Bilateral Vocal Cord Palsy Secondary to Rheumatoid Arthritis.

Rheumatoid arthritis (RA) can cause a number of laryngeal manifestations; however, most of these do not cause an airway emergency. Airway obstruction due to vocal cord fixation of one or both vocal cords occurs late in the disease process of RA and can present as an inspiratory stridor. We report the case of an elderly lady who presented with acute stridor secondary to RA-induced bilateral vocal cord palsy and describe the various management options that were considered. An 85-year-old woman presented to A&E Resus with tachypnoea, stridor, and drowsiness. An arterial blood gas (ABG) was performed which showed hypercapnic respiratory failure on 60% oxygen with blood tests revealing moderately raised infective markers and a chest X-ray displaying right lower zone consolidation. A flexible nasendoscopy was performed which demonstrated bilaterally fixed and adducted vocal cords due to bilateral cricoarytenoid joint fixation, with a rima glottidis measurement of approximately 3 mm and evidence of paradoxical breathing. The patient had been admitted with a similar presentation 18 months before, however not as severe, and once again, the bilateral vocal cord palsy had been attributed to her longstanding RA. She was stabilised with non-invasive ventilation and transferred to the acute respiratory care unit. Long-term surgical options were thoroughly discussed including tracheostomy, vocal cord lateralisation, cordotomy, and arytenoidectomy, but ultimately, these options were all deemed unsuitable for the patient and so a palliative care approach was adopted following the withdrawal of bilevel positive airway pressure. Stridor is a late but life-threatening complication of RA that has viable surgical options of tracheostomy and static glottis enlarging procedures; however, the appropriateness of such procedures should always be correlated with the patient's current clinical status and the extent to which they may impact on the patient's quality of life.

Combining four screening tools for cost effective screening of OSA in train drivers: A UK experience.

Background: Obstructive sleep apnoea (OSA) in drivers/workers has been implicated in railway and road traffic safety incidents; however, there are insufficient data on its prevalence and cost-effective screening methods. Aim: This pragmatic study examines four OSA screening tools: the Epworth sleepiness scale (ESS), the STOP-Bang (SB), the adjusted neck circumference (ANC) and the body mass index (BMI), exploring their suitability and effectiveness separately and in combination. Method: Using all four tools, 292 train drivers were opportunistically screened between 2016 and 2017. A polygraph (PG) test was carried out when OSA was suspected. Patients with an apnoea-hypopnea index (AHI) ≥5 were referred to a clinical specialist and reviewed annually. Those who had continuous positive airway pressure (CPAP) treatment were evaluated for compliance and control. Results: Of the 40 patients who had PG testing, 3 and 23 participants met the ESS >10 and SB >4, criteria, respectively, whereas 25 participants each had an ANC >48 and a BMI >35 with a risk factor or ≥40 with none. OSA was detected in 3, 18 and 16 of them who met the ESS, SB and ANC criteria, respectively, and was positive for OSA in addition to 16 others who met the BMI criteria. A total of 28 (72%) were diagnosed with OSA. Conclusion: Although when used individually, these screening methods are less effective/inadequate, combining them is easy, feasible and offers the maximum chance of OSA detection in train drivers.

Patient-reported outcome measures following maxillomandibular advancement surgery in patients with obstructive sleep apnoea syndrome.

Maxillomandibular advancement (MMA) is an effective treatment for obstructive sleep apnoea syndrome (OSAS) that is refractory to conventional treatment. However, it is a highly invasive procedure with several recognised side effects, and we know of few data on its effect on important patient-reported outcome measures (PROMS). Here we describe a case series of patients selected for MMA through our joint respiratory/maxillofacial surgery clinic, detailing the effect of MMA on objective physiological measurements and important PROMS. Patients with confirmed moderate/severe symptomatic OSAS who could not tolerate continuous positive airway pressure (CPAP) or mandibular advancement devices (MAD) were assessed in the clinic for consideration of MMA. Preoperative and postoperative airway measurements, apnoea/hypopnoea index (AHI), Epworth sleepiness scale (ESS) score, and quality of life (10-point Likert scale), were recorded. A customised questionnaire was administered postoperatively to assess selected psychosocial and functional domains (sleep quality, energy levels, appearance, ability to perform daily activities, and mood) and patient satisfaction using five-point Likert scales. Over an 18 month period, 39 patients were referred for consideration of MMA. Ten patients (7 men and 3 women, mean age 49.9, mean BMI 27.5) underwent surgery, which resulted in significant improvements in ESS, quality of life, AHI, and airway diameters. All patients reported improvements in all psychosocial/functional domains except appearance, in which five reported no change or worsened appearance. All subjects felt that MMA provided better symptom control than CPAP. The most commonly reported side effects were facial/lip numbness (9/10) and affected bite (6/10). MMA resulted in significant improvements in ESS, quality of life, and a range of PROMS, with a high level of patient satisfaction.

The variation of FiO2 with circuit type and peak inspiratory flow rate during non-invasive respiratory support using domiciliary ventilators and its significance during the COVID-19 pandemic.

Background: The COVID-19 pandemic has resulted in increased admissions with respiratory failure and there have been reports of oxygen failure and shortages of machines to deliver ventilation and Continuous Positive Airway Pressure (CPAP). Domiciliary ventilators which entrain room air have been widely used during the pandemic. Poor outcomes reported with non-invasive respiratory support using ventilators which lack an oxygen blender could be related to an unreliable Fraction of inspired O2 (FiO2). Additionally, with concerns about oxygen failure, the variety of ventilator circuits used as well as differing peak inspiratory flow rates (PIFR) could impact on the FiO2 delivered during therapy with domiciliary ventilators. Methods: In a series of bench tests, we tested the effect of choice of circuit and different PIFR on the FiO2 achieved during simulation of ventilation and CPAP therapy using domiciliary ventilators. Results: FiO2 was highly dependent upon the type of circuit used with circuits with an active exhalation valve achieving similar FiO2 at lower oxygen flow rates than circuits using an exhalation port. During CPAP therapy, high PIFR resulted in significantly lower FiO2 than low PIFR. Conclusions: This study has implications for oxygen usage as well as delivery of non-invasive respiratory support during therapy with domiciliary ventilators when these are used during the second wave of COVID-19.

How to eat, drink and speak on non-invasive ventilation.

We report our observations on six individuals with non-bulbar neuromuscular disorders using non-invasive ventilation (NIV), who were able to maintain adequate hydration and nutrition orally despite being ventilator-dependant. All had severe respiratory muscle weakness, with a vital capacity less than 500 mL and cough peak flow rate less than 250 L/min. Their median (range) age was 49 (23-64) years; they had been on NIV for 8 (2-24) years. We compared them with an age- and sex-matched normal control. Individuals with neuromuscular disorders needed to chew each mouthful of food significantly more times (median 44, range 18-120 chews) than normal controls (median 15, range 10-20 chews). They took longer to completely swallow a mouthful of food (median 37, range 24-100 s) compared to normal controls (median 14.5, range 10-21 s). Multiple swallows for each mouthful were seen in all neuromuscular individuals, but in only one normal control. Two individuals coughed after swallowing; both these subjects were clinically stable at the time of the study. The median number of NIV breaths associated with chest expansion for each mouthful was 11 (range 5-49). All subjects blocked some NIV breaths whilst eating. Before swallowing, they always waited until the expiratory phase of the NIV breath was complete; no post-swallow expiration was seen, whereas normal subjects invariably exhibited post-swallow expiration. All individuals were able to block several ventilator breaths whilst swallowing un-thickened liquids. The median (range) number of words between breaths was 5 (4-7) for the neuromuscular individuals on NIV, significantly fewer than 11 (8-13) for the matched controls. Eating, drinking and speaking are possible whilst on NIV. Use of cough-assist after eating is recommended, given the likelihood of silent aspiration.

Spot the Pneumothorax.

Subcutaneous emphysema (SE) and pneumomediastinum are commonly associated with critically ill patients with blunt or penetrating trauma, in particular lower rib fractures. It however rarely needs urgent intervention, and routine use of chest tube tracheostomy or mediastinal drains is not recommended as the patients do not go on to develop a respiratory compromise. Our case is novel as it describes a case of subcutaneous emphysema with acute upper airway compromise and respiratory distress requiring urgent bilateral wide bore subcutaneous drains and thoracic drain insertion. The patient required a prolonged recovery period. This case serves to illustrate the technical difficulty in establishing a cause of subcutaneous emphysema, the limitations of standard imaging in identifying a pneumothorax in subcutaneous emphysema, and the value of prompt insertion of bilateral subcutaneous wide bore drains to buy precious time for definitive imaging and management.

A retrospective cohort study of idiopathic diaphragmatic palsy: a diagnostic triad, natural history and prognosis.

BACKGROUND: Isolated diaphragmatic palsy in the absence of progressive neuromuscular disease is uncommon. It poses diagnostic challenges and limited data are available regarding prognosis. We present retrospective cohort data from two large teaching hospitals in the United Kingdom. METHOD: 60 patients who were assessed either as inpatients or outpatients were included in this study. Patients with progressive neuromuscular disease were excluded. Clinical presentation, tests of respiratory muscle function (sitting/supine vital capacity, maximal expiratory pressure (MEP), maximal inspiratory pressure (MIP) and sniff nasal inspiratory pressure (SNIP)) and outcomes were recorded. RESULTS: For patients with diaphragmatic palsy, mean±sd seated and supine vital capacity pre-noninvasive ventilation (NIV) were reduced at 1.7±1.2 L and 1.1±0.9 L, respectively, with a mean±sd postural fall in vital capacity of 42±0.16%. The mean MEP/MIP and MEP/SNIP ratios for diaphragmatic palsy were 3 and 3.5, respectively. After a year of treatment with NIV, mean±sd upright and supine vital capacity had increased to 2.1±0.9 L and 1.8±1 L, respectively, and the mean±sd fall in vital capacity from sitting to supine reduced to 29±0.17%. MEP/MIP and MEP/SNIP ratios reduced to 2.6 and 2.9, respectively, from the pre-NIV values. The values of postural fall in vital capacity correlated (p<0.05) with MEP/MIP and MEP/SNIP ratio (r2=0.86 and r2=0.7, respectively). CONCLUSION: Tests of respiratory muscle strength are valuable in the diagnostic workup of patients with unexplained dyspnoea. A triad of 1) orthopnoea, with 2) normal lung imaging and 3) MEP/MIP and/or MEP/SNIP ratio ≥2.7 points towards isolated diaphragmatic palsy. This needs to be confirmed by prospective studies.

A Case Report of Acute Flaccid Paralysis Caused by Enterovirus D68 Infection: The Beginning of a Polio-Like Epidemic?

Enterovirus D68 (EV-D68) is a non-polio enterovirus that occasionally causes respiratory illnesses. EV-D68 infections have occurred over the last couple of years and have a high prevalence worldwide. This virus has recently been linked to acute flaccid paralysis and particularly affects children. We report the case of a young adult who presented with acute neurological manifestations along with respiratory involvement. EV-D68 was detected in the patient's broncho-alveolar lavage and was followed by a prolonged recovery period. Clinicians should consider EV-D68 infection in the differential diagnosis of acute flaccid paralysis (AFP) and respiratory failure.

Long-Term Survival on Noninvasive Ventilation in Adults With Thoracic Scoliosis.

BACKGROUND: Subjects with thoracic scoliosis were an important group in early studies of noninvasive ventilation (NIV). The aim of this study was to describe current rates of initiation of NIV and survival after initiation in this population. METHODS: This study included patients identified as having thoracic scoliosis and established between 1993 and 2018 on home NIV. Patients with scoliosis secondary to neuromuscular disease (other than poliomyelitis) were excluded. Survival rates were calculated for various time intervals up to 25 y. RESULTS: A total of 53 subjects with thoracic scoliosis were successfully established on NIV. [Formula: see text] levels prior to starting NIV were 55 ± 23 mm Hg. FVC was 0.5 ± 0.1 L, 18.5 ± 9% of predicted, with a Cobb angle of 101 ± 3.5 degrees. The 5-, 10-, 15-, 20-, and 25-y survival rates were 96%, 88%, 61%, 46%, and 39%, respectively. At the time of death, subjects had been on home NIV for 9.2 ± 5.1 y and were 75.5 ± 9.2 y old. There was no significant correlation between mortality and age at time of commencing home NIV, initial arterial blood gas results, FVC, or Cobb angle. There was no significant difference in survival between those with and without poliomyelitis. In 8 of 10 of the most recent years of this survey, subjects with scoliosis have been commenced on home NIV. CONCLUSIONS: Small numbers of subjects with scoliosis continued to present with respiratory failure. Once established on home NIV, around 40% survived ≥ 25 y. Long-term care will be needed for many years to come for this patient population.

The Noninvasive Ventilation Outcomes (NIVO) score: prediction of in-hospital mortality in exacerbations of COPD requiring assisted ventilation.

INTRODUCTION: Acute exacerbations of COPD (AECOPD) complicated by acute (acidaemic) hypercapnic respiratory failure (AHRF) requiring ventilation are common. When applied appropriately, ventilation substantially reduces mortality. Despite this, there is evidence of poor practice and prognostic pessimism. A clinical prediction tool could improve decision making regarding ventilation, but none is routinely used. METHODS: Consecutive patients admitted with AECOPD and AHRF treated with assisted ventilation (principally noninvasive ventilation) were identified in two hospitals serving differing populations. Known and potential prognostic indices were identified a priori. A prediction tool for in-hospital death was derived using multivariable regression analysis. Prospective, external validation was performed in a temporally separate, geographically diverse 10-centre study. The trial methodology adhered to TRIPOD (Transparent Reporting of a Multivariable Prediction Model for Individual Prognosis or Diagnosis) recommendations. RESULTS: Derivation cohort: n=489, in-hospital mortality 25.4%; validation cohort: n=733, in-hospital mortality 20.1%. Using six simple categorised variables (extended Medical Research Council Dyspnoea score 1-4/5a/5b, time from admission to acidaemia >12 h, pH <7.25, presence of atrial fibrillation, Glasgow coma scale ≤14 and chest radiograph consolidation), a simple scoring system with strong prediction of in-hospital mortality is achieved. The resultant Noninvasive Ventilation Outcomes (NIVO) score had area under the receiver operating curve of 0.79 and offers good calibration and discrimination across stratified risk groups in its validation cohort. DISCUSSION: The NIVO score outperformed pre-specified comparator scores. It is validated in a generalisable cohort and works despite the heterogeneity inherent to both this patient group and this intervention. Potential applications include informing discussions with patients and their families, aiding treatment escalation decisions, challenging pessimism and comparing risk-adjusted outcomes across centres.

Assessing the impact of posture on diaphragm morphology and function using an open upright MRI system-A pilot study.

PURPOSE: The diaphragm is the most important muscle of respiration. Disorders of the diaphragm can have a deleterious impact on respiratory function. We aimed to evaluate the use of an open-configuration upright low-field MRI system to assess diaphragm morphology and function in patients with bilateral diaphragm weakness (BDW) and chronic obstructive pulmonary disease (COPD) with hyperinflation. METHOD: The study was approved by the National Research Ethics Committee, and written consent was obtained. We recruited 20 healthy adult volunteers, six subjects with BDW, and five subjects with COPD with hyperinflation. We measured their vital capacity in the upright and supine position, after which they were scanned on the 0.5 T MRI system during 10-s breath-holds at end-expiration and end-inspiration in both positions. We developed and applied image analysis methods to measure the volume under the dome, maximum excursion of hemidiaphragms, and anterior-posterior and left-right extension of the diaphragm. RESULTS: All participants were able to complete the scanning protocol. The patients found scanning in the upright position more comfortable than the supine position. All differences in the supine inspiratory-expiratory parameters, excluding left-right extension, were significantly smaller in the BDW and COPD groups compared with healthy volunteers. No significant correlation was found between the postural change in diaphragm morphology and vital capacity in either group. CONCLUSION: Our combined upright-supine MR imaging approach facilitates the assessment of the impact of posture on diaphragm morphology and function in patients with BDW and those with COPD with hyperinflation.

Non-invasive ventilation during surgery under neuraxial anaesthesia: a pathophysiological perspective on application and benefits and a systematic literature review.

Unlike general anaesthesia, neuraxial anaesthesia (NA) reduces the burden and risk of respiratory adverse events in the post-operative period. However, both patients affected by chronic obstructive pulmonary disease (COPD) and chest wall disorders and/or neuromuscular diseases may experience the development or the worsening of respiratory failure, even during surgery performed under NA; this latter negatively affects the function of accessory respiratory muscles, resulting in a blunted central response to hypercapnia and possibly in an exacerbation of cardiac dysfunction (NA-induced relative hypovolemia). According to European Respiratory Society (ERS) and American Thoracic Society (ATS) guidelines, non-invasive ventilation (NIV) is effective in the post-operative period for the treatment of both impaired pulmonary gas exchange and ventilation, while the intra-operative use of NIV in association with NA is just anecdotally reported in the literature. Whilst NIV does not assure a protected patent airway and requires the patient's cooperation, it is a handy tool during surgery under NA: NIV is reported to be successful for treatment of acute respiratory failure; it may be delivered through the patient's home ventilator, may reverse hypoventilation induced by sedatives or inadvertent spread of anaesthetic up to cervical dermatomes, and allow the avoidance of intubation in patients affected by chronic respiratory failure, prolonging the time of non-invasiveness of respiratory support (i.e., neuromuscular patients needing surgery). All these advantages could make NIV preferable to oxygen in carefully selected patients.

Correction of Paradoxical Ribcage Motion in Scoliosis by Noninvasive Ventilation.

STUDY DESIGN: Observational study of ribcage motion in scoliosis. OBJECTIVE: To see whether noninvasive ventilation corrected paradoxical inward motion of the ribs during inspiration. SUMMARY OF BACKGROUND DATA: Paradoxical inward motion of the ribs is observed after rib fractures, low cervical cord injury, and in chronic obstructive pulmonary disease. It is not well recognized in scoliosis and the mechanism in this group has not been studied. METHODS: Linearized magnetometers were used to measure the diameter of the ribcage. Changes in diameter during tidal breathing were recorded during spontaneous ventilation and noninvasive ventilation in 10 subjects with idiopathic or congenital thoracic scoliosis. RESULTS: During spontaneous breathing, the median change in ribcage diameter during inspiration was -1.5 (range -2.3 to -0.8) cm. The median change in ribcage diameter during noninvasive ventilation was +0.5 (range -1.1 to +1.2) cm. Noninvasive ventilation improved paradoxical motion in all subjects, completely correcting it in six. CONCLUSION: Paradoxical inward motion of the ribcage is seen in some subjects with severe scoliosis. This abnormal motion is improved or abolished by noninvasive ventilation. Since noninvasive ventilation takes over the work of breathing from the respiratory muscles, we suggest that inspiratory muscle contraction causes distortion of part of the ribcage in scoliosis, probably because of the abnormal orientation of diaphragmatic muscle fibers. LEVEL OF EVIDENCE: 2.

High prevalence of recurrent nocturnal desaturations in systemic AL amyloidosis: a cross-sectional pilot study.

OBJECTIVES: Cardiac involvement and/or macroglossia with soft tissue deposits are risk factors for central sleep apnoea (CSA) and obstructive sleep apnoea (OSA), and common features of systemic AL amyloidosis. Little data exist on the occurrence of sleep-disordered breathing (SDB) or recurrent nocturnal hypoxia in amyloidosis, which this study sought to investigate. METHODS: A total of 72 consecutive patients with systemic amyloidosis (mean age 69 years and mean BMI 25) were evaluated for occurrence of SDB, by overnight continuous pulse oximetry, and completed Epworth Sleepiness Score (ESS) and STOPBANG questionnaires. Patients included: AL cardiac (AL-C), AL macroglossia (AL-M), AL both (AL-CM) and transthyretin (ATTR). RESULTS: Mean overnight oxygen saturations were 93% (SD ± 2, 95% CI 87-96) with abnormal oximetry (4% oxygen desaturation index (ODI) >5/hour): AC-C 84%, AL-M 57%, AL-CM 62% and ATTR 47%. NYHA class directly correlated with a higher 4% ODI, NYHA class I vs 3, (p = 0.01). Two-thirds of patients had STOPBANG scores >3 and abnormally high ESS scores (>10) were seen in up to 30% of patients. CONCLUSION: Recurrent nocturnal hypoxaemia, suggestive of sleep-disordered breathing, is frequent in systemic AL amyloidosis. The higher incidence in cardiac amyloidosis highlights CSA and recurrent hypoxia as possible mechanisms for morbidity/mortality in these cases. A detailed polysomnography study is planned to clarify and further investigate these findings.