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Background: The treatment of type 1 Chiari malformation (CM-1) with posterior fossa decompression without (PFD) or with duraplasty (PFDD) is controversial. The authors analyze both options in a national sample of pediatric patients. Methods: Utilizing the Kids' Inpatient Database, CM-1 patients undergoing PFD or PFDD from 2000 through 2009 were analyzed. Results: 1,593 patients with PFD and 1,056 with PFDD were evaluated. The average age was 10.3 years, slightly younger in PFD (9.8 vs. 10.9 years, p = 0.001). PFDD patients were more likely White (81.2 vs 75.6%, p = 0.04) and less likely admitted emergently (8.4 vs. 13.8%, p = 0.007). They also underwent more reoperations (2.1 vs. 0.7%, p = 0.01), had more procedure-related complications (2.3 vs. 0.8%, p = 0.003), a longer length of stay (4.4 vs. 3.8 days, p = 0.001) and higher charges (USD 35,321 vs. 31,483, p = 0.01). Conclusions: This large national study indicates that PFDD is performed more often in Caucasians, less so emergently, and associated with significantly more complications and immediate reoperations, while PFD is more frequent in those with syringomyelia and more economical, requiring fewer hospital resources. Overall, PFD is more favorable for CM-1, though it would be prudent to conduct a prospective trial, as this analysis is limited by data on preoperative presentations and long-term outcomes.

Objective Our study aims to prospectively compare an autologous duraplasty in situ technique (IS group) with the synthetic dural graft duraplasty (SDG group) to clarify the effectiveness and superiority of the former in the treatment of patients with Chiari malformation type 1 (CM-I). Method 29 patients with CM-I were randomly assigned to either IS or SDG group. In both groups, a dissection from the occipital bone was performed. All procedures were performed by the same surgeon. The two duraplasty methods were compared in terms of surgical factors and complications. Data analysis was done for the baseline material, the neurological outcome and MRI-documented syrinx size at the 6 month follow-up. Result 29 patients were enrolled in this study, 14 in the IS group and 15 in the SDG group. The results showed no significant difference in operation time ( P  = 0.916), amount of bleeding ( P  = 0.120), operation complications, hospitalization time ( P  = 0.854) and prognosis between the two groups. The hospitalization cost of IS group was 15,125 yuan less than that of SDG group ( P  < 0.05). Conclusion The autogenous duraplasty in situ technique is a novel, simple, effective and economical surgical management for patients with CM-I.

There has been a transition to using patient-reported outcome instruments (PROi) to assess surgical effectiveness. However, none of these instruments have been validated for outcomes of adult Chiari I malformation (CMI). The aim of this study was to determine the relative validity and responsiveness of various PROi in measuring outcomes after surgery for CMI. Fifty patients undergoing suboccipital craniotomy for adult CMI were prospectively followed for 1 year. Baseline and 1-year patient-reported outcomes (visual analog scale for head pain and visual analog scale for neck pain, Neck Disability Index [NDI], Headache Disability Index, SF-12, Zung Self-Rating Depression Scale, and EuroQol-5D [EQ-5D]) were assessed. A level of improvement in general health after surgery was defined as meaningful improvement. Receiver-operating characteristic curves were generated to assess the validity of PROi to discriminate between meaningful improvement and not. The difference between standardized response means (SRMs) in patients reporting meaningful improvement vs not as calculated to determine the relative responsiveness of each outcome instrument. For pain and disability, the NDI was the most accurate discriminator of meaningful effectiveness (area under the curve: 0.90) and also most responsive to postoperative improvement (standardized response means difference: 1.87). For general health and quality of life, the SF-12 PCS, EQ-5D, and Zung Self-Rating Depression Scale were all accurate discriminators; however, SF-12 Physical Component Scale (SF-12 PCS) and EQ-5D were most accurate. SF-12 PCS was also most responsive. For pain and disability, NDI is the most valid and responsive measure of improvement after surgery for CMI. For health-related quality of life, SF-12 PCS and EQ-5D are the most valid and responsive measures. NDI with SF-12 or EQ-5D is the most valid in patients with CMI and should be considered in cost-effectiveness studies.

Symptomatic CM1, also called the Chiari syndrome, is characterized mainly by headache but also by numbness or weakness in the hands or feet and visual, hearing, balance, and cranial-nerve abnormalities. It is treated surgically.

Introduction Chiari malformation type I (CM-I) is a congenital hindbrain anomaly that requires surgical decompression in symptomatic patients. Posterior fossa decompression with duraplasty (PFDD) has been widely practiced in Chiari decompression, but dural opening carries a high risk of surgical complications. A minimally invasive technique, dural splitting decompression (DSD), preserves the inner layer of the dura without dural opening and duraplasty, potentially reducing surgical complications, length of operative time and hospital stay, and cost. If DSD is non-inferior to PFDD in terms of clinical improvement, DSD could be an alternative treatment modality for CM-I. So far, no randomised study of surgical treatment of CM-I has been reported. This study aims to evaluate if DSD is an effective, safe and cost-saving treatment modality for adult CM-I patients, and may provide evidence for using the minimally invasive procedure extensively. Methods and analysis DECMI is a randomised controlled, single-masked, non-inferiority, single centre clinical trial. Participants meeting the criteria will be randomised to the DSD group and the PFDD group in a 1:1 ratio. The primary outcome is the rate of clinical improvement, which is defined as the complete resolution or partial improvement of the presenting symptoms/signs. The secondary outcomes consist of the incidence of syrinx reduction, postoperative morbidity rates, reoperation rate, quality of life (QoL) and healthcare resource utilisation. A total of 160 patients will be included and followed up at 3 and 12 months postoperatively. Ethics and dissemination The study protocol was approved by the Biological and Medical Ethics Committee of West China Hospital. The findings of this trial will be published in a peer-reviewed scientific journal and presented at scientific conferences. Trial registration number ChiCTR-TRC-14004099.

Purpose In patient with Chiari type I malformations (CM1), indication for surgery can be difficult to establish. Headaches are a common complaint. Factors that predict headache relief have not been clearly identified. Several studies have aimed to examine cerebrospinal fluid (CSF) hydrodynamics in patients with CM1 by using phase-contrast MRI (pcMRI), which is currently the only non-invasive method for assessing craniospinal hydrodynamics and hemodynamics. People with CM1 present alterations in cerebrospinal fluid (CSF) and cerebral blood dynamics. The objective of the present study was to identify hydrodynamic criteria that are predictive of positive clinical outcome (headache relief) after posterior fossa decompression surgery in patient with CM1. Method 41 patients who underwent posterior fossa decompression surgery at Amiens-Picardie University Hospital (Amiens, France) between 2016 and 2021 were retrospectively included. We used preoperative pcMRI to analyze CSF dynamics. Stroke volumes of cerebrospinal fluid were quantified at the aqueduct of Sylvius (SV aqu ), subarachnoid spaces near to C2-C3 (SV C2C3 ) vertebral junction, prepontine cisterns, foramen magnum, and brainstem. CSF pulsatility was analyzed in relation to whether patients reported postoperative headache relief. Statistical analyses were based on Student's t-test. Results 12 patients reported headache relief. The mean SV aqu was significantly higher in patients with headache relief than in those without relief (65 and 32.13µL/CC, p ≤ 0.05). The mean SV C2-C3 was significantly lower in patients with headache relief than in patients without relief (484.58 and 612.94µL/CC, p ≤ 0.05). The two groups of patients did not differ significantly in terms of the area of the narrowest part of the aqueduct of Sylvius or the Evans index. Conclusion SVaqu may have prognostic value for headache relief following surgery for CM1. Further investigation is warranted. This association is likely related to the recruitment of intraventricular pulsatility, which may help regulate potential intracranial pressure changes. Notably, this pulsatility does not appear to be linked to morphological features.

Fibrous dysplasia is a benign bone disease characterized by the replacement of normal bone tissue with fibrous tissue, resulting in irregular bone structure. Cases of craniofacial fibrous dysplasia in children associated with Chiari type I malformation and syringomyelia are extremely rare. This case illustrates the complex clinical manifestations of craniofacial fibrous dysplasia along with Chiari type I malformation and syringomyelia, in which surgical intervention significantly improved the prognosis, and follow-up revealed near-complete resolution of the syringomyelia. It offers valuable insights for managing similar cases in the future.

Purpose Spontaneous regression of Chiari malformation type 2 (CM2) is observed rarely, as CM2 is associated with meningomyelocele (MMC) that is surgically repaired either pre- or early postnatally. While the radiological regression of CM2 occurs frequently following prenatal repair of MMC, it has been reported in only a few studies after postnatal repair. Methods From the consecutive series of children with postnatally repaired MMC, we reviewed the clinical and radiological data regarding CM2, particularly its regression either spontaneously or following CSF diversion. Results Eighteen children underwent postnatal repair of MMC between February 2011 and April 2023. CM2 was present in 16 (89%), and hydrocephalus in 15 children (83%), requiring shunting in 14 of them. During the mean clinical observation time (from birth to April 2023) of 59 ± 51 months, three children with CM2 (19%) underwent 1–2 foramen magnum decompressions (FMD), five children (28%) 1–4 surgical untethering procedures and 13 children with shunted hydrocephalus (93%) 1–5 shunt revisions. Out of sixteen children with CM2, we observed regression of CM2 on MRI in only one case (6%) during the mean radiological follow-up (from birth to the last MRI taken) of 49 ± 51 months. Conclusion In our experience, spontaneous regression of CM2 in children with postnatally repaired MMC occurs quite rarely. Pathophysiological mechanisms behind the development of CM2 in children with MMC remain unclear, but our observation supports the hypothesis of an association between the downward displacement of the hindbrain and the low intraspinal pressure secondary to CSF leakage in children born with MMC.

BACKGROUND:To develop evidence-based treatment guidelines for Chiari malformation type 1 (CM-1), preoperative prognostic indices capable of stratifying patients for comparative trials are needed. OBJECTIVE:To develop a preoperative Chiari Severity Index (CSI) integrating the clinical and neuroimaging features most predictive of long-term patient-defined improvement in quality of life (QOL) after CM-1 surgery. METHODS:We recorded preoperative clinical (eg, headaches, myelopathic symptoms) and neuroimaging (eg, syrinx size, tonsillar descent) characteristics. Brief follow-up surveys were administered to assess overall patient-defined improvement in QOL. We used sequential sequestration to develop clinical and neuroimaging grading systems and conjunctive consolidation to integrate these indices to form the CSI. We evaluated statistical significance using the Cochran-Armitage test and discrimination using the C statistic. RESULTS:Our sample included 158 patients. Sequential sequestration identified headache characteristics and myelopathic symptoms as the most impactful clinical parameters, producing a clinical grading system with improvement rates ranging from 81% (grade 1) to 58% (grade 3) (P = .01). Based on sequential sequestration, the neuroimaging grading system included only the presence (55% improvement) or absence (74% improvement) of a syrinx ≥6 mm (P = .049). Integrating the clinical and neuroimaging indices, improvement rates for the CSI ranged from 83% (grade 1) to 45% (grade 3) (P = .002). The combined CSI had moderately better discrimination (c = 0.66) than the clinical (c = 0.62) or neuroimaging (c = 0.58) systems alone. CONCLUSION:Integrating clinical and neuroimaging characteristics, the CSI is a novel tool that predicts patient-defined improvement after CM-1 surgery. The CSI may aid preoperative counseling and stratify patients in comparative effectiveness trials. ABBREVIATIONS:CM-1, Chiari malformation type 1CSI, Chiari Severity IndexQOL, quality of life

Background Chiari malformation type III (CM III), a rare hindbrain anomaly, often presents with various concurrent anomalies. This paper reports a unique case of CM III associated with Klippel-Feil syndrome (KFS), a condition previously unreported in Saudi Arabia and documented in only one other case globally in Turkey. This study aims to share insights into the unusual association between CM III and KFS, considering their close embryological development and involvement in the craniocervical junction. Methodology The study presents a case of a 2.5-year-old female diagnosed with CM III and KFS. Diagnostic tools such as ultrasound, CT scans, MRI, and physical examinations were used to confirm the patient’s condition. Surgical interventions, including decompression and encephalocele repair, were performed. Results Successful surgical interventions, including encephalocele repair and duraplasty, were carried out. Follow-up visits indicated a stable condition, marked improvement in lower limb strength, and the patient’s ability to walk with assistance. CT follow-up affirmed a satisfactory surgical outcome. Conclusion This case study illustrates the potential for an optimistic prognosis in CM III, even when accompanied by complex conditions such as KFS, through early diagnosis and intervention. It underscores the significance of antenatal screening for effective care planning and calls for further research and publications due to the rarity of this association. These findings contribute to our understanding of CM III and its related conditions, emphasizing the need for open-minded consideration of potential embryological associations.