Pediatric Neurosurgery最新文献

Introduction: Dysregulation of spinal cord development can lead to serious neuronal damage and dysfunction, causing significant health problems in newborns. MiRNA-138 appears to be crucial for proliferation, differentiation, and apoptosis of cells. However, the regulation of miRNA-138 and downstream molecules in embryonic spinal cord development remain elusive. The aim of this experiment is to determine whether overexpression of miRNA-138 or RNA interference (RNAi) can regulate the development of spinal cord in fetal rats.

Methods: Two plasmid vectors including pLenti-III-mico-GFP (miRNA-138 open reading frame [ORF]) and pLenti-III-miR-Off (miRNA-138 short hairpin) were constructed and injected into the tail vein of rats on the 14th day of pregnancy. Hematoxylin-eosin (HE) staining was used to observe the cell morphology. QRT-PCR, Western blot, and immunostaining confirmed the regulatory relationship between miRNA-138 and downstream molecules sonic hedgehog (Shh).

Results: Overexpression of miRNA-138 increased neuron regeneration significantly and decreased neuronal apoptosis when compared with the control. Silencing of miRNA-138 increased neuronal apoptosis and spinal cord atrophy significantly. Furthermore, miRNA-138 ORF treatment effectively increased the expression level of miRNA-138 and also upregulated the level of Shh. Comparatively, knockdown of miRNA-138 downregulated Shh levels in myelodysplastic regions.

Conclusion: These findings indicated that miRNA-138 overexpression could protect the spinal cord development of fetal rats, and the underlying mechanisms were associated with Shh expression. The present study provides a novel strategy to promote the molecular mechanism of embryonic spinal cord development.

Introduction: Pediatric meningiomas (PMs) are rare tumors; they differ from their adult counterparts by their atypicality of location, higher rates of malignant change, male preponderance, recurrence, and sometimes, their association with neurofibromatosis. This case series analyzes the clinical behavior, pathological presentation, location, and its association with neurofibromatosis type 2 (NF2).

Methods: This case series consists of pediatric patients between the ages of 4 and 16 years who were hospitalized in the neurosurgical department of our hospital from 2012 to 2021 with different neurological symptoms and a literature review using the PubMed/MEDLINE database.

Results: Sixty percent of the patients were males, while 40% were females. The most common neurological manifestations were signs of increased intracranial pressure. NF2 was absent in all patients. The predominant histopathology subtypes are atypical and WHO grade II, representing 30% and 40%, respectively.

Conclusion: This study supports the relationship between NF2 and pediatric cerebral meningioma but at a lower concomitant rate from 0 to 13%, taking into consideration our original data and the literature review, contrasting some reported cases, which suggest rates as high as 33%, 50%, and 100% in a very small number of patients. Gross total resection without postoperative radiation therapy for nonmalignant and non-NF2-associated PM proved to be a sufficient and a good treatment option.

Introduction: The reported prevalence of tethered spinal cord in patients with anorectal malformations (ARMs) ranges from 9% to 64%. Practice patterns surrounding the diagnosis and management of tethered cord (TC) are suspected to vary, with consideration to the type of spine imaging, adjunct imaging modalities, what patients are offered surgical intervention, and how patients are followed after detethering. We sought to determine what consensus, if any, exists among pediatric neurosurgeons in the USA in terms of diagnosis and management of TC and, specifically, patients with TC and ARM.

Methods: A survey was sent to members of the American Society of Pediatric Neurosurgeons (ASPN). Members of the ASPN received an email with a link to an anonymous REDCap survey that asked about their experience with detethering procedures, indications for surgery, diagnostic tools used, and follow-up protocols.

Results: The survey was completed by 93 of the 192 ASPN members (48%). When respondents were asked about the total number of all simple filum detetherings they performed annually, 61% (N = 57) indicated they perform less than 10 for all TC patients. Ninety-three percent (N = 87) of neurosurgeons performed these procedures in patients with simple filum TC and ARM patients (TC + ARM) specifically. When asked about prophylactic detethering in those with a confirmed diagnosis of low-lying conus and with a filum fatty terminale, 59.1% (N = 55) indicated they would offer this to TC + ARM patients regardless of their age. Regarding preoperative workup for simple filum detethering, all respondents indicated they would order an MRI in both TC and TC + ARM patients, with a minority also requiring additional testing such as urodynamics, neurodevelopmental assessments, and anorectal manometry for both groups. When following patients postoperatively, almost all respondents indicated they would require clinical neurosurgical follow-up with a clinic visit (100% in all simple filum TC patients, 98.9% in fatty filum/low-lying conus TC + ARM patients), but there was wide variation in the use of other tools such as urological testing, neurodevelopmental assessment, and anorectal manometry.

Discussion/conclusions: A wide variety of diagnostic criteria and indication for procedural intervention exists for management of TC patients with and without ARM. Further studies are needed to determine outcomes. Prospective protocols need to be developed and evaluated to standardize care for this patient population and determine best practices.

Background: Obese patients with adolescent idiopathic scoliosis (AIS) have been shown to present with larger curve magnitudes preoperatively. However, the effect of obesity on shoulder balance in AIS remains unknown. The purpose of our study was to determine if overweight and obese patients with AIS have worse radiographic shoulder balance on initial presentation when compared with normal weight patients.

Methods: AIS patients <18 years old, with Lenke 1 or 2 curves, who underwent a posterior spinal fusion between March 2013 and December 2018 were retrospectively evaluated. BMI-for-age percentiles as defined by the Center for Disease Control and Prevention were used: obese (≥95th percentile), overweight (85th to <95th percentile), and normal weight (5th to <85th percentile). Shoulder height was measured via the radiographic shoulder height (RSH) method, with an RSH ≤ 1 cm considered balanced. The primary outcome was preoperative shoulder balance. Secondary outcomes included postoperative shoulder balance, major curve correction, and UIV selection.

Results: One hundred eighty-four patients (116 [63%] normal weight and 68 [37%] overweight/obese) were included. The mean age at surgery was 13.1 ± 2 years, and mean follow-up was 17.4 ± 13 months. Preoperative shoulder imbalance was significantly greater in the overweight/obese group compared to the normal weight group (1.9 ± 1 cm vs. 1.5 ± 1 cm, p = 0.04). The odds ratio of presenting with unbalanced shoulders was 2.0 (95% CI: 1.02-3.83, p = 0.04) for the overweight/obese group. No significant differences were found for postoperative shoulder balance, UIV selection, or major curve correction.

Conclusions: Overweight and obese patients with AIS are twice as likely to present with unbalanced shoulders preoperatively; however, this difference is not clinically relevant with a mean difference of 0.4 cm between cohorts. Finally, the preoperative BMI percentile did not show a significant effect on the chosen UIV or curve magnitude correction.

Level of evidence: Level III: this is a retrospective case-control study.

Introduction: The literature indicates that decompression of Chiari I malformations (CM-1) may resolve symptoms of sleep apnea. This study aims to identify the incidence of obstructive sleep apnea (OSA), central sleep apnea (CSA), and mixed sleep apnea in a cohort of pediatric CM-1 patients treated at our institution. We also assessed apnea-hypopnea index and symptomatology before and after surgery to investigate if Chiari decompression is a viable treatment for sleep apnea in CM-1 patients. Improvement relative to ENT surgical intervention was also considered.

Methods: We identified 75 patients who underwent polysomnography (PSG) from our database of 465 CM-1 patients. Sleep apnea diagnosis was based on the sleep physician's overall interpretation of the PSG. Symptomatology pre- and post-surgery was analyzed.

Results: Of the 75 CM-1 patients that underwent PSG, 23 were diagnosed with sleep apnea. Sixteen had OSA, 6 had CSA, and 1 had mixed apnea. Twelve OSA patients received ENT intervention. Eight improved and 2 further improved after Chiari decompression. Of the 4 patients that did not improve, one of those later improved following Chiari decompression. Of the 6 CSA patients, 2 underwent Chiari decompression, but only one improved. The mixed apnea patient underwent several ENT interventions that did not relieve symptoms but improved following Chiari decompression.

Discussion/conclusions: Based on our results, sleep apnea in CM-1 patients may be obstructive, central, or mixed and is likely multifactorial. A multidisciplinary approach to the management of these patients is important, including neurosurgery, otolaryngology, and sleep medicine. Future prospective studies will lend further insight into this condition and its management.