Sinus arrest in a p.Arg160X-DSP-positive patient without evidence of desmoplakin-mediated cardiomyopathy: a case report.

Background: Pathogenic/Likely pathogenic variants in DSP-encoded desmoplakin are strongly associated with arrhythmogenic cardiomyopathy (ACM). However, their contribution towards sinus node dysfunction has not been well-delineated. Case summary: A 74-year-old man with a pathogenic variant of DSP-encoded desmoplakin (c.478C >T; p.Arg160X) but no evidence of ACM presented with one episode of syncope in the setting of a gastrointestinal illness. Workup including echocardiography, cardiac magnetic resonance imaging, and Holter monitor did not show evidence of ACM or significant arrhythmias. One month later, he experienced several closely-spaced episodes of syncope associated with long sinus pauses and sinus arrest documented on telemetry. He underwent urgent dual chamber pacemaker implantation, during which a ventricular programmed stimulation study was performed and was negative for sustained ventricular arrhythmias. His syncopal episodes resolved and he had no recurrent events on three-month follow-up. Discussion: As highlighted here, DSP-encoded desmoplakin pathogenic/Likely pathogenic variants may contribute to isolated sinus node dysfunction. This clinical link should be further explored in larger studies involving patients with DSP variants.

Vertebral artery dissection presenting with multilevel combined sensorimotor radiculopathy: a case report and literature review.

STUDY DESIGN: This study is a case report demonstrating a rare clinical presentation of vertebral artery dissection. We believe that this is the first reported case with multilevel combined sensorimotor radiculopathy. We have also included a literature review. OBJECTIVE: The purpose of this report is to inform the reader of a unique clinical presentation and to draw attention to some of the rare features of extracranial vertebral artery dissection. We believe that this condition is perhaps underrecognized and this paper may help to increase awareness, thereby encouraging prompt investigation, diagnosis, and implementing early treatment. The literature review also includes a brief anatomic and physiologic description of the underlying pathologic process. SUMMARY OF BACKGROUND DATA: Only a small number of similar cases are described in the literature, though most describe a motor deficit at a single root level. Our case included both motor and sensory deficits at more than 1 level and we describe the possible physiologic and anatomic reasons for this. METHODS: The case described is one that presented to our institution and was initially assessed by the first (corresponding) author. The literature review is based on articles identified from a PubMed search on vertebral artery dissection. RESULTS: The clinical and radiologic findings are discussed in the case report. CONCLUSIONS: Peripheral motor deficits are a rare clinical presentation of spontaneous vertebral artery dissection and tend to affect a solitary root level, typically C5. Sensation is usually preserved. We describe a case presenting with multilevel combined sensorimotor radiculopathy, which we believe has not previously been reported.

Perioperative aspects of endoscopic anterior scoliosis surgery: the learning curve for a consecutive series of 100 patients.

The reported benefits of endoscopic versus open scoliosis surgery include improved visualization, a muscle sparing approach, reduced pulmonary morbidity, reduced pain, and improved cosmesis. Some aspects of the surgical learning curve for this technically demanding method have been previously reported; however, improvements in other factors with increasing experience have not been quantified. This paper presents a series of 100 consecutive endoscopic anterior scoliosis corrections performed between April 2000 and February 2006. We report changes in the following perioperative factors with increasing experience; operative set-up time, operative time, x-ray irradiation time, number of instrumented levels, blood loss, intercostal catheter drainage, chest drain removal time, days in intensive care, days to mobilize, days in hospital, and early complications. Statistical comparisons were made between the first 20 (1 to 20), middle 20 (41 to 60), and last 20 (81 to 100) cases. Results showed statistically significant improvements and increased consistency in operative time, operative set-up time, x-ray irradiation time, blood loss, hospital stay, and mobilization time with experience. The complication rate was comparable to other recently published endoscopic studies. In the last 20 cases of the series, operative times had reduced to 35 minutes per level, x-ray irradiation times to 15 seconds per level, and blood loss to 38 mL per level. Most perioperative surgical factors therefore improve significantly with increasing experience in endoscopic anterior scoliosis correction.

Recovery of pulmonary function following endoscopic anterior scoliosis correction: evaluation at 3, 6, 12, and 24 months after surgery.

STUDY DESIGN: A series of patients with scoliosis undergoing endoscopic anterior instrumentation and fusion undertaking repeated pulmonary function assessments. OBJECTIVE: To assess recovery of pulmonary function in the 2 years following endoscopic anterior scoliosis correction. SUMMARY OF BACKGROUND DATA: Recent studies have found that pulmonary function returns to preoperative levels 12-24 months following endoscopic anterior scoliosis correction, and a small improvement in forced expiratory volume (FEV1) has also been reported. METHODS: A series of 44 patients with endoscopic anterior scoliosis correction had pulmonary function tests before surgery, and at 3, 6, 12, and 24 months after surgery. Forced vital capacity (FVC), FEV1, and total lung capacity (TLC) were measured. Nonparametric statistical analysis was used to investigate changes in pulmonary function between successive assessments. RESULTS: Pulmonary function decreased by approximately 10% at 3 months after surgery. At 24 months after surgery, FVC and FEV1 recovered to 5% to 8% higher than preoperative levels, while TLC returned to preoperative levels. Statistically significant improvements in most pulmonary function values occurred between 3 and 6, and 6-12 months. Improvements in mean FVC, FEV1, and TLC continue between 12 and 24 months, although only the increase in absolute FVC for this time is statistically significant. CONCLUSIONS: Endoscopic anterior scoliosis surgery has no lasting negative effect on pulmonary function, and with prolonged follow-up, pulmonary capacity improves beyond preoperative levels.

Variability in Cobb angle measurements using reformatted computerized tomography scans.

STUDY DESIGN: Survey of intraobserver and interobserver measurement variability. OBJECTIVE: To assess the use of reformatted computerized tomography (CT) images for manual measurement of coronal Cobb angles in idiopathic scoliosis. SUMMARY OF BACKGROUND DATA: Cobb angle measurements in idiopathic scoliosis are traditionally made from standing radiographs, whereas CT is often used for assessment of vertebral rotation. Correlating Cobb angles from standing radiographs with vertebral rotations from supine CT is problematic because the geometry of the spine changes significantly from standing to supine positions, and 2 different imaging methods are involved. METHODS: We assessed the use of reformatted thoracolumbar CT images for Cobb angle measurement. Preoperative CT of 12 patients with idiopathic scoliosis were used to generate reformatted coronal images. Five observers measured coronal Cobb angles on 3 occasions from each of the images. Intraobserver and interobserver variability associated with Cobb measurement from reformatted CT scans was assessed and compared with previous studies of measurement variability using plain radiographs. RESULTS: For major curves, 95% confidence intervals for intraobserver and interobserver variability were +/-6.6 degrees and +/-7.7 degrees, respectively. For minor curves, the intervals were +/-7.5 degrees and +/-8.2 degrees, respectively. Intraobserver and interobserver technical error of measurement was 2.4 degrees and 2.7 degrees, with reliability coefficients of 88% and 84%, respectively. There was no correlation between measurement variability and curve severity. CONCLUSIONS: Reformatted CT images may be used for manual measurement of coronal Cobb angles in idiopathic scoliosis with similar variability to manual measurement of plain radiographs.

Regional orthopaedic courses--rationale and practice.

BACKGROUND: The intensity and length of higher surgical training has changed, and with this the need for a more formalised training programme has been recognised. With limited budgets for training and the high cost of national training days, it was proposed that within the Wessex Deanery regional courses should be run to complement the existing weekly training session. METHODS: In 1998, a questionnaire was sent to all the orthopaedic consultants and specialist registrars within the region to identify the training requirements of the specialist registrars and the availability of consultants to organise and teach during these training courses. RESULTS: A regionally published report based on the questionnaire's findings identified these training requirements as indicated by both the specialist registrars and the consultants. This information has been used to organise eight 1-day training courses using the expertise of consultants within the region with more planned in the near future. CONCLUSIONS: Four years following this report, we identified the training requirements within the Wessex region and have successfully run regional courses to meet this requirement.

Preventing methicillin-resistant Staphylococcus aureus infection in joint replacements: a new problem requiring old solutions.

AIM: To analyse prospectively the prevalence of methicillin-resistant Staphylococcus aureus (MRSA) colonisation and infection in wounds and surgical vacuum drains. METHODS: The study group was 100 patients undergoing primary total hip and total knee replacements in a single orthopaedic unit. RESULTS: Two patients had undergone revision joint surgery within the same unit in the year prior to the study due to confirmed MRSA infection. Following a change in the unit's infection control policy, joint replacement surgery continued within the unit even when there were cases of active MRSA present on the same ward. None of the patients undergoing joint replacement grew MRSA in their postoperative drain tips or wound cultures. CONCLUSIONS: It is safe to continue with joint replacement surgery when other patients on the same ward have active MRSA as long as strict infection control measures are followed as outlined.