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Diaphragmatic palsy after lung transplantation has been reported infrequently. Given the role of the diaphragm in respiration, the palsy may play a significant role in the post-surgical recovery as well as morbidity and mortality. This review summarises the current literature to better understand diaphragmatic palsy in the post lung-transplant setting among adults. A thorough literature search was conducted through multiple databases and 91 publications were identified that fit the research question. The review aimed to report the burden of this problem, explore different modalities of diagnosis reported, the effect of various clinical factors and treatment modalities, as well as their effects on outcomes. Additionally, it aimed to highlight the variability, limitations of reported data, and the absence of a standardised approach. This review emphasises the crucial need for more dedicated research to better address this clinical challenge., Competing Interests: Conflict of interest: All authors have nothing to disclose., (Copyright ©The authors 2024.)

Study Design: Case report and narrative review., Objective: To explore the therapeutic role of surgical and nonsurgical treatment of diaphragmatic paralysis secondary to spinal cord and nerve root compression., Summary of Background Data: Phrenic nerve dysfunction due to central or neuroforaminal stenosis is a rare yet unappreciated etiology of diaphragmatic paralysis and chronic dyspnea. Surgical spine decompression, diaphragmatic pacing, and intensive physiotherapy are potential treatment options with varying degrees of evidence., Methods: The case of a 70-year-old male with progressive dyspnea, reduced hemi-diaphragmatic excursion, and C3-C7 stenosis, who underwent a microscopic foraminotomy is discussed. Literature review (MEDLINE, PubMed, Google Scholar) identified 19 similar reports and discussed alternative treatments and outcomes., Results and Conclusions: Phrenic nerve root decompression and improvement in neuromonitoring signals were observed intraoperatively. The patient's postoperative course was uncomplicated, and after 15 months, he experienced significant symptomatic improvement and minor improvement in hemi-diaphragmatic paralysis and pulmonary function tests. All case reports of patients treated with spinal decompression showed symptomatic and/or functional improvement, while one of the 2 patients treated with physiotherapy showed improvement. More studies are needed to further describe the course and outcomes of these interventions, but early identification and spinal decompression can be an effective treatment., Ocebm Level of Evidence: Level-4., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: A frequent complication of Fontan operations is unilateral diaphragmatic paresis, which leads to hemodynamic deterioration of the Fontan circulation. A potential new therapeutic option is the unilateral diaphragmatic pacemaker. In this study, we investigated the most effective stimulation location for a potential fully implantable system in a porcine model., Methods: Five pigs (20.8 ± 0.95 kg) underwent implantation of a customized cuff electrode placed around the right phrenic nerve. A bipolar myocardial pacing electrode was sutured adjacent to the motor point and peripherally at the costophrenic angle (peripheral diaphragmatic muscle). The electrodes were stimulated 30 times per minute with a pulse duration of 200 µs and a stimulation time of 300 ms. Current intensity was the only variable changed during the experiment., Results: Effective stimulation occurred at 0.26 ± 0.024 mA at the phrenic nerve and 7 ± 1.22 mA at the motor point, a significant difference in amperage (p = 0.005). Even with a maximum stimulation of 10 mA at the peripheral diaphragm muscle, however, no effective stimulation was observed., Conclusion: The phrenic nerve seems to be the best location for direct stimulation by a unilateral thoracic diaphragm pacemaker in terms of the required amperage level in a porcine model., (© 2024. The Author(s).)

The diaphragm is the key muscle of respiration, especially in infants. Diaphragmatic dysfunction and paralysis can have significant implications for medical management and treatment, and they can be challenging to diagnose by clinical parameters alone. Multiple imaging modalities are useful for assessing the diaphragm, but US - specifically M-mode US - offers several distinct advantages and few limitations compared to fluoroscopy, radiography, CT and MRI. The purpose of this manuscript is to discuss the pathophysiology of the diaphragm, review common indications for dynamic diaphragmatic US, describe optimal imaging technique, and discuss how to avoid imaging pitfalls., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Rationale: Respiratory muscle paralysis due to low cervical spinal cord injury (CSCI) can lead to dysphagia. Noninvasive positive airway pressure (PAP) therapy can effectively treat this type of dysphagia. High-flow nasal cannula (HFNC) oxygen therapy can generate a low level of positive airway pressure resembling PAP therapy, it may improve the dysphagia., Patient Concerns: The patient was an 87-year-old man without preexisting dysphagia. He suffered a CSCI due to a dislocated C5/6 fracture, without brain injury, and underwent emergency surgery. Postoperatively (day 2), he complained of dysphagia, and the intervention was initiated., Diagnosis: Based on clinical findings, dysphagia in this case, may have arisen due to impaired coordination between breathing and swallowing, which typically occurs in patients with CSCI who have reduced forced vital capacity., Interventions: HFNC oxygen therapy was started immediately after the surgery, and swallowing rehabilitation was started on Day 2. Indirect therapy (without food) and direct therapy (with food) were applied in stages. HFNC oxygen therapy appeared to be effective because swallowing function temporarily decreased when the HFNC oxygen therapy was changed to nasal canula oxygen therapy., Outcomes: Swallowing function of the patient improved and he did not develop aspiration pneumonia., Lessons: HFNC oxygen therapy improved swallowing function in a patient with dysphagia associated with respiratory-muscle paralysis following a CSCI. It may have prolonged the apnea tolerance time during swallowing and may have improved the timing of swallowing. HFNC oxygen therapy can facilitate both indirect and direct early swallowing therapy to restore both swallowing and respiratory function., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objectives: Bilateral diaphragmatic dysfunction results in severe dyspnoea, usually requiring oxygen therapy and nocturnal ventilatory support. Although treatment options are limited, phrenic nerve reconstruction (PR) offers the opportunity to restore functional activity. This study aims to evaluate combination treatment with PR and placement of a diaphragm pacemaker (DP) compared to DP placement alone in patients with bilateral diaphragmatic dysfunction., Methods: Patients with bilateral diaphragmatic dysfunction were prospectively enrolled in the following treatment algorithm: Unilateral PR was performed on the more severely impacted side with bilateral DP implantation. Motor amplitudes, ultrasound measurements of diaphragm thickness, maximal inspiratory pressure, forced expiratory volume, forced vital capacity and subjective patient-reported outcomes were obtained for retrospective analysis following completion of the prospective database., Results: Fourteen male patients with bilateral diaphragmatic dysfunction confirmed on chest fluoroscopy and electrodiagnostic testing were included. All 14 patients required nocturnal ventilator support, and 8/14 (57.1%) were oxygen-dependent. All patients reported subjective improvement, and all 8 oxygen-dependent patients were able to discontinue oxygen therapy following treatment. Improvements in maximal inspiratory pressure, forced vital capacity and forced expiratory volume were 68%, 47% and 53%, respectively. There was an average improvement of 180% in motor amplitude and a 50% increase in muscle thickness. Comparison of motor amplitude changes revealed significantly greater functional recovery on the PR + DP side., Conclusions: PR and simultaneous implantation of a DP may restore functional activity and alleviate symptoms in patients with bilateral diaphragmatic dysfunction. PR plus diaphragm pacing appear to result in greater functional muscle recovery than pacing alone., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Diaphragm Pacing(DP) demonstrates benefits over mechanical ventilation(MV) for spinal cord injured(SCI) patients. The hypothesis of this report is that phrenic nerve conduction study(PNCS) results cannot differentiate success or failure in selection of patients for DP. Direct surgical evaluation of the diaphragm should be performed., Methods: Observational report of prospective databases of patients undergoing laparoscopic evaluation of their diaphragms to assess for ability to stimulate to cause contraction for ventilation., Results: In 50 SCI patients who could not be weaned from MV, PNCS results showed latencies in stimulated patients (n = 44) and non-stimulated(n = 6) overlapped (7.8 ± 2.5 ms vs 9.4 ± 2.8 ms) and the null hypothesis cannot be rejected (p-value>0.05). Amplitudes overlapped (0.4 ± 0.2 mV vs 0.2 ± 0.2 mV) and the null hypotheses cannot be rejected (P-value >0.05). In 125 non SCI patients with diaphragm paralysis, there were 78(62.4%) with false negative PNCS., Conclusion: PNCS are inadequate pre-operative studies. Direct laparoscopic evaluation should be offered for all SCI patients to receive the benefit of DP., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Introduction: Guillain-Barré syndrome (GBS) is one of the most common causes of acute flaccid paralysis, with up to 20%-30% of patients requiring mechanical ventilation. The aim of our study was to develop and validate a mechanical ventilation risk nomogram in a Chinese population of patients with GBS., Methods: A total of 312 GBS patients were recruited from January 1, 2015, to June 31, 2018, of whom 17% received mechanical ventilation. The least absolute shrinkage and selection operator (LASSO) regression model was used to select clinicodemographic characteristics and blood markers that were then incorporated, using multivariate logistic regression, into a risk model to predict the need for mechanical ventilation. The model was characterized and assessed using the C-index, calibration plot, and decision curve analysis. The model was validated using bootstrap resampling in a prospective study of 114 patients recruited from July 1, 2018, to July 10, 2019., Results: The predictive model included hospital stay, glossopharyngeal and vagal nerve deficits, Hughes functional grading scale scores at admission, and neutrophil/lymphocyte ratio (NLR). The model showed good discrimination with a C-index value of 0.938 and good calibration. A high C-index value of 0.856 was reached in the validation group. Decision curve analysis demonstrated the clinical utility of the mechanical ventilation nomogram., Conclusions: A nomogram incorporating hospital stay, glossopharyngeal and vagal nerve deficits, Hughes functional grading scale scores at admission, and NLR may reliably predict the probability of requiring mechanical ventilation in GBS patients., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Phrenic nerve injury (PNI) from birth trauma is a recognized phenomenon, generally occurring with ipsilateral brachial plexus palsy (BPP). In severe cases, PNI results in diaphragm paresis (DP) and respiratory insufficiency. Surgical diaphragmatic plication (SDP) is a potential management strategy for patients with PNI and DP, but timing and outcomes associated with SDP have not been rigorously studied., Methods: Records from 49 tertiary United States pediatric hospitals in the Pediatric Health Information System from 2004 to 2018 were analyzed. The study cohort included patients diagnosed with BPP from birth trauma who were documented to have PNI or DP. Patients who underwent congenital cardiac operations were excluded., Results: A total of 5832 patients were identified with BPP from birth trauma during the study period, 122 (2%) of whom were found to have concomitant DP. Of those, 65 (53%) were male, 39 (32%) were infants of diabetic mothers, 80 (65%) required mechanical ventilation, and 33 (27%) underwent SDP. SDP was performed at a median (range) age of 36 (7-95) days. Median (range) total and postoperative hospital lengths of stay (LOS) were 34 (6-180) and 15 (4-132) days, respectively. There was also an observed increase in post-operative LOS with increase in age at operation., Conclusion: Neonatal DP is rare and is managed with SDP in a minority of instances. Age at repair affects total and postoperative length of stay, proxies for resource utilization and morbidity. Repair prior to 45 days of life appears to result in a shorter postoperative hospital stay. This analysis will help guide surgeons with respect to indications and operative timing for infant DP., Type of Study: Retrospective Comparative Study., Level of Evidence: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose of Review: Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects approximately 1 in 2,500 individuals globally [Ashizawa et al.: Neurol Clin Pract 2018;8(6):507-20]. In patients with DM1, respiratory muscle weakness frequently evolves, leading to respiratory failure as the main cause of death in this patient population, followed by cardiac complications [de Die-Smulders et al.: Brain 1998;121(Pt 8):1557-63], [Mathieu et al.: Neurology 1999;52(8):1658-62], [Groh et al.: Muscle Nerve 2011;43(5):648-51]. This paper provides a more detailed outline on the diagnostic and management protocols, which can guide pulmonologists who may not have experience with DM1 or who are not part of a neuromuscular multidisciplinary clinic. A group of neuromuscular experts in DM1 including pulmonologists, respiratory physiotherapists and sleep specialists discussed respiratory testing and management at baseline and during follow-up visits, based on their clinical experience with patients with DM1. The details are presented in this report., Recent Findings: Myotonic recruited 66 international clinicians experienced in the treatment of people living with DM1 to develop and publish consensus-based care recommendations targeting all body systems affected by this disease [Ashizawa et al.: Neurol Clin Pract. 2018;8(6):507-20]. Myotonic then worked with 12 international respiratory therapists, pulmonologists and neurologists with long-standing experience in DM respiratory care to develop consensus-based care recommendations for pulmonologists using a methodology called the Single Text Procedure. This process generated a 7-page document that provides detailed respiratory care recommendations for the management of patients living with DM1. This consensus is completely based on expert opinion and not backed up by empirical evidence due to limited clinical care data available for respiratory care management in DM patients. Nevertheless, we believe it is of relevance for professionals treating adults with myotonic dystrophy because it addresses practical issues related to respiratory management and care, which have been adapted to meet the specific issues in patients with DM1., Summary: The resulting recommendations are intended to improve respiratory care for the most vulnerable of DM1 patients and lower the risk of untoward respiratory complications and mortality by providing pulmonologist who are less experienced with DM1 with practical indications on which tests and when to perform them, adapting the general respiratory knowledge to specific issues related to this multiorgan disease., (© 2020 S. Karger AG, Basel.)

Diaphragmatic paralysis (DP) is a rare cause of respiratory distress in young children. In the first years of life, the main cause is phrenic nerve injury after cardiothoracic surgery or obstetrical trauma. DP usually presents as respiratory distress. Asymmetrical thorax elevation, difficulty weaning from mechanical ventilation, pulmonary atelectasis, and repeated pulmonary infections are other suggestive signs or complications. DP is usually suspected on chest X-ray showing abnormal hemidiaphragm elevation. Although fluoroscopy was considered the gold standard for DP confirmation, it has gradually been replaced by ultrasound, which can be done at the bedside. Some electrophysiological tools may be useful for a better characterization of phrenic nerve injury and chance of recovery. The management of DP is mainly based on clinical severity. In mild asymptomatic cases, DP may only require close monitoring. In more severe cases, adequate ventilatory support and/or surgical diaphragmatic plication may be needed. Electrophysiological tools may help clinicians assess the ideal timing for diaphragmatic plication., (© 2019 Wiley Periodicals, Inc.)

The diaphragm is the main breathing muscle and contraction of the diaphragm is vital for ventilation so any disease that interferes with diaphragmatic innervation, contractile muscle function, or mechanical coupling to the chest wall can cause diaphragm dysfunction. Diaphragm dysfunction is associated with dyspnoea, intolerance to exercise, sleep disturbances, hypersomnia, with a potential impact on survival. Diagnosis of diaphragm dysfunction is based on static and dynamic imaging tests (especially ultrasound) and pulmonary function and phrenic nerve stimulation tests. Treatment will depend on the symptoms and causes of the disease. The management of diaphragm dysfunction may include observation in asymptomatic patients with unilateral dysfunction, surgery (i.e., plication of the diaphragm), placement of a diaphragmatic pacemaker or invasive and/or non-invasive mechanical ventilation in symptomatic patients with bilateral paralysis of the diaphragm. This type of patient should be treated in experienced centres. This review aims to provide an overview of the problem, with special emphasis on the diseases that cause diaphragmatic dysfunction and the diagnostic and therapeutic procedures most commonly employed in clinical practice. The ultimate goal is to establish a standard of care for diaphragmatic dysfunction., (Copyright © 2018 Sociedade Portuguesa de Pneumologia. Published by Elsevier España, S.L.U. All rights reserved.)

Currently, more than 3000 patients in the Netherlands receive long-term ventilatory support. In the majority of patients, long-term ventilatory support leads to increased survival without any complications. Diaphragm pacing with an external pacemaker (diaphragm pacing system, DPS) seems an attractive alternative for long-term ventilatory support by mask or tracheostomy. Scientific research has since shown that DPS is effective in patients with high cervical paraplegia. In addition, patients with congenital central hypoventilation syndrome are also eligible for DPS. Patients with diaphragm paralysis are a new group of patients who may be eligible for DPS. Two European studies have shown that DPS should not be used in patients with amyotrophic lateral sclerosis. In our experience, patients are no longer completely dependent on a ventilator or may even be able to discontinue using one if the procedure was successful. In the Netherlands, as far as we know, the technique is only used at the University Medical Center Groningen.

Pseudocholinesterase deficiency is a rare genetic as well as an acquired disorder that affects the body's ability to metabolize choline esters such as succinylcholine and mivacurium. It can be inherited as an autosomal recessive trait, occurring in approximately 1 in 3,200 to 1 in 5,000 people. In most cases of pseudocholinesterase deficiency, no signs or symptoms of the condition exist. It is first suspected after a prolonged recovery from paralysis following general anesthesia in which succinylcholine or mivacurium are administered. We emphasize the importance of obtaining a detailed history prior to any endoscopic intervention or surgery requiring monitored anesthesia care or general anesthesia. In addition, a family history of anesthesia complications may help identify patients at risk. This article examines a case of a patient who underwent a successful endoscopic pneumatic dilation under general anesthesia for the treatment of achalasia, but was subsequently admitted to the intensive care unit, requiring mechanical ventilator support, for 18 hours. The patient made a complete recovery and was discharged home with no further complications. This case stimulated a review of this entity and we provide recommendations relevant to all proceduralist and anesthesia staff, as well as all personnel involved in procedures., (Copyright © 2018. Published by Elsevier Inc.)

The aim of this paper is to highlight the symptomatology in three Conium maculatum intoxication incidents, one of which was fatal. A number of studies were reviewed in order to update and summarize the relevant literature on the incidence, sociodemographic variables, method of poisoning, pathophysiology, diagnosis, variables associated with survival and fatality, management, and treatment of C. maculatum intoxication as well as the biosynthesis and biological effects of poison hemlock alkaloids. Results show that hemlock poisoning is relatively rare, although incidence varies in different regions, despite its worldwide distribution. Hemlock poisoning is more common in European and especially Mediterranean countries. The majority of the patients are adult males over 38 years of age. The clinical course of hemlock poisoning includes neurotoxicosis, tremor, vomiting, muscle paralysis, respiratory paralysis/failure, rhabdomyolysis, and acute renal failure. The therapeutic management focuses on absorption reduction, close observation for complications, and supportive therapy (especially for respiration). Acute occurrence is severe and life-threatening, but the survival rate is high if treatment is provided promptly. Recovery is rapid, generally taking only a few days., (Copyright © 2019 Central Police University.)

Aim of Study: The repair of esophageal atresia (EA) carries an increased risk of anastomotic leak and stricture formation, especially in patients with anastomotic tension. To minimize this risk, pediatric surgeons perform elective post-operative muscle paralysis, positive-pressure ventilation, and head flexion (PVF) to reduce movement and tension at the anastomosis. We systematically reviewed and analyzed the effect of post-operative PVF on reducing anastomotic complications., Methods: Embase, MEDLINE, Web of Science, and PubMed databases were used to conduct searches. Articles reporting pediatric EA undergoing primary anastomosis, anastomotic complications, and comparisons between patients who received post-operative PVF to those who did not were included. Odds ratios (OR) for all post-operative anastomotic complications were calculated using random effects modelling., Main Results: Three of the 2268 papers retrieved met inclusion criteria (all retrospective cohort studies). There were no randomized controlled trials. Post-operative PVF showed a significant reduction in anastomotic leak (OR 0.07; 95% CI 0.01-0.35) when compared to no PVF. Stricture formation was not statistically different between groups. Potential sources of bias include patient allocation., Conclusions: Based on available data, our analysis indicates PVF may reduce anastomotic post-operative leak. To confirm these results, a prospective study with clearer definitions of treatment allocation should be performed.

The diaphragm is the primary muscle of inspiration. Its capacity to respond to the load imposed by pulmonary disease is a major determining factor both in the onset of ventilatory failure and in the ability to successfully separate patients from ventilator support. It has recently been established that a very large proportion of critically ill patients exhibit major weakness of the diaphragm, which is associated with poor clinical outcomes. The two greatest risk factors for the development of diaphragm weakness in critical illness are the use of mechanical ventilation and the presence of sepsis. Loss of force production by the diaphragm under these conditions is caused by a combination of defective contractility and reduced diaphragm muscle mass. Importantly, many of the same molecular mechanisms are implicated in the diaphragm dysfunction associated with both mechanical ventilation and sepsis. This review outlines the primary cellular mechanisms identified thus far at the nexus of diaphragm dysfunction associated with mechanical ventilation and/or sepsis, and explores the potential for treatment or prevention of diaphragm weakness in critically ill patients through therapeutic manipulation of these final common pathway targets., (Copyright © 2018 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

In this case report, we describe the course of a female patient who is known to have neuromyelitis optica (NMO) and developed left hemiparesis and unilateral diaphragmatic paralysis. She was initially treated with intravenous methylprednisolone 1 g daily for 5 days without improvement. Subsequently, she received five sessions of a plasmapheresis. Her hemiparesis had improved after few days. however, the phrenic nerve palsy remained., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

We report a preterm neonate born with respiratory distress. The neonate was found to have diaphragmatic palsy and brachial palsy. The neonate was born by caesarean section and there was no history of birth trauma. On examination, there was bilateral congenital talipes equinovarus and a scar was present on the forearm. The mother had a history of chickenpox during the 16 weeks of pregnancy for which no treatment was sought. On investigation, PCR for varicella was found to be positive in the neonate., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

We present a case reported on the SENSAR database. A patient with a spinal infusion pump was admitted for reservoir refill. On administration of 22ml of 0.75% bupivacaine the patient suffered a total spinal block with widespread loss strength and respiratory arrest. The patient required emergency orotracheal intubation, mechanical ventilation and admission to ICU, where extubation was achieved within two hours without incidences. At a later stage it was stated that the local anaesthetic had been administered via the access port for bolus or contrast administration instead of via the access to the reservoir. Analysis of the incident showed up latent factors related to absence lack of personnel training and internal protocols. The following measures were taken: pain unit meeting, alert sent to SENSAR bulletin and training request for members of the service., (Copyright © 2017 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.)

Interscalene block (ISB) impairs ipsilateral lung function and generally is not used for patients with respiratory insufficiency. We present a 49-year-old man with chronic obstructive pulmonary disease scheduled for shoulder surgery. He was given a regional technique with an ISB (short-acting local anesthetic to minimize duration of diaphragmatic dysfunction) and suprascapular and axillary nerves blocks (long-acting local anesthetic). He was supported with noninvasive ventilation during the time of hemidiaphragmatic paralysis as documented by serial ultrasound examination. A discussion about ISB and its alternatives (general anesthesia versus brachial plexus block versus selective peripheral nerve blocks) always should occur for patients at risk for pulmonary complications.

Neuralgic Amyotrophy (NA) or Parsonage-Turner syndrome is an idiopathic neuropathy commonly affecting the brachial plexus. Associated phrenic nerve involvement, though recognised, is thought to be very rare. We present a case series of four patients (all male, mean age 53) presenting with dyspnoea preceded by severe self-limiting upper limb and shoulder pain, with an elevated hemi-diaphragm on clinical examination and chest X-ray. Neurological examination of the upper limb at the time of presentation was normal. Diaphragmatic fluoroscopy confirmed unilateral diaphragmatic paralysis. Pulmonary function testing demonstrated characteristic reduction in forced vital capacity between supine and sitting position (mean 50%, range 42-65% predicted, mean change 23%, range 22-46%), reduced maximal inspiratory pressures (mean 61%, range 43-86% predicted), reduced sniff nasal inspiratory pressure (mean 88.25, range 66-109 cm H 2 O) and preserved maximal expiratory pressure (mean 107%, range 83-130% predicted). Phrenic nerve conduction studies confirmed phrenic nerve palsy. All patients were managed conservatively. Follow-up ranged from 6 months to 3 years. Symptoms and lung function variables normalised in three patients and improved significantly in the fourth. The classic history of severe ipsilateral shoulder and upper limb neuromuscular pain should be elicited and thus NA considered in the differential for a unilateral diaphragmatic paralysis, even in the absence of neurological signs. Parsonage-Turner syndrome is likely to represent a significantly under-diagnosed aetiology of phrenic nerve palsy. Conservative management as opposed to surgical intervention is advocated as most patients demonstrate gradual resolution over time in this case series.

Objective To investigate the clinical feasibility of the injection test for balloon placement during oesophageal pressure measurement in patients without spontaneous breathing. Methods The injection test was performed in 12 mechanically ventilated patients under deep sedation and paralysis. During withdrawal of the balloon from the stomach and air injection into the gastric lumen of the catheter, the presence of the injection test wave in the balloon pressure tracing indicated that the whole balloon was positioned above the lower oesophageal sphincter (LES). The positive pressure occlusion test was performed at different balloon positions. Results In each patient, the injection test wave appeared at a distinct balloon depth, with a mean ± standard deviation of 41.9 ± 3.3 cm and range from 37 cm to 47 cm. The optimal ratio of changes in the balloon and airway pressure (0.8-1.2) during the positive pressure occlusion test was obtained when the balloon was located 5 cm and 10 cm above the LES in nine (75%) and three (25%) patients, respectively. Conclusions The injection test is feasible for identification of the whole balloon position above the LES during passive ventilation. The middle third of the oesophagus might be the optimal balloon position.

Background Diaphragmatic paralysis (DP) is a rare disease, usually secondary to systemic processes, although idiopathic forms have been described. Aim To describe the use of non-invasive ventilation as a treatment to patients with DP. Material and methods Descriptive study about consecutive cases of DP in a general hospital. Results It has been described 4 patients, all of them with a diminished the maximum mouth pressures and vital capacity in supine position. Three patients presented hypercapnia, and one of them, required intubation with invasive mechanical ventilation. All patients were treated with non-invasive positive pressure (NPPV) mode S/T bilevel, allowing with a reduction in the PCO2, improvement in the symptoms and in the parameters to the nocturnal respiratory polygraphy. Conclusions Use of NPPV in patients with DP could provide a clinical improvement and contributing to recovery of lung functionality that make it recommendable in selected cases.

Diaphragmatic paralysis is a rare cause of respiratory distress in the newborn. In this paper, a patient with unilateral phrenic nerve injury after traumatic delivery is presented. The child inadequately responded to standard respiratory supportive measures. Non-invasive neurally adjusted ventilatory assist (NIV-NAVA®), providing an optimally synchronized respiratory support proportional to the effort of the patient, resulted in prompt clinical and biological improvement of the patient's respiratory condition. NAVA is a relatively new mode of ventilation in neonatal care. In this case of unilateral diaphragmatic paralysis, it provided an alternative strategy of non-invasive respiratory support avoiding prolonged mechanical ventilation. Pediatr Pulmonol. 2016;51:E37-E39. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

There are many reasons for an impairment of the diaphragmatic function potentially affecting all components of the respiratory pump. Particularly, diagnosis and treatment of unilateral and bilateral phrenic nerve paralysis are challenging. Neuromuscular disorders, trauma, iatrogenic conditions, tumor compression, but also infectious and inflammatory conditions in addition to neuralgic amyotrophy and idiopathic phrenic nerve paralysis are reasons for phrenic nerve paralysis. Primarily, diagnostic procedures include the anamnesis, physical examination, blood gas analysis, lung function testing and the diagnosis of the underlying disease. In addition, specific respiratory muscle testing and respiratory imaging are available today. Current established treatment options include respiratory muscle training, long-term non-invasive ventilation and surgical diaphragm plication in selected patients., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background and Aims: To evaluate the outcome of patients with Guillain -: Barre syndrome (GBS) having respiratory failure treated with modified intubation policy., Design and Methods: Consecutive patients with GBS having single breath count below 12 and respiratory rate >30/min were included and their clinical details noted. The patients were intubated and mechanically ventilated (MV) if their PaO2 was <60 mmHg on venturi mask, PaCo2 > 50 mmHg or pH < 7.3. Their electrophysiological subtypes and complications were noted. The hospital mortality and 3 months outcome were compared in MV and those could be managed without MV even with respiratory compromise., Results: Out of 369 patients, 102 (27.6%) patients had respiratory compromise who were included in this study. Of the patients with respiratory compromise, 44 (43.1%) were intubated and mechanically ventilated after a median of 4 days of hospitalization. The median duration of MV was 21 (range 1-88) days. The patients with autonomic dysfunction (56.8% vs. 19%), facial weakness (78% vs. 36.2%), bulbar weakness (81.8% vs. 31%), severe weakness (63.8% vs. 31%) and high transaminase level (47.7% vs. 25.9%) needed MV more frequently. In our study, 6.8% patients died and 26.6% had poor outcome which was similar between MV and non-MV patients. The MV patients had longer hospitalization and more complications compared with non-MV group., Conclusion: In GBS patients with respiratory compromise, conservative intubation does not increase mortality and disability., (© The Author 2015. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Purpose of Review: Neurologists working in the hospital are often called to evaluate patients with severe muscle weakness. Some of these patients can develop ventilatory compromise and require admission to the intensive care unit (ICU). This article reviews the general evaluation of neuromuscular respiratory failure, discusses its differential diagnosis, and provides practical advice on the management of its most common causes., Recent Findings: Determining the cause of acute neuromuscular respiratory failure is crucial because functional prognosis is poor in patients for whom the cause cannot be defined. The differential diagnosis is extensive, but the first step is to discriminate between cases related to a primary neurologic disease (primary neuromuscular respiratory failure) and those provoked by systemic disease, most often critical illness from sepsis and multiorgan failure (secondary neuromuscular respiratory failure). Guillain-Barré syndrome (GBS) and myasthenic crisis are the two most frequent causes of primary neuromuscular respiratory failure. Although they are both autoimmune conditions that benefit from the administration of plasma exchange or IV immunoglobulin (IVIg), they are otherwise very different disorders with unique features and distinct complications. Optimal strategies for mechanical ventilation also differ between these two conditions; while myasthenic crisis is ideally managed with noninvasive bilevel positive airway pressure (BiPAP) ventilation, GBS demands early intubation., Summary: Prompt recognition of neuromuscular respiratory failure can be lifesaving, and identification of its cause has substantial prognostic implications. Adequate management of these patients requires a multidisciplinary team with the neurologist at its center, not only to guide the diagnostic evaluation but often also to prescribe the optimal management.

Amyotrophic neuralgia is an uncommon neuropathy characterized by severe unilateral shoulder pain. Isolated or concomitant involvement of other peripheral motor nerves depending on the brachial plexus such as phrenic or laryngeal nerves is unusual(1). Its etiology is unknown, yet several explanatory factors have been proposed. Phrenic nerve involvement, either unilateral or bilateral, is exceedingly rare. Diagnosis relies on anamnesis, functional and imaging investigations and electromyogram. We report the case of a 48-year-old woman with a past history of renal transplantation due to proliferative glomerulonephritis with subsequent transplant rejection, who was eventually diagnosed with amyotrophic neuralgia with bilateral phrenic involvement, and who required sustained non-invasive mechanical ventilation., (Copyright © 2015 Elsevier España, S.L.U. All rights reserved.)

Bilateral diaphragmatic paralysis (BDP) manifests as respiratory muscle weakness, and its association with critical illness polyneuropathy (CIP) was rarely reported. Here, we present a patient with BDP related to CIP, who successfully avoided tracheostomy after diagnosis and management.A 71-year-old male presented with acute respiratory failure after sepsis adequately treated. Repeated intubation occurred because of carbon dioxide retention after each extubation. After eliminating possible factors, septic shock-induced respiratory muscle weakness was suspected. Physical examination, a nerve conduction study, and chest ultrasound confirmed our impression.Pulmonary rehabilitation and reconditioning exercises were arranged, and the patient was discharged with a diagnosis of BDP.The diagnosis of BDP is usually delayed, and there are only sporadic reports on its association with polyneuropathy, especially in patients with preserved limb muscle function. Therefore, when physicians encounter patients that are difficult to wean from mechanical ventilation, CIP associated with BDP should be considered in the differential diagnosis.

Background: Diaphragm dysfunction (DD) can cause sleep abnormalities, dyspnea, atelectasis, and respiratory failure. Historical treatments, including positive pressure ventilation or diaphragm plication, may alleviate symptoms but do not restore physiologic diaphragm function. Diaphragm pacing (DP) is approved for spinal cord-injured patients and in amyotrophic lateral sclerosis. We report a series of DD patients undergoing use of DP outside of these initial indications., Methods: This report involves a prospective, nonrandomized, interventional trial under institutional review board approval at a single institution. DP involves laparoscopic motor point mapping with implantation of intramuscular electrodes in each hemidiaphragm. Postoperatively, diaphragm conditioning ensues., Results: Twenty-seven patients were evaluated; all patients had symptomatic and objective hypoventilation for an average of 36 months of symptoms. Causes included idiopathic (n = 13), chest surgery (n = 5), shoulder surgery or trauma (n = 6), and others (n = 3); 17 had bilateral involvement, 6 had nonstimulable diaphragms and were not implanted, and 21 were implanted. Thirteen (62%) had substantial clinically relevant respiratory improvements. Four ventilator patients were weaned completely. Four had partial improvement, 3 had no improvement, and 1 patient was lost to follow-up for objective analysis., Conclusion: This is the first report of DP being used to treat diverse causes of DD. Eighty-one percent of implanted patients experienced improvements. This success suggests a potential for a wider use of DP and areas for future research., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Mutations in the butyrylcholinesterase gene can lead to a prolonged effect of the neuromuscular blocking agents, succinylcholine and mivacurium. If the anaesthesiologist is not aware of this condition, it may result in insufficient respiration after tracheal extubation. However, this can be avoided with the use of objective neuromuscular monitoring if used adequately. Three case reports of prolonged effect of succinylcholine or mivacurium were presented to illustrate the importance of neuromuscular monitoring during anaesthesia. In the first case, continuous intraoperative neuromuscular monitoring allowed a prolonged neuromuscular blockade to be discovered prior to tracheal extubation of the patient. The patient was extubated after successful reversal of the neuromuscular blockade. On the contrary, neuromuscular monitoring was not used during anaesthesia in the second patient; hence, the prolonged effect of the neuromuscular blocking agent was not discovered until after extubation. In the third patient, the lack of response to nerve stimulation was interpreted as a technical failure and the prolonged effect of succinylcholine was discovered when general anaesthesia was terminated. Both patients had insufficient respiration. They were therefore re-sedated, transferred to the intensive care unit and the tracheas were extubated after full recovery from neuromuscular blockade. We recommend the use of monitoring every time these agents are used, even with short-acting drugs like succinylcholine and mivacurium., (© 2014 The Acta Anaesthesiologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

A completely different treatment approach was chosen for 2 patients with unilateral diaphragmatic paralysis and complaints of dyspnea despite similar anatomic and physiologic abnormalities. These decisions were supported by results obtained by functional respiratory imaging (FRI). FRI generated functional information on lobar ventilation and local drug deposition. In the first patient, some lobes were poorly ventilated, and drug deposition simulation showed that some regions were undertreated. This patient underwent diaphragmatic plication to restore ventilation. In the second patient, all lobes were still ventilated. A conservative approach with regular follow-ups was chosen to wait for spontaneous recovery of the diaphragmatic function. Both patients improved subjectively and objectively. These cases demonstrate how novel medical imaging techniques such as FRI can be used to personalize respiratory treatment in patients with unilateral diaphragmatic paralysis., (Copyright © 2014 by Daedalus Enterprises.)

Pufferfish poisoning has rarely been reported in the southwestern Indian Ocean and in the French overseas territories. In Reunion Island, the last notified documented case occurred in 1989 and people are no longer aware of the potential toxicity of pufferfish. We report a family hospitalized for a tetrodotoxin poisoning following the consumption of Lagocephalus sceleratus caught on the coast of Reunion Island in September 2013. Two patients presenting acute vital functions failures were admitted in an ICU. Ten people were admitted simultaneously to the emergency department after consuming L. sceleratus with signs of toxicity appearing within 2 hours. Treatment was supportive, but included the need for mechanical ventilation for two patients. All those affected had complete and uneventful recoveries within a few days. The fish consumed was identified as L. sceleratus, a species known to contain tetrodotoxin. The diagnosis of tetrodotoxin poisoning was suggested by typical clinical manifestations together with the history of very recent consumption of tetrodotoxin-containing fish. Tetrodotoxin was later detected at high levels in food remnants. To the best of our knowledge, there has been no documented case series of tetrodotoxin poisoning reported from Reunion Island for the last 25 years and from the entire Indian Ocean area since 1998. Pufferfish intoxication is one of the most common causes of poisoning among people in coastal regions of Asia but it has also recently been reported in areas where it was previously unknown, particularly along the Mediterranean shores and in Spain. Public health education in French overseas territories and along the Mediterranean shores should be adapted to include increased awareness of the danger of consuming pufferfish. Health teams must be aware of such clinical presentations.

Background: Mechanical insufflation-exsufflation (MI-E), more commonly known as 'cough assist therapy', is a method which produces inspiratory and expiratory assistance to improve cough performances. However, other alternatives or combinations are possible., Objective: The objective was to compare the effects of mechanical insufflation combined with manually assisted coughing (MAC), insufflation-exsufflation alone and insufflation-exsufflation combined with MAC in neuromuscular patients requiring cough assistance., Methods: Eighteen neuromuscular patients with severe respiratory muscle dysfunction and peak cough flow (PCF) lower than 3 liters/s or maximal expiratory pressure (MEP) lower than +45 cm H2O were studied. Patients were studied under three cough-assisted conditions, which were used in random order: insufflation by intermittent positive-pressure breathing (IPPB) combined with MAC, MI-E and MI-E + MAC., Results: Overall, PCF was higher with IPPB + MAC than with MI-E + MAC or MI-E alone. Among the 12 patients who had higher PCF values with IPPB + MAC than with the two other techniques, 9 exhibited mask pressure swings during MI-E exsufflation, with a transient positive-pressure value due to the expiratory flow produced by the combined patient cough effort and MAC. Each of these 9 patients had higher PCF values (>5 liters/s) than did the other 9 patients when using IPPB + MAC., Conclusion: Our results indicate that adding the MI-E device to MAC is unhelpful in patients whose PCF with an insufflation technique and MAC exceeds 5 liters/s. This is because the expiratory flow produced by the patient's effort and MAC transitorily exceeds the vacuum capacity of the MI-E device, which therefore becomes a transient load against the PCF.