Australasian Malignant PLeural Effusion (AMPLE)-4 trial: study protocol for a multi-centre randomised trial of topical antibiotics prophylaxis for infections of indwelling pleural catheters.

BACKGROUND: Malignant pleural effusion (MPE) is a debilitating condition as it commonly causes disabling breathlessness and impairs quality of life (QoL). Indwelling pleural catheter (IPC) offers an effective alternative for the management of MPE. However, IPC-related infections remain a significant concern and there are currently no long-term strategies for their prevention. The Australasian Malignant PLeural Effusion (AMPLE)-4 trial is a multicentre randomised trial that evaluates the use of topical mupirocin prophylaxis (vs no mupirocin) to reduce catheter-related infections in patients with MPE treated with an IPC. METHODS: A pragmatic, multi-centre, open-labelled, randomised trial. Eligible patients with MPE and an IPC will be randomised 1:1 to either regular topical mupirocin prophylaxis or no mupirocin (standard care). For the interventional arm, topical mupirocin will be applied around the IPC exit-site after each drainage, at least twice weekly. Weekly follow-up via phone calls or in person will be conducted for up to 6 months. The primary outcome is the percentage of patients who develop an IPC-related (pleural, skin, or tract) infection between the time of catheter insertion and end of follow-up period. Secondary outcomes include analyses of infection (types and episodes), hospitalisation days, health economics, adverse events, and survival. Subject to interim analyses, the trial will recruit up to 418 participants. DISCUSSION: Results from this trial will determine the efficacy of mupirocin prophylaxis in patients who require IPC for MPE. It will provide data on infection rates, microbiology, and potentially infection pathways associated with IPC-related infections. ETHICS AND DISSEMINATION: Sir Charles Gairdner and Osborne Park Health Care Group Human Research Ethics Committee has approved the study (RGS0000005920). Results will be published in peer-reviewed journals and presented at scientific conferences. TRIAL REGISTRATION: Australia New Zealand Clinical Trial Registry ACTRN12623000253606. Registered on 9 March 2023.

Anti-synthetase syndrome associated interstitial lung disease after combination dual immune checkpoint inhibition.

Immune checkpoint inhibitors (ICIs) unleash potent anti-tumour responses but with frequent off-target immune-mediated adverse events (irAE). ICIs can induce a spectrum of rheumatologic manifestations including inflammatory arthritis, Sjögren's syndrome, scleroderma and systemic lupus erythematosus. Here, we describe a case of antisynthetase syndrome associated interstitial lung disease (ILD) following dual Programmed Cell Death 1 and Cytotoxic T Lymphocyte-Associated Protein 4 checkpoint inhibition in a patient with metastatic melanoma. Initial treatment course was complicated by a number of irAEs including pneumonitis, colitis and thyroiditis. Suspicion of an underlying systemic rheumatic disease was heightened by the severe, relapsing and fibrosing nature of the interstitial pneumonitis. A diagnosis of amyopathic antisynthetase syndrome was made upon detection of circulating aminoacyl-tRNA synthetase (anti-EJ) autoantibodies. Intensification of induction immunosuppression followed by maintenance mycophenolate, prednisone and monthly intravenous immunoglobulin achieved long-term disease control. Detection of de novo ICI-induced inflammatory myositis ILD requires a high index of suspicion and carries important prognostic and treatment implications.

Australasian Malignant PLeural Effusion (AMPLE)-3 trial: study protocol for a multi-centre randomised study comparing indwelling pleural catheter (±talc pleurodesis) versus video-assisted thoracoscopic surgery for management of malignant pleural effusion.

INTRODUCTION: Malignant pleural effusions (MPEs) are common. MPE causes significant breathlessness and impairs quality of life. Indwelling pleural catheters (IPC) allow ambulatory drainage and reduce hospital days and re-intervention rates when compared to standard talc slurry pleurodesis. Daily drainage accelerates pleurodesis, and talc instillation via the IPC has been proven feasible and safe. Surgical pleurodesis via video-assisted thoracoscopic surgery (VATS) is considered a one-off intervention for MPE and is often recommended to patients who are fit for surgery. The AMPLE-3 trial is the first randomised trial to compare IPC (±talc pleurodesis) and VATS pleurodesis in those who are fit for surgery. METHODS AND ANALYSIS: A multi-centre, open-labelled randomised trial of patients with symptomatic MPE, expected survival of ≥ 6 months and good performance status randomised 1:1 to either IPC or VATS pleurodesis. Participant randomisation will be minimised for (i) cancer type (mesothelioma vs non-mesothelioma); (ii) previous pleurodesis (vs not); and (iii) trapped lung, if known (vs not). Primary outcome is the need for further ipsilateral pleural interventions over 12 months or until death, if sooner. Secondary outcomes include days in hospital, quality of life (QoL) measures, physical activity levels, safety profile, health economics, adverse events, and survival. The trial will recruit 158 participants who will be followed up for 12 months. ETHICS AND DISSEMINATION: Sir Charles Gairdner and Osborne Park Health Care Group (HREC) has approved the study (reference: RGS356). Results will be published in peer-reviewed journals and presented at scientific meetings. DISCUSSION: Both IPC and VATS are commonly used procedures for MPE. The AMPLE-3 trial will provide data to help define the merits and shortcomings of these procedures and inform future clinical care algorithms. TRIAL REGISTRATION: Australia New Zealand Clinical Trial Registry ACTRN12618001013257 . Registered on 18 June 2018. PROTOCOL VERSION: Version 3.00/4.02.19.

A Case of Portal Vein Thrombosis in a Patient With Methylenetetrahydrofolate Reductase A1298C Polymorphism.

Portal vein thrombosis (PVT) is a prothrombotic state caused by blood flow stasis, vascular injury, and/or hypercoagulability, resulting in partial or complete occlusion of the portal vein. PVT is a rare diagnosis, particularly among those without liver disease. Typical risk factors for PVT include cirrhosis, hepatocellular carcinoma, myeloproliferative neoplasms, other malignancies, oral contraceptive use, bowel infections, and inherited hypercoagulable disorders. The goal of this study is to analyze a case of PVT in a patient in which no clear etiology could be identified and to evaluate whether the patient's methylenetetrahydrofolate reductase (MTHFR) polymorphism may have been a risk factor. This is a case of a 44-year-old female with a history of irritable bowel syndrome, hypertension, hyperlipidemia, sleep apnea, gastric bypass surgery, and MTHFR polymorphism who presented to a walk-in clinic with five days of severe abdominal pain associated with diarrhea, nausea, and anorexia. Hypertension and tenderness over the right lower quadrant prompted a referral to the emergency department for evaluation of possible appendicitis. A contrasted computerized tomography (CT) scan of the abdomen and pelvis revealed a normal appendix and acute portal vein thrombosis. She was then admitted for treatment with intravenous (IV) heparin, fluids, and pain management. After an uneventful three-day hospital course, the patient was discharged on rivaroxaban with a plan to continue anticoagulation therapy for six months and follow up with a hematologist, who later confirmed the patient did not have any inherited hypercoagulable disorders. It is unclear whether the patient's MTHFR polymorphism prompted her PVT as existing data on MTHFR's effects are limited and conflicting. One cannot conclude that MTHFR caused a state of hyperhomocysteinemia to prompt hypercoagulability, as this has not been consistently proven in current literature, and the patient's homocysteine levels were not measured at the time of diagnosis. This case illustrates that further research on the various MTHFR polymorphisms and their effects on coagulation, potentially via homocysteinemia, is warranted. Further research on the MTHFR polymorphisms may help determine whether providers should test for MTHFR in the evaluation of thrombotic risk factors and may help optimize the treatment of thrombotic events for affected individuals.

Performance of myositis-specific antibodies detected on myositis line immunoassay to diagnose and sub-classify patients with suspected idiopathic inflammatory myopathy, a retrospective records-based review.

AIM: To evaluate myositis line immunoassay (LIA) for diagnosis and sub-classification of suspected idiopathic inflammatory myopathy (IIM). To investigate if test performance is improved by increasing signal strength cut-off for myositis-specific antibody (MSA) or combining MSA with indirect immunofluorescence (IIF). METHODS: A retrospective, consecutive case series of patients investigated for MSAs from June 2013 to June 2020 for suspected IIM. Specificity, sensitivity, positive predictive value, and negative predictive value were calculated with 95% confidence intervals for diagnosis of IIM. Association of IIM diagnosis with increased signal strength and presence of an expected IIF pattern on Hep-2 cells was assessed by Fisher's exact test in MSA-positive patients. RESULTS: A total of 195 patients were evaluated. IIM was diagnosed in 32/195 (16.4%) patients. MSAs were detected in 41/195 (21%) patients, 18/41 (43.9%) patients with an MSA had a diagnosis of IIM. The probability of an IIM diagnosis was increased in MSA-positive patients with high compared with low signal strength (83.3% vs 43.5%; P = 0.01) and an expected compared with unexpected IIF pattern (61.5% vs 23.8%; P = 0.04). Specificity for IIM was not significantly improved by increasing signal strength cut-off (85.9% vs 93.8%). Positive predictive value of myositis LIA was only modest and not significantly improved by either increasing signal strength cut-off or requiring an expected IIF pattern for determination of MSA positivity (43.9% vs 60% vs 61.5%). Sub-classification of IIM correlated closely for respective MSAs (88.9%). CONCLUSION: Increased MSA signal strength on myositis LIA and the presence of an expected IIF pattern were associated with IIM diagnosis. Test performance was non-significantly improved by these methods. Prevalence of IIM in this patient cohort was low; it is not excluded that LIA performance could be improved by these methods in a higher prevalence cohort.

Routine use of the Bath Adolescent Pain Questionnaire in a paediatric pain clinic.

INTRODUCTION: Assessment of outcomes from health interventions are of increasing importance, primarily to identify effective and safe treatment, but also to justify funding decisions. The Bath Adolescent Pain Questionnaire (BAPQ) is a self-report questionnaire, validated in 11-18 year olds, assessing the impact of pain in multiple domains of adolescent life. The similarly validated Bath Adolescent Pain Questionnaire for Parents (BAPQ-P) uses the same domains as the BAPQ, assessing the functioning and development of the adolescent from the parents' perspective. METHODS: We conducted a prospective study, planning to routinely collect BAPQ/BAPQ-P data at initial assessment and 6 months later. All patients aged between 5 and 19 attending our chronic pain clinic for the first time between December 2009 and December 2014 were mailed BAPQ and BAPQ-P questionnaires before the first appointment and 6 months after the first appointment. RESULTS: In total, 376 of 386 families returned questionnaires at time 0 and 96 after 6 months, 26% of those responded at time 0. We found statistically significant differences on patients' BAPQ questionnaires from 0 to 6 months showing improvement in all domains. A different result was found on parents' questionnaires where we only found a statistically significant difference on daily and emotional functioning. When comparing patient and parent questionnaires at 0 and 6 months, we found statistically significant differences between patients' and parents' questionnaires in the daily functioning and development domains. CONCLUSION: We believe BAPQ and BAPQ-P measurement proved useful tools to assess response to pain management input in adolescents over a 6-month period. Our experience and results suggest that these tools can, with appropriate administrative support, be used in routine clinical practice to assess patient outcomes. We also believe that BAPQ and BAPQ-P measurements have a utility to audit pain clinic activity and potentially a use in demonstrating beneficial outcomes to commissioners.

Associations Between Systolic Interarm Differences in Blood Pressure and Cardiovascular Disease Outcomes and Mortality: Individual Participant Data Meta-Analysis, Development and Validation of a Prognostic Algorithm: The INTERPRESS-IPD Collaboration.

Systolic interarm differences in blood pressure have been associated with all-cause mortality and cardiovascular disease. We undertook individual participant data meta-analyses to (1) quantify independent associations of systolic interarm difference with mortality and cardiovascular events; (2) develop and validate prognostic models incorporating interarm difference, and (3) determine whether interarm difference remains associated with risk after adjustment for common cardiovascular risk scores. We searched for studies recording bilateral blood pressure and outcomes, established agreements with collaborating authors, and created a single international dataset: the Inter-arm Blood Pressure Difference - Individual Participant Data (INTERPRESS-IPD) Collaboration. Data were merged from 24 studies (53 827 participants). Systolic interarm difference was associated with all-cause and cardiovascular mortality: continuous hazard ratios 1.05 (95% CI, 1.02-1.08) and 1.06 (95% CI, 1.02-1.11), respectively, per 5 mm Hg systolic interarm difference. Hazard ratios for all-cause mortality increased with interarm difference magnitude from a ≥5 mm Hg threshold (hazard ratio, 1.07 [95% CI, 1.01-1.14]). Systolic interarm differences per 5 mm Hg were associated with cardiovascular events in people without preexisting disease, after adjustment for Atherosclerotic Cardiovascular Disease (hazard ratio, 1.04 [95% CI, 1.00-1.08]), Framingham (hazard ratio, 1.04 [95% CI, 1.01-1.08]), or QRISK cardiovascular disease risk algorithm version 2 (QRISK2) (hazard ratio, 1.12 [95% CI, 1.06-1.18]) cardiovascular risk scores. Our findings confirm that systolic interarm difference is associated with increased all-cause mortality, cardiovascular mortality, and cardiovascular events. Blood pressure should be measured in both arms during cardiovascular assessment. A systolic interarm difference of 10 mm Hg is proposed as the upper limit of normal. Registration: URL: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42015031227.

Multiple Sleep Latency Test: when are 4 naps enough?

STUDY OBJECTIVES: The Multiple Sleep Latency Test (MSLT) is central to the diagnosis of narcolepsy and idiopathic hypersomnia. This study is the first to assess the impact of a 5-nap protocol on meeting MSLT-derived diagnostic criteria in a general cohort referred for MSLT, without selection bias. METHODS: Data for all MSLTs performed at 2 tertiary sleep units in Australia between May 2012 and May 2018 were retrospectively assessed for the impact of the fifth nap on mean sleep latency (MSL) and sleep onset rapid eye movement periods. RESULTS: There were 122 MSLTs included. The MSL was 8.7 ± 5.1 minutes after 4 naps, compared with 9.2 ± 5.2 minutes for 5 naps (P < .0001). In 8 cases, inclusion of the fifth nap changed the MSL to a value above the diagnostic threshold of 8 minutes. There were no instances in which the MSL moved to ≤ 8 minutes based on fifth nap data. A sleep onset rapid eye movement period occurred in the fifth nap in 9 patients and altered the interpretation in 2 cases. CONCLUSIONS: The fifth nap in an MSLT is associated with an increased MSL, although this difference is rarely clinically significant. In patients with borderline MSL or 1 sleep onset rapid eye movement period after 4 naps, a fifth nap can alter the outcome and should be performed. However, for many cases, a 4-nap MSLT protocol will suffice, potentially allowing resource savings without compromising diagnostic accuracy. We propose the adoption of a conditional 4-nap or 5-nap protocol based on specific criteria.

Lidocaine 5%-medicated plaster (Versatis) for localised neuropathic pain: results of a multicentre evaluation of use in children and adolescents.

The Lidocaine 5% plaster is licensed for the symptomatic relief of neuropathic pain associated with post-herpetic neuralgia in adult patients over 18 years of age. Studies in adults also demonstrate efficacy of Lidocaine 5% plasters in other neuropathic pain conditions. Case reports and experience suggested efficacy of Lidocaine 5% plasters in children and adolescents with localised neuropathic pain. Initiated by the Pain in Children Special Interest Group (PICSIG) of the British Pain Society, a 3-year prospective multicentre service evaluation was undertaken to document the usage and efficacy of the Lidocaine 5% plaster in paediatric patients being managed by paediatric pain teams in the United Kingdom. Five paediatric pain teams provided anonymised data pre-treatment and 3-6 months after commencing Lidocaine 5% plaster. Changes in pain score, function, sleep and continuing use were evaluated. Data were obtained for 115 patients; age range 5-18 years (mean: 12 years). Diagnosis and site of application varied. Benefit from use of a Lidocaine 5% plaster in an individual was deemed if two or more of the following were reported: reduction in pain score, functional improvement, sleep improvement and continuing use of Lidocaine 5% plaster. Benefit was recorded for 79 patients (69%); 32 patients were recorded as receiving no benefit and data were unavailable for 4 patients, and 7 patients reported minor skin reactions. This prospective service evaluation supports the efficacy of the Lidocaine 5% plaster in children and adolescents with localised neuropathic pain and confirms tolerability and safety. It is the opinion of the PICSIG of the British Pain Society that the Lidocaine 5% plaster should be considered early in the multidisciplinary management of localised neuropathic pain in children and adolescents.

Acute exacerbation of COPD.

The literature of acute exacerbation of chronic obstructive pulmonary disease (COPD) is fast expanding. This review focuses on several aspects of acute exacerbation of COPD (AECOPD) including epidemiology, diagnosis and management. COPD poses a major health and economic burden in the Asia-Pacific region, as it does worldwide. Triggering factors of AECOPD include infectious (bacteria and viruses) and environmental (air pollution and meteorological effect) factors. Disruption in the dynamic balance between the 'pathogens' (viral and bacterial) and the normal bacterial communities that constitute the lung microbiome likely contributes to the risk of exacerbations. The diagnostic approach to AECOPD varies based on the clinical setting and severity of the exacerbation. After history and examination, a number of investigations may be useful, including oximetry, sputum culture, chest X-ray and blood tests for inflammatory markers. Arterial blood gases should be considered in severe exacerbations, to characterize respiratory failure. Depending on the severity, the acute management of AECOPD involves use of bronchodilators, steroids, antibiotics, oxygen and noninvasive ventilation. Hospitalization may be required, for severe exacerbations. Nonpharmacological interventions including disease-specific self-management, pulmonary rehabilitation, early medical follow-up, home visits by respiratory health workers, integrated programmes and telehealth-assisted hospital at home have been studied during hospitalization and shortly after discharge in patients who have had a recent AECOPD. Pharmacological approaches to reducing risk of future exacerbations include long-acting bronchodilators, inhaled steroids, mucolytics, vaccinations and long-term macrolides. Further studies are needed to assess the cost-effectiveness of these interventions in preventing COPD exacerbations.

Use of inhaled corticosteroids in COPD: improving efficacy.

Chronic obstructive pulmonary disease (COPD) is a chronic, inflammatory lung disease characterized by airflow limitation that is not fully reversible. The pathological changes in COPD lead to alveolar destruction (emphysema) and chronic airway inflammation, resulting in airflow obstruction and recurrent exacerbations. Inhaled corticosteroids (ICS) are anti-inflammatory agents that are widely used, especially in combination with long-acting beta-agonists, in patients with COPD. Here, we will summarize the benefits and risks of ICS use for COPD, and discuss approaches to more personalized medicine when selecting COPD patients to commence (or withdraw) ICS use. The conclusion arising is that further validation of clinical and biological markers should be undertaken in COPD, in order to individualize ICS therapy to maximize efficacy for patients.

The spectral-structural relationship of a series of oxyluciferin derivatives.

The absorption and emission spectra of a series of oxyluciferin derivatives with different substituents, as well as 6'-amino oxyluciferins in different enol and keto forms, with or without an active-site model of luciferase, were systematically investigated using density functional theory. The effects of substituents, microenvironment, and the luciferase on the structures, absorption spectra, and fluorescent emission were all taken into account. It was found that a wide range of emission colors can be obtained from various oxyluciferin derivatives with the inclusion of active site residues modeling the luciferase active site. Enol and keto forms are responsible for the emissions observed in experiments. It was suggested that the active site of luciferase must be included in the calculation in order to determine the form of the emitters.

Bone-anchored hearing device placement with translabyrinthine tumor removal.

OBJECTIVE: Translabyrinthine resection of intracranial tumors results in single-sided deafness, which can be treated by surgical and nonsurgical means. Here we describe the first series examining complication and device usage rates among patients receiving a surgically implanted bone-anchored hearing device (BAHD) at the time of translabyrinthine tumor removal. STUDY DESIGN: Case series with chart review. SETTING: Private tertiary neurotologic referral center. PATIENTS: Patients (N = 154) undergoing concurrent BAHD placement and translabyrinthine tumor resection. INTERVENTION: Concurrent BAHD placement and translabyrinthine tumor removal. MAIN OUTCOME MEASURES: Postoperative complication rates and BAHD usage. RESULTS: Of the 154 patients, 121 (78.6%) had no device-related complications. The most common device-related complications were skin overgrowth (8.4%), acute infection (5.2%), and chronic infection (3.2%). The overall and specific complication rates did not differ from published BAHD complication rates. One patient (0.6%) developed a cerebrospinal leak through the surgical site for the device. At the time of last follow-up (mean, 39.8 months), 151 patients (95.0%) were still using their devices. CONCLUSION: Patients undergoing concurrent translabyrinthine tumor removal and BAHD placement exhibit similar device-related complication profiles as patients undergoing standard device placement. Based on these outcomes and the high long-term usage rates, BAHD insertion at the time of translabyrinthine intracranial surgery can be considered a safe and useful procedure.

Implementing clinical guidelines for chronic obstructive pulmonary disease: barriers and solutions.

Chronic obstructive pulmonary disease (COPD) is a complex chronic lung disease characterised by progressive fixed airflow limitation and acute exacerbations that frequently require hospitalisation. Evidence-based clinical guidelines for the diagnosis and management of COPD are now widely available. However, the uptake of these COPD guidelines in clinical practice is highly variable, as is the case for many other chronic disease guidelines. Studies have identified many barriers to implementation of COPD and other guidelines, including factors such as lack of familiarity with guidelines amongst clinicians and inadequate implementation programs. Several methods for enhancing adherence to clinical practice guidelines have been evaluated, including distribution methods, professional education sessions, electronic health records (EHR), point of care reminders and computer decision support systems (CDSS). Results of these studies are mixed to date, and the most effective ways to implement clinical practice guidelines remain unclear. Given the significant resources dedicated to evidence-based medicine, effective dissemination and implementation of best practice at the patient level is an important final step in the process of guideline development. Future efforts should focus on identifying optimal methods for translating the evidence into everyday clinical practice to ensure that patients receive the best care.

Management of incus necrosis in revision stapedectomy using hydroxyapatite bone cement.

OBJECTIVE: Review audiological outcomes after revision stapedectomy using hydroxyapatite cement. STUDY DESIGN: Retrospective case review. SETTING: Tertiary neurotological referral center. PATIENTS: Thirty-seven cases of previously treated otosclerosis where incus erosion was observed during revision stapedectomy. INTERVENTION(S): Hydroxyapatite cement was used to rebuild the eroded incus and stabilize the prosthesis during revision stapedectomy. MAIN OUTCOME MEASURE(S): Pre- and postoperative bone conduction (BC) and air conduction (AC) pure-tone averages (PTA) (0.5, 1, 2, 3 kHz) including high tone BC (1, 2, 4 kHz), air-bone gap (ABG), and speech discrimination scores were measured. Short-term (3 wk) and longer-term (average 12 mo) hearing outcomes were measured. Data are reported according to the most recent guidelines of the AAO-HNS Committee on Hearing and Equilibrium. RESULTS: Among 37 ears undergoing revision stapedectomy with hydroxyapatite cement, the AC PTA was 59.8 dB preoperatively and 34.6 dB postoperatively (p < 0.0001) at latest follow-up. The mean postoperative ABG was 8.8 dB (SD = 8.6, range -1.3-36.3 dB) while a mean improvement of 2.1 dB (SD = 6.5, range -8.33-15.0 dB) of the high tone BC PTA was observed. One revision case was noted during the follow-up period. CONCLUSION: Hydroxyapatite cement is useful to reconstruct and stabilize the prosthesis in revision stapedectomy when erosion of the long process is encountered. Short- and longer-term hearing results are favorable when compared to previously reported results of revision stapedectomy.

Outcomes following Semicircular Canal Plugging.

OBJECTIVES: To examine audiometric outcomes, symptom control rates, and complication rates following semicircular canal plugging for superior semicircular canal dehiscence syndrome. STUDY DESIGN: Retrospective chart review. SETTING: Private, neurotologic tertiary referral center. SUBJECTS AND METHODS: Patients undergoing semicircular canal plugging for superior semicircular canal dehiscence syndrome from January 1, 2007, to December 31, 2012. Pre- and postoperative audiometry, vestibular testing, operative findings, and clinical symptoms were assessed through chart review. RESULTS: A total of 24 ears underwent a canal plugging procedure during the study period for superior canal dehiscence syndrome. Pre- and postoperative air conduction pure-tone averages were 21.1 and 22.5 dB (P = .42, not significant [NS]). The average pre- and postoperative word recognition scores were 95.8% and 95.1% (P = .48, NS). Vestibular evoked myogenic potential data showed reduced thresholds in 7 patients with canal dehiscence. Complications were limited to a single, temporary facial weakness. Complete symptom improvement was noted in 35.7% of all patients, while at least partial symptom improvement was found in over 80% of patients. CONCLUSION: Semicircular canal plugging procedures are associated with excellent hearing outcomes and may reduce preoperative symptoms in patients with superior semicircular canal dehiscence.

Novel use of intrathecal baclofen drug delivery system for periodic focal dystonia in a teenager.

INTRODUCTION: Focal dystonia, often affecting part of a limb, is a manifestation of complex regional pain syndrome (CRPS). This can be difficult to diagnose and treat. Furthermore, there may be significant latency between the onset of dystonia after the diagnosis of CRPS. We present the case of a 15-year-old girl with periodic focal dystonia who has been successfully treated with an intrathecal baclofen pump. MATERIALS AND METHODS: The patient had sustained a minor ankle fracture four years prior to presentation. Despite radiologic evidence of adequate bony union, the patient continued to complain of spasms and pain in her left foot leading to dystonic posturing of the foot. Multiple therapies including subcutaneous morphine infusion, ankle splinting, physiotherapy, and local botulinum injections had not provided adequate relief. Intrathecal baclofen on three separate occasions resulted in successful temporary resolution of the dystonia. A placebo double-blinded injection of intrathecal saline at a separate setting however did not resolve the dystonia. RESULTS: We then proceeded with permanent delivery of baclofen by implantation of an intrathecal drug delivery pump, which resulted in resolution of the dystonia. The patient also was able to receive bolus doses of intrathecal baclofen. The patient is now able to partake in sporting and dancing activities. A detailed history of the patient, along with the difficulties in diagnosis and management, is presented. CONCLUSION: Intrathecal baclofen therapy can be effective in the management of focal dystonia after rigorous preoperative testing and counseling of adolescents with CRPS.

Bilateral posterior semicircular canal dehiscence in the setting of Hallermann-Streiff syndrome.

Hallermann-Streiff syndrome, also known as oculomandibulofacial syndrome, is a rare congenital disorder affecting growth and cranial, dental, ocular, pilocutaneous, and mental development. In addition to routine audiologic testing in patients with this syndrome, high-resolution computed tomography of the temporal bones should be performed in those with documented or suspected sensorineural hearing loss. Cochlear implantation may be considered, as in other children with syndromic hearing loss and certain inner ear abnormalities, if the appropriate audiologic, psychosocial, and medical criteria are met. The current case report radiographically and clinically characterizes inner ear dysplasia in an 8-year-old patient with Hallermann-Streiff syndrome. High-resolution computed tomography of the temporal bones revealed a hypoplastic bony island between the vestibule and horizontal semicircular canals, as well as incomplete bony coverage of the posterior semicircular canal crura bilaterally. To our knowledge, this is the first report of a pediatric patient demonstrating bilateral posterior semicircular canal dehiscence.