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Craniocervical instability (CCI) is increasingly recognized in hereditary disorders of connective tissue and in some patients following suboccipital decompression for Chiari malformation (CMI) or low-lying cerebellar tonsils (LLCT). CCI is characterized by severe headache and neck pain, cervical medullary syndrome, lower cranial nerve deficits, myelopathy, and radiological metrics, for which occipital cervical fusion (OCF) has been advocated. We conducted a retrospective analysis of patients with CCI and Ehlers-Danlos syndrome (EDS) to determine whether the surgical outcomes supported the criteria by which patients were selected for OCF. Fifty-three consecutive subjects diagnosed with EDS, who presented with severe head and neck pain, lower cranial nerve deficits, cervical medullary syndrome, myelopathy, and radiologic findings of CCI, underwent open reduction, stabilization, and OCF. Thirty-two of these patients underwent suboccipital decompression for obstruction of cerebral spinal fluid flow. Questionnaire data and clinical findings were abstracted by a research nurse. Follow-up questionnaires were administered at 5-28 months (mean 15.1). The study group demonstrated significant improvement in headache and neck pain (p < 0.001), decreased use of pain medication (p < 0.0001), and improved Karnofsky Performance Status score (p < 0.001). Statistically significant improvement was also demonstrated for nausea, syncope (p < 0.001), speech difficulties, concentration, vertigo, dizziness, numbness, arm weakness, and fatigue (p = 0.001). The mental fatigue score and orthostatic grading score were improved (p < 0.01). There was no difference in pain improvement between patients with CMI/LLCT and those without. This outcomes analysis of patients with disabling CCI in the setting of EDS demonstrated significant benefits of OCF. The results support the reasonableness of the selection criteria for OCF. We advocate for a multi-center, prospective clinical trial of OCF in this population., (© 2023. The Author(s).)

The management of Chiari I malformation (CMI) is controversial because treatment methods vary and treatment decisions rest on incomplete understanding of its complex symptom patterns, etiologies, and natural history. Validity of studies that attempt to compare treatment of CMI has been limited because of variable terminology and methods used to describe study subjects. The goal of this project was to standardize terminology and methods by developing a comprehensive set of Common Data Elements (CDEs), data definitions, case report forms (CRFs), and outcome measure recommendations for use in CMI clinical research, as part of the CDE project at the National Institute of Neurological Disorders and Stroke (NINDS) of the US National Institutes of Health. A working group, comprising over 30 experts, developed and identified CDEs, template CRFs, data dictionaries, and guidelines to aid investigators starting and conducting CMI clinical research studies. The recommendations were compiled, internally reviewed, and posted online for external public comment. In October 2016, version 1.0 of the CMI CDE recommendations became available on the NINDS CDE website. The recommendations span these domains: Core Demographics/Epidemiology; Presentation/Symptoms; Co-Morbidities/Genetics; Imaging; Treatment; and Outcome. Widespread use of CDEs could facilitate CMI clinical research trial design, data sharing, retrospective analyses, and consistent data sharing between CMI investigators around the world. Updating of CDEs will be necessary to keep them relevant and applicable to evolving research goals for understanding CMI and its treatment., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Background: The peak oxygen uptake (VO 2 peak) test is the gold standard for cardiorespiratory fitness. However, the test is demanding, requiring sophisticated apparatus for measuring the oxygen uptake. Peak work rate (WRpeak) testing is a promising substitute for peak VO 2 peak testing. However, there is a paucity of research conducted to determine reference values (RVs) for WRpeak. Methods: The database from the LowLands fitness registry was used, containing data from health checks from apparently healthy subjects among various professions. Exercise tests were performed using a cycle ergometer and a calibrated metabolic cart. Analysis was executed on 3463 subjects, 2868 male and 595 female. Reference values with corresponding centiles were developed using the LMS method. Results: VO 2 peak and WRpeak where highly associated with Pearson's correlation of 0.9 ( p  = .001). Weight, height, maximum heart rate, and the respiratory exchange ratio had a positive significant effect ( p  < .001) on WRpeak, and age a negative significant effect, in both males and females. The following formulas were computed for RVs: W R p e a k = - 102 + ( 1.5   *   w e i g h t [ k g ] ) + ( 1.9   *   h e i g h t [ c m ] ) - ( 2.0   *   a g e ) - ( s e x   *   60 [ M : 0 ; V : 1 ] ) W R p e a k / k g = 2.45 - ( 0.026   *   w e i g h t [ k g ] ) + ( 0.024   *   h e i g h t [ c m ] ) - ( 0.024   *   a g e ) - ( s e x   *   0.84   [ M : 0 ; V : 1 ] ) Conclusions: WRpeak can be used as a substitute for VO 2 peak when a respiratory gas-analysis system is not available. This study provides RVs for WRpeak and WRpeak/kg in a healthy Dutch/Flemish adult population using cycle ergometry. Further research is needed to obtain RVs for elderly subjects, specific diseases, ethnicities and ergometers.

Objectives: To describe a cranioplasty procedure used in conjunction with foramen magnum decompression (FMD) for the treatment of canine caudal occipital malformation syndrome (COMS), and to evaluate the clinical outcome., Study Design: Prospective clinical study., Animals: Dogs (n=21) with COMS diagnosed by magnetic resonance imaging (MRI)., Methods: After FMD, titanium screws were placed around the perimeter of the foramen magnum defect and a skull plate fashioned from titanium mesh and polymethylmethacrylate was attached to the back of the skull, using the titanium screws as anchor posts. Follow-up was obtained by direct examination by the authors, telephone interviews with owners and referring veterinarians, and a questionnaire sent to owners of surviving dogs designed to assign objective measures of response to surgical intervention. Surgical success was defined as improvement in >or=1 aspects of clinical dysfunction (e.g. scratching, pain) postoperatively. Owner-assigned pre- and postoperative quality-of-life (QOL) scores (1-5) for surviving dogs were compared using a Wilcoxon's signed rank test for paired data (P

Children with atrial septal defects (ASD) have less respiratory compliance (Crs) than normal cohorts. There could be implications for anaesthetic management if these children also have decreased compliance during anaesthesia. To examine the changes in Crs before, during and following surgical correction of the atrial defect, Crs was measured in 29 infants and children, 15 undergoing correction of secundum type atrial septal defects, and a group of 14 children of similar weight undergoing non-thoracic surgery. During sedation, Crs was measured using the single breath technique (SBT) and during anaesthesia, both before and after the surgical procedure, an inflation technique was applied to determine Crs. To investigate the aetiology of the difference in Crs, the pulmonary to systemic flow ratio (Qp:Qs) was determined using echocardiography during sedation in the ASD patients. During sedation, Crs in the ASD group was 52.7 +/- 19.5% less than in the control group. The slope of the line of regression of Crs vs height for the ASD group was significantly less (P less than 0.05) than that of the control group during sedation. However, during anaesthesia, Crs in the ASD group was not significantly different from the control group either before or after surgery. The per cent decrease in Crs during sedation in the children with ASD, in comparison with the control group, did not correlate with the Qp:Qs ratio of the ASD group (r2 = 0.012,NS). We conclude that, in spite of lower Crs during sedation, infants and children with ASD do not have lower Crs during anaesthesia and cardiopulmonary bypass than normal controls undergoing non-thoracic surgery.(ABSTRACT TRUNCATED AT 250 WORDS)