يعرض 1 - 10 نتائج من 11 نتيجة بحث عن '"FORAMEN magnum"', وقت الاستعلام: 0.98s تنقيح النتائج
  1. 1

    المصدر: Child's Nervous System. 38:1137-1145

    الوصف: To characterize natural history and early changes of craniovertebral junction stenosis in achondroplasia correlating with clinical and radiological outcome.Retrospective measures on craniovertebral junction were performed blindly, on sagittal T2-weighted images, in 21 patients with achondroplasia referred from 2008 to 2020. Clinical and polysomnography data were retrospectively collected. Each patient was paired for age and gender with four controls. Wilcoxon means comparison or Student's t-tests were applied.Twenty-one patients (11 females, from 0.1 to 39 years of age) were analyzed and paired with 84 controls. A craniovertebral junction stenosis was found in 11/21 patients (52.4%), all before the age of 2 years. Despite a significant reduction of the foramen magnum diameter (mean ± SD: patients 13.6 ± 6.2 mm, controls 28.5 ± 4.7 mm, p .001), craniovertebral junction stenosis resulted from the narrowing of C2 dens-opisthion antero-posterior diameter (8.7 ± 3.9 mm vs 24.6 ± 5.1 mm, p .001). Other significant changes were opisthion anterior placement (-0.4 ± 2.8 mm vs 9.4 ± 2.3 mm, p .001), posterior tilt of C2 (46.2 ± 13.7° vs 31.6 ± 7.9°, p .001) and of C1 (15.1 ± 4.3° vs 11.9 ± 5.0°, p = 0.01), and dens thickening (9.4 ± 2.2 mm vs 8.5 ± 2.1 mm, p = 0.03), allowing to define three distinguishable early craniovertebral junction patterns in achondroplasia. All children with C2-opisthion antero-posterior diameter of more than 6 mm had a better clinical and radiological outcome.Craniovertebral junction in achondroplasia results from narrowing between C2 dens and opisthion related to anterior placement of opisthion, thickening of C2 dens, and posterior tilt of C1-C2. A threshold of 6 mm for dens-opisthion sagittal diameter seems to correlate with clinical and radiological outcome.

  2. 2

    المصدر: Child's Nervous System. 35:1769-1776

    الوصف: The role of intraoperative neurophysiological monitoring (IONM) during surgery for Chiari I malformation has not been fully elucidated. Questions remain regarding its utility as an adjunct to foramen magnum decompression surgery, specifically, does IONM improve the safety profile of foramen magnum decompression surgery and can IONM parameters help in intraoperative surgical decision-making. This study aimed to describe a single institution experience of IOM during paediatric Chiari I surgery. The methodology comprised a retrospective review of prospectively collected electronic neurosurgical departmental operative database. Inclusion criteria were children under 16 years of age who had undergone foramen magnum decompression for Chiari I malformation with IONM. In addition to basic demographic data, details pertaining to presenting features and post-operative outcomes were obtained. These included primary symptoms of Chiari I malformation and indications for surgery. MRI findings, including the presence of syringomyelia on pre-and post-operative imaging, were reviewed. Details of the surgical technique for each patient were recorded. Only patients with either serial brainstem auditory evoked potential (BAEP) and/or upper limb somatosensory evoked potential (SSEP) recordings were included. Two time points were used for the purposes of analysing IONM data; initial baseline before skin incision and final at the time of skin closure. Thirty-seven children underwent foramen magnum decompression (FMD) with IONM. Mean age was 10.5 years (range 1–16 years) with a male:female ratio 13:24. The commonest clinical features on presentation included headaches (15) and scoliosis (13). Twenty-four patients had evidence of associated syringomyelia (24/37 = 64.9%). A reduction in the SSEP latency was observed in all patients. SSEP amplitude was more variable, with a decrease seen in 18 patients and an increase observed in 12 patients. BAEP recordings decreased in 13 patients and increased in 4 patients. There were no adverse neurological events following surgery; the primary symptom was resolved or improved in all patients at 3-month follow-up. Resolution or improvement in syringomyelia was observed in 19/24 cases. Our data shows that FMD for Chiari malformation (CM) is associated with changes in SSEPs and BAEPs. However, we did not identify a definite link between clinical outcomes and IONM, nor did syrinx outcome correlate with IONM. There may be a role for IONM in CM surgery but more robust data with better-defined parameters are required to further understand the impact of IONM in CM surgery.

  3. 3

    المصدر: Child's Nervous System. 35:1695-1699

    الوصف: Foramen magnum decompression is a common surgical treatment for Chiari type 1 malformation (CM-1). There are several methods for the intervention to the dura matter such as dural plasty sectioning the dura, also removing the outer membrane of the dura without intradural manipulation. We compared retrospectively our cases with a presentation of illustrative cases. Finally, we add our comments to the worldwide questionnaire which was offered. Twenty-six patients (9 males and 17 females) who underwent surgery from 2010 to 2019 were included. The age distribution was 1 to 55 years old (mean: 16.4 ± 11.3).They were divided into two groups, group A which underwent outer membranectomy and group B which underwent dural plasty. There were 15 cases in group A and 11 cases in group B, and tonsillectomy was performed in 5 cases out of the 11. Symptoms improved in 7 cases (47%) and 5 cases (45%), respectively. Improvement of the syrinx was seen in 14 cases (93%) and 10 cases (90.9%), respectively. No complications were seen in group A; however, one CSF leak and 2 pseudomeningoceles were seen in group B. The number of our cohort is not large enough to analyze statistics, but it seems that outer membranectomy is similar to dural plasty in terms of the results of surgery with a lower risk of complications for the treatment in non-complicated CM-1.

  4. 4

    المصدر: Child's Nervous System. 34:2275-2281

    الوصف: Management of cervicomedullary compression due to foramen magnum stenosis in achondroplasia remains controversial, especially for patients with no symptoms or mild symptoms. We examined the effectiveness of polysomnography (PSG) as an indicator for cervicomedullary decompression treatment. We retrospectively reviewed nine achondroplasia cases (mean age 1 year and 9 months) treated from 2008 to 2015. All patients were examined by PSG, magnetic resonance imaging (MRI), and otolaryngeal fibroscopy. We analyzed demographic data, clinical presentation, degree and type of respiratory impairment, severity of foramen magnum stenosis and concomitant cervicomedullary compression, treatment (conservative or surgical), and clinical outcome. Eight of nine patients presented with no severe symptoms in the daytime. However, MRI revealed four severe, four moderate, and one mild case of cervicomedullary compression, and PSG demonstrated severe sleep apnea in four cases and moderate sleep apnea in five cases. All sleep apnea cases were obstructive or obstructive-dominant. Fibroscopy revealed no upper airway stenosis in six cases and mild stenosis in three cases. Four patients who had severe sleep-related respiratory disturbance on PSG and severe or moderate cervicomedullary compression were treated by cervicomedullary decompression. Three of these patients demonstrated improved sleep respiration soon after surgery, while one required temporary tracheostomy due to bilateral vocal cord paralysis caused by compression during intratracheal intubation. Polysomnography can be a useful indicator for cervicomedullary decompression surgery, especially in cases of seemingly asymptomatic achondroplasia with severe foramen magnum stenosis.

  5. 5

    المصدر: Child's Nervous System. 31:129-133

    الوصف: Achondroplasia is a skeletal dysplasia with diminished growth of the skull base secondary to defective enchondral bone formation. This leads to narrowing of the foramen magnum and jugular foramina, which further leads to ventricular dilatation and prominence of the emissary veins. The primary goal of our study was to determine a correlation between the degree of ventricular dilatation, jugular foramina and foramen magnum narrowing, as well as emissary vein enlargement.Conventional T2-weighted MR images were evaluated for surface area of the foramen magnum and jugular foramina, ventricular dilatation, and emissary veins enlargement in 16 achondroplasia patients and 16 age-matched controls. Ratios were calculated for the individual parameters using median values from age-matched control groups to avoid age as a confounder.Compared to age-matched controls, in children with achondroplasia, the surface area of the foramen magnum (median 0.50 cm(2), range 0.23-1.37 cm(2) vs. 3.14 cm(2), 1.83-6.68 cm(2), p 0.001) and jugular foramina (median 0.02 cm(2), range 0-0.10 cm(2) vs. 0.21 cm(2), 0.03-0.61 cm(2), p 0.001) were smaller, whereas ventricular dilatation (0.28, 0.24-0.4 vs. 0.26, 0.21-0.28, p 0.001) and enlargement of emissary veins (6, 0-11 vs. 0, p 0.001) were higher. Amongst the patients, Spearman correlation and multiple regression analysis did not reveal correlation for severity between the individual parameters.Our study suggests that in children with achondroplasia, (1) the variation in ventricular dilatation may be related to an unquantifiable interdependent relationship of emissary vein enlargement, venous channel narrowing, and foramen magnum compression and (2) stable ventricular size facilitated by interdependent factors likely obviates the need for ventricular shunt placement.

  6. 6

    المصدر: Child's Nervous System. 32:1069-1078

    الوصف: To document long-term morphological changes of Chiari type II malformation (CM-II) following closure of spina bifida manifesta (SBM). We retrospectively evaluated postnatal magnetic resonance images of the CM-II and posterior fossa (PF) in 28 consecutive cases. We measured changes in vertebral level and length of the cerebellar peg (CP), cerebrospinal fluid (CSF) spaces anterior and posterior to the cerebrospinal junction, PF area, and the anteroposterior diameters of the foramen magnum (FM) and C1 vertebra. We examined the morphological differences between the cases with and without ventriculoperitoneal (VP) shunting and derived predicted means by nonlinear mixed-effect modeling. At birth, there were significant differences in CP length, PF area, and FM and C1 diameters between those who underwent VP shunting and those who did not. In cases with a CP below C1, VP shunting was required in every case but one. In those with visible CSF space at birth, VP shunts were not required. In 17 of 18 cases with a CP below C1, the vertebral level ascended by mean two vertebral levels (range 0–5 levels) within 4–6 months of delivery. In the remaining case, slowly progressive hydrocephalus and delayed CP descent required VP shunting at 8 months. Predicted mean CP length and FM and C1 diameters were greater in those who underwent VP shunting, but there was no difference in predicted mean PF area. The morphology of CM-II and the presence of hydrocephalus influence each other in children who have undergone postnatal SBM repair.

  7. 7

    المصدر: Child's Nervous System. 31:743-750

    الوصف: Achondroplasia is the most common form of dwarfism. Respiratory failure is responsible for most deaths among these children and is often related to cervicomedullary compression (CMC). We present our experience with early cervicomedullary decompression in infants with achondroplasia.Data was retrospectively collected for infants with achondroplasia who underwent CMC decompression between 1998 and 2013. Data included presurgical and postsurgical neurological examinations, MRI scans, and sleep study results.Ten infants were included. Ages at surgery were 4 to 23 months (12.5 ± 6.88 months). All infants displayed neurological findings prior to surgery, although often subtle. All infants underwent a foramen magnum opening with a wide C1 laminectomy. Following surgery, seven patients (70 %) demonstrated improved neurological status, and one displayed neurological deterioration. Seven patients demonstrated improved sleep quality 1 year after surgery. These patients had a good or improved neurological status following surgery. Preoperative radiological findings included abnormal hyperintense T2 changes in all children (improved following surgery in six children), brainstem distortion in four children (improved in all), and diminished cerebrospinal fluid (CSF) spaces at the level of the foramen magnum in eight children (improved in seven). One child with extensive preoperative T2 changes accompanied by neurological and respiratory decline, deteriorated following surgery, and remains chronically ventilated.Infants with achondroplasia are prone to neurological and respiratory symptoms. We believe that early diagnosis and early surgery for decompression of the foramen magnum and C1 lamina can alleviate respiratory symptoms, improve neurological status, and perhaps prevent sudden infant death in this population.

  8. 8

    المصدر: Child's Nervous System. 30:613-623

    الوصف: The optimal treatment for Chiari I malformation in children is still under debate. The aim of this study was to evaluate the surgical outcome of the pediatric Chiari I malformation, focusing on clinico-radiological factors and technical aspects. Fifty-six patients with Chiari I malformation who received surgery at Seoul National University Children’s Hospital were included. The mean age was 7.9 years. The patients were divided into three groups: group I (n = 8) with hydrocephalus, group II (n = 11) without syrinx, and group III (n = 37) with syrinx. Group I received shunting operation initially, and others received foramen magnum decompression (FMD). Group III was further subdivided: group IIIa (n = 9), minimal intradural manipulation, and group IIIb (n = 27), active intradural manipulation. The outcomes were compared between the groups. The mean follow-up period was 75.9 months. In group I, symptoms were resolved or had improved in most patients, with only one patient received additional FMD. Symptoms resolved or improved in 10 (91 %) and 25 cases (84 %) in groups II and III, respectively. Syrinx was markedly decreased in 31 cases (86 %) in group III. FMD was less effective for scoliosis (improved or stabilized in 57 %). The persistence of syrinx was related with an aggravation of scoliosis. The outcomes between group IIIa and IIIb showed no significant difference. In most pediatric Chiari I patients with hydrocephalus, a shunting operation was sufficient. FMD showed high efficacy in treating patients without hydrocephalus. The extent of the intradural procedure did not have a significant effect on the clinical outcome.

  9. 9

    المصدر: Child's Nervous System. 24:329-336

    الوصف: Chiari I malformations (CMI) involve pathological hindbrain abnormalities reported to be correlated with a hypoplastic posterior fossa. CMI was traditionally characterized by the downward herniation of the cerebellar tonsils with a descent of 5 mm or more below the foramen magnum. The fullness of the cisterna magna and CSF flow at the level of the cervicomedullary junction have been shown to be more useful in selecting symptomatic patients for surgical decompression. The present study calculates posterior fossa dimension and volume estimates in pediatric patients using magnetic resonance imaging. The combination of neuroradiological and clinical findings is used to re-examine the criteria used for diagnosis and treatment of pediatric CMI patients.A retrospective chart review was conducted on patients who were admitted to the Division of Neurosurgery of the Children's hospital of Eastern Ontario between 1990 and 2007. Clinical and radiological assessments were performed on all patients. Posterior fossa volumes (PFV) and intracranial volumes (ICV) were measured from sagittal head magnetic resonance imaging scans using the Cavalieri method.Sixty-one CMI patients were identified. There were 32 male and 29 female patients with a mean age of 10 years (range: 8 weeks-18 years). Thirty-four (55%) of these patients were symptomatic with scoliosis (38%), suboccipital headaches (29%), and motor/sensory deficits (26%) being the most prominent symptoms. The mean PFV/ICV ratio for all the CMI patients (0.110) was found to be statistically smaller than that of the control patients (0.127, p=0.022). Mean PFV/ICV ratios for asymptomatic and symptomatic CMI patients were found to be similar for children aged 0-9 years (p=0.783) but different for children aged 10-18 years (p=0.018).Mean PFV values were found to be smaller in pediatric CMI patients than control patients; this complements earlier studies in adults and supports the present theory concerning the pathophysiological mechanism of CMI. Subtle morphometric differences among asymptomatic and symptomatic patients aged 0-9 years stress the importance of monitoring asymptomatic patients for the onset of symptoms in their adult years. Symptom development in CMI is likely multifactorial and is much more extensive than the degree of cerebellar tonsillar herniation.

  10. 10

    المصدر: Child's Nervous System. 28:1767-1771

    الوصف: We sought to examine the pre- and postoperative changes of cerebellar tonsillar herniation by MR imaging in asymptomatic pediatric patients with nonsyndromic, single-suture craniosynostosis (N-SSSC), who underwent cranial vault remodeling surgery without suboccipital decompression. We required cerebellar tonsillar herniation through foramen magnum ≥3 mm for Chiari type I malformation (CMI). We hypothesized that the increase of intracranial volume by cranial vault remodeling would correct the asymptomatic CMI.We identified 9 patients among 121 N-SSSC children undergoing craniofacial surgery from January 2004 to October 2010 with CMI. However, two of them were excluded from the study due to missing postoperative MR images. In the final study population, six were males, five were scaphocephalic, while two were diagnosed with coronal synostosis.In four of the cases, the CMI was decreased in postoperative MR imaging varying from 6 to 12 mm. In three cases, the herniation remained stable. The median change of cerebellar tonsillar herniation was -6.5 mm.We conclude that asymptomatic patients with existing CMI may benefit from cranial vault remodeling surgery alone increasing the intracranial volume.