-detethering curve magnitude and rate of curve progression > 10° [HR: 1.027, 95% CI 0.977; 1.079, p = 0.297]. CONCLUSION: In a small multicenter cohort of EOS patients with TSC, younger age, but not curve size, at the time of detethering was associated with a lower rate of scoliosis progression. Although these results indicate a potential role for early spinal cord detethering in the EOS population, they require further prospective investigation with a larger number of patients. LEVEL OF EVIDENCE: Level II.

Hanging and Strangulation Injuries: An Institutional Review From a Level 1 Pediatric Trauma Center.

BACKGROUND: We sought to define the incidence and outcomes of pediatric hanging and strangulation injuries to inform best practices for trauma triage and management. METHODS: A retrospective review was conducted that included all patients who presented after hanging or strangulation to a Level I Pediatric Trauma Center from 2011 through 2021. Patient demographics, injury characteristics, and clinical outcomes were collected. All imaging modalities of the head and neck were reviewed to determine if a bony fracture or vascular injury was present. RESULTS: Over the 11-year study period, 128 patients met inclusion criteria. The median age of the cohort was 13 years [IQR: 8.5-15], most patients were male (60.9%), and the median GCS was 11 [3, 15]. There were 96 cases (75%) that were intentional injuries. 76 patients (59.4%) received imaging in the form of plain radiographs, CT, or MRI of the neck and cervical spine. No fractures were identified and there were 0 clinically significant cervical spine injuries. CT angiograms of the neck identified no cerebral vascular injuries. Mortality was high (32%), and 25% of patients with nonaccidental injuries had a documented prior suicide attempt. CONCLUSION: We identified no cervical spine fractures and no blunt cerebral vascular injuries after a hanging or strangulation in over 10 years at a Level 1 Pediatric Trauma Center. Use of CT and CT angiography of the neck and cervical spine should be minimized in this patient population without high clinical index of suspicion and/or significant mechanism. LEVEL OF EVIDENCE: IV.