Electrodiagnostic and ultrasound evaluation of respiratory weakness.

Phrenic nerve conduction studies (NCSs) and needle electromyography (EMG) can provide important information on the underlying pathophysiology in patients presenting with unexplained shortness of breath, failure to wean from the ventilator, or consideration of phrenic nerve pacemaker implantation. However, these techniques are often technically challenging, require experience, can lack sensitivity and specificity, and, in the case of diaphragm EMG, involve some degree of risk. Diagnostic high-resolution ultrasound has been introduced in recent years as an adjuvant technique readily available at the bedside that can increase the overall sensitivity and specificity of the neurophysiologic evaluation of respiratory symptoms. Two-dimensional ultrasound in the zone of apposition can identify atrophy and evaluate contractility of the diaphragm, in addition to localizing a safe zone for needle EMG. M-mode ultrasound can identify decreased excursion or paradoxical motion of the diaphragm and can increase the reliability of phrenic NCSs. When used in combination, ultrasound, phrenic NCSs and EMG of the diaphragm can differentiate neuropathic, myopathic, and central disorders, and can offer aid in prognosis that is difficult to arrive at solely from clinical examination. This article will review techniques to successfully perform phrenic NCSs, needle EMG of the diaphragm, and ultrasound of the diaphragm. The discussion will include technical pitfalls and clinical pearls as well as future directions and clinical indications.

Ultrasonic detection of vagus, accessory, and phrenic nerve atrophy in amyotrophic lateral sclerosis: Relation to impairment and mortality.

BACKGROUND AND PURPOSE: In amyotrophic lateral sclerosis (ALS), phrenic nerve (PN) atrophy has been found, whereas there is controversy regarding vagus nerve (VN) atrophy. Here, we aimed to find out whether PN atrophy is related to respiratory function and 12-month survival. Moreover, we investigated the relevance of VN and spinal accessory nerve (AN) atrophy in ALS. METHODS: This prospective observational monocentric study included 80 adult participants (40 ALS patients, 40 age- and sex-matched controls). The cross-sectional area (CSA) of bilateral cervical VN, AN, and PN was measured on high-resolution ultrasonography. Clinical assessments included the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R), the Non-Motor Symptoms Questionnaire, and handheld spirometry of forced vital capacity (FVC). One-year survival was documented. RESULTS: The CSA of each nerve, VN, AN, and PN, was smaller in ALS patients compared to controls. VN atrophy was unrelated to nonmotor symptom scores. PN CSA correlated with the respiratory subscore of the ALSFRS-R (Spearman test, r = 0.59, p < 0.001), the supine FVC (r = 0.71, p < 0.001), and the relative change of sitting-supine FVC (r = -0.64, p = 0.001). Respiratory impairment was predicted by bilateral mean PN CSA (p = 0.046, optimum cutoff value of ≤0.37 mm2 , sensitivity = 92%, specificity = 56%) and by the sum of PN and AN CSA (p = 0.036). The combination of ALSFRS-R score with PN and AN CSA measures predicted 1-year survival with similar accuracy as the combination of ALSFRS-R score and FVC. CONCLUSIONS: Ultrasonography detects degeneration of cranial nerve motor fibers. PN and AN calibers are tightly related to respiratory function and 1-year survival in ALS.

Successful termination of persistent hiccups via combined ultrasound and nerve stimulator-guided singular phrenic nerve block: a case report and literature review.

Persistent hiccups that occur after abdominal surgery seriously affect postoperative rehabilitation. Phrenic nerve block therapy has been recommended after failure of medication or physical maneuvers. However, the phrenic nerve is often difficult to accurately identify because of its small diameter and anatomic variations. We combined ultrasound with the use of a nerve stimulator to quickly and accurately identify and block the phrenic nerve in a patient with postoperative persistent hiccups. The ongoing hiccups were immediately terminated with no adverse effects. The patient reported no recurrence during the 2-week follow-up period. We conclude that the combined use of real-time ultrasound guidance and a nerve stimulator for singular phrenic nerve block might be an effective intervention for terminating postoperative persistent hiccups, although further studies are needed to evaluate the safety and efficacy of this technique. The findings in this case suggest a potential clinical application for this technique in managing persistent hiccups, thereby contributing to improved patient care and outcomes.

Contrast-enhanced computed tomography in the venous rather than the arterial phase is essential for the evaluation of the right phrenic nerve.

BACKGROUND: Preprocedural detection of the running course of the right pericardiophrenic bundles (PBs) is considered to be useful in preventing phrenic nerve (PN) injury during catheter ablation for atrial fibrillation (AF). However, previous studies using the arterial phase of contrast-enhanced computed tomography (CT) reported a relatively low right PBs detection rate. METHODS: This study included 63 patients with AF who underwent catheter ablation and preoperative contrast-enhanced CT imaging of the venous and arterial phases (66.7 ± 10.2 years; 44 male). The venous phase of contrast-enhanced CT significantly improved the detection rate of PBs compared to the arterial phase (96.8% vs. 60.3%, p < .001), and PBs were detected in the venous phase only in 23 (36.7%) patients. No significant differences were observed between the right PBs detection rate using non-contrast CT versus the arterial phase of contrast-enhanced CT (p = .37). Patients without visualization of the right PBs during the arterial phase had a higher frequency of chronic heart failure (p = .0083), lower left ventricular ejection fraction (p = .021), and a higher CHADS2 score (p = .048) than those with visualization. In five patients whose right PBs could only be detected during the venous phase of contrast-enhanced CT, the reconstructed running course of the right PBs corresponded with the PN generated by electrical high-output pacing. CONCLUSION: Contrast-enhanced CT images of the venous phase, rather than the arterial phase, are useful in detecting the right PBs, especially in patients with heart failure or reduced left ventricular ejection fraction.

Ultrasound-guided bilateral pulsed radiofrequency of the phrenic nerve for prolonged hiccups: a case report.

Singultus is the sudden onset of erratic diaphragmatic and intercostal muscle contraction immediately followed by laryngeal closure. Pathophysiology involves afferent, central and efferent components. Bilateral phrenic nerve block was performed to a 46-year-old woman with a brain tumor with persistent hiccups, with initially positive response but later symptom recurrence. Bilateral pulsed radiofrequency of the phrenic nerve was performed guided by ultrasonography (US). In the follow-up, absence of hiccups was confirmed. The patient was discharged 24 h later. Persistent or untreatable singultus is an infrequent condition that should not be dismissed. This approach is a safe, accurate and effective therapeutic approach for patient's refractory to conservative treatment. Further studies are needed to establish safety and effectiveness of the treatment.

Ultrasound imaging of the phrenic nerve at the scalene muscle level.

The aim of this study is to utilize ultrasound to evaluate the normal cross-sectional area (CSA) of the phrenic nerve (PN), at the level of the anterior scalene muscle. The study included 62 PNs in 31 healthy subjects (13 men, 18 women); mean age, 36.6 years; mean height, 161.1 cm; mean weight, 69.6 kg; and mean body mass index 25.8 kg/m2. High-resolution ultrasound images of the bilateral PNs were obtained by a radiologist with 15 years of experience in neuromusculoskeletal ultrasound. Three separate CSA measurements for the bilateral PNs bilaterally were obtained. Images were also reviewed by an experienced neurologist to evaluate for inter-rater variability. The mean CSA of the right PN was 0.54 mm2 ± 0.16. The mean CSA of the left PN was 0.53 mm2 ± 0.18. We believe that the reference values for the normal CSA of the PNs obtained in our study could help in the sonographic evaluation of PN enlargement, as it relates to the diagnosis of various diseases affecting the PN. Furthermore, knowledge of its location and size, at the level of the scalene muscle, could help prevent PN-related complications during interventional procedures in that area. Additionally, for each participant, demographic information of age and gender as well as body mass index, weight, and height were documented.

The diagnostic utility of inspiratory-expiratory radiography for the assessment of phrenic nerve palsy associated with brachial plexus injury.

BACKGROUND: The phrenic nerve is commonly injured with trauma to the brachial plexus. Hemi-diaphragmatic paralysis may be well-compensated in healthy individuals at rest but can be associated with persistent exercise intolerance in some patients. This study aims to determine the diagnostic value of inspiratory-expiratory chest radiography compared to intraoperative stimulation of the phrenic nerve for assessing phrenic nerve injury associated with brachial plexus injury. METHODS: Over a 21-year period, the diagnostic utility of three-view inspiratory-expiratory chest radiography for identification of phrenic nerve injury was determined by comparison to intraoperative phrenic nerve stimulation. Multivariate regression analysis was used to identify independent predictors of phrenic nerve injury and having an incorrect radiographic diagnosis. RESULTS: A total of 237 patients with inspiratory-expiratory chest radiography underwent intraoperative testing of phrenic nerve function. Phrenic nerve injury was present in approximately one-fourth of cases. Preoperative chest radiography had a sensitivity of 56%, specificity of 93%, positive predictive negative of 75%, and negative predictive value of 86% for identification of a phrenic nerve palsy. Only C5 avulsion was found to be a predictor of having an incorrect diagnosis of phrenic nerve injury on radiography. CONCLUSION: While inspiratory-expiratory chest radiography has good specificity for detecting phrenic nerve injuries, a high number of false negatives suggest that it should not be relied upon for routine screening of dysfunction after traumatic brachial plexus injury. This is likely multifactorial and relates to variation in diaphragm shape and position, as well as limitations regarding static image interpretation of a dynamic process.

Phrenic Nerve Dysfunction Secondary to Cervical Neuroforaminal Stenosis: A Literature Review.

BACKGROUND: Phrenic nerve dysfunction has been associated with cervical neuroforaminal stenosis in limited case reports and case-controlled studies. It is unclear if magnetic resonance imaging of the cervical spine should be included in the workup of patients with pulmonary dysfunction. A systematic review of the current literature was conducted on the topic to provide an outline of the body of knowledge and some guidance for neurosurgeons that receive these patient referrals. METHODS: A systematic literature review was conducted through the PubMed database to identify articles related to phrenic nerve dysfunction secondary to cervical stenosis. RESULTS: A total of 12 case reports were found. The median subject age was 64 years, 11 were male. Presenting symptoms included shortness of breath (n = 9), radiculopathy (n = 7), myelopathy (n = 5), reduced pulmonary function (n = 6), weakness (n = 4), and neck pain (n = 5). Ten of these patients underwent surgical intervention, all having improvements in their pulmonary and neurological symptoms at follow-up ranging from 10 days to 2 years. CONCLUSIONS: Cervical stenosis, resulting in neuroforaminal stenosis, may be related to phrenic nerve dysfunction in select patients with idiopathic diaphragmatic paralysis or pulmonary dysfunction. Surgical decompression improves pulmonary and neurological symptoms.

Ultrasound-guided selective trunk block (SeTB): a cadaver anatomic study to evaluate the spread of dye after a simulated injection.

BACKGROUND AND OBJECTIVES: Recent reports suggest that a selective trunk block (SeTB) can produce sensorimotor blockade of the entire upper extremity, except for the T2 dermatome. There are no data demonstrating the anatomic mechanism of SeTB. This cadaver study aimed to evaluate the spread of an injectate after a simulated ultrasound-guided (USG) SeTB. METHODS: USG SeTB (n=7) was performed on both sides of the neck in four adult human cadavers with 25 mL of 0.1% methylene blue dye. Anatomic dissection was performed to document staining (deep, faint, and no stain) of the various elements of the brachial plexus from the level of the roots to the cords, including the phrenic, dorsal scapular, and long thoracic nerves. Only structures that were deeply stained were defined as being affected by the SeTB. RESULTS: All the trunks and divisions of the brachial plexus, as well as the ventral rami of C5-C7 and suprascapular nerve, were deeply stained in all (100%) the simulated injections. The ventral rami of C8 and T1 (86%), dorsal scapular and long thoracic nerve (71%), and the phrenic nerve (57%) were also deeply stained in a substantial number of the injections. CONCLUSION: This cadaver study demonstrates that an USG SeTB consistently affects all the trunks and divisions of the brachial plexus, as well as the suprascapular nerve. This study also establishes that SeTB may not be phrenic nerve sparing. Future research to evaluate the safety and efficacy of SeTB as an all-purpose brachial plexus block technique for upper extremity surgery is warranted. TRIAL REGISTRATION NUMBER: Registered at https://www.thaiclinicaltrials.org on December 13, 2021 under the trial registration number TCTR20211213005.

Diaphragm and Phrenic Nerve Ultrasound in COVID-19 Patients and Beyond: Imaging Technique, Findings, and Clinical Applications.

The diaphragm, the principle muscle of inspiration, is an under-recognized contributor to respiratory disease. Dysfunction of the diaphragm can occur secondary to lung disease, prolonged ventilation, phrenic nerve injury, neuromuscular disease, and central nervous system pathology. In light of the global pandemic of coronavirus disease 2019 (COVID-19), there has been growing interest in the utility of ultrasound for evaluation of respiratory symptoms including lung and diaphragm sonography. Diaphragm ultrasound can be utilized to diagnose diaphragm dysfunction, assess severity of dysfunction, and monitor disease progression. This article reviews diaphragm and phrenic nerve ultrasound and describes clinical applications in the context of COVID-19.

The many paths to diagnose diaphragmatic paralysis: Cardiopulmonary ultrasound on stage.

Abnormal diaphragmatic motion (ADM) due to phrenic nerve injury is a recognized complication of cardiac surgery and several diagnostic techniques can be used to determine the diagnosis. Due to its relationship with the diaphragm, cardiac kinetics is affected by the abnormal movement of the diaphragm in cases of left hemidiaphragm paralysis. The authors present a case of diaphragmatic paralysis in which the initial diagnosis is made through echocardiography.

Radiologic Manifestations of Pulmonary Vein Ablation Complications: A Pictorial Review.

In patients with atrial fibrillation refractory to drug therapy and cardioversion, pulmonary vein ablation is an alternative treatment that eradicates arrhythmogenic activity originating in the muscles of the pulmonary veins. While this procedure has a low incidence of significant complications, iatrogenic injuries are possible. Through multimodality pictorial examples utilizing computed tomography, nuclear medicine, fluoroscopy, and chest radiographs, the complications associated with pulmonary vein ablation will be reviewed. Examples of pulmonary vein stenosis, right phrenic nerve injury with associated diaphragmatic paralysis, atrioesophageal fistula, and pericardioesophageal fistula will be illustrated.

Utility of phrenic nerve ultrasound in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting the upper and lower motor neurons causing progressive weakness. It eventually involves the diaphragm which leads to respiratory paralysis and subsequently death. Phrenic nerve (PN) conduction studies and diaphragm ultrasound has been studied and correlated with pulmonary function tests in ALS patients. However, PN ultrasonography has not been employed in ALS. This study aims to sonographically evaluate the morphologic appearance of the PN of ALS patients. Thirty-eight ALS patients and 28 normal controls referred to the neurophysiology laboratory of two institutions were retrospectively included in the study. Baseline demographic and clinical variables such as disease duration, ALS Functional Rating Scale-Revised score, and ALS region of onset were collected. Ultrasound was used to evaluate the PN cross-sectional area (CSA) of ALS and control subjects. The mean PN CSA of ALS patients were 1.08 ± 0.39 mm on the right and 1.02 ± 0.34 mm on the left. The PN CSA of ALS patients were significantly decreased compared to controls (p value < 0.00001). The PN CSA of ALS patients was not correlated to any of the demographic and clinical parameters tested. This study demonstrates that ALS patients have a smaller PN size compared to controls using ultrasonography.

Evaluating the spread of costoclavicular brachial plexus block: an anatomical study.

BACKGROUND AND OBJECTIVES: The costoclavicular brachial plexus block is performed deep and posterior to the midpoint of the clavicle. There are limited data evaluating the spread of the costoclavicular brachial plexus block. We performed a cadaveric study to evaluate the spread of injectate after a costoclavicular brachial plexus block. METHODS: Five ultrasound-guided costoclavicular block injections were performed with 20 mL of 0.1% methylene blue. The brachial plexus and its branches were dissected from the level of C4 to the lower axilla. The extent of dye spread was recorded including spread to the phrenic nerve, suprascapular nerve, roots, trunks, divisions, cords and terminal branches of the brachial plexus. RESULTS: The dye extended cephalad to the level of the cricoid cartilage in two of the five injections; three injections had dye extending 0.75 cm, 1.5 cm and 2 cm caudad to the level of the cricoid cartilage, respectively. The C7, C8 and T1 nerve roots were stained in all injections. The dye did not extend cephalad to the C5 and C6 nerve roots. All trunks, cords and divisions of the brachial plexus were stained, as was the suprascapular nerve. There was no spread of dye to the phrenic nerve in any of the specimens. CONCLUSIONS: This cadaveric study demonstrates that ultrasound-guided injection in the costoclavicular space spreads cephalad to the brachial plexus in the supraclavicular space, consistently reaching the suprascapular nerve and all trunks and cords of the brachial plexus, while sparing the phrenic nerve.

Ganglioneuroma dependiente del nervio frénico: localización inusual / Ganglioneuroma of the Phrenic Nerve: An Unusual Site

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Ultrafast ultrasound coupled with cervical magnetic stimulation for non-invasive and non-volitional assessment of diaphragm contractility.

KEY POINTS: Twitch transdiaphragmatic pressure elicited by cervical magnetic stimulation of the phrenic nerves is a fully non-volitional method for assessing diaphragm contractility in humans, yet it requires invasive procedures such as oesophageal and gastric catheter balloons.  Ultrafast ultrasound enables a very high frame rate allowing the capture of transient events, such as muscle contraction elicited by nerve stimulation (twitch). Whether indices derived from ultrafast ultrasound can be used as an alternative to the invasive measurement of twitch transdiaphragmatic pressure is unknown.  Our findings demonstrate that maximal diaphragm tissue velocity assessed using ultrafast ultrasound following cervical magnetic stimulation is reliable, sensitive to change in cervical magnetic stimulation intensity, and correlates to twitch transdiaphragmatic pressure.  This approach provides a novel fully non-invasive and non-volitional tool for the assessment of diaphragm contractility in humans. ABSTRACT: Measuring twitch transdiaphragmatic pressure (Pdi,tw ) elicited by cervical magnetic stimulation (CMS) is considered as a reference method for the standardized evaluation of diaphragm function. Yet, the measurement of Pdi requires invasive oesophageal and gastric catheter-balloons. Ultrafast ultrasound is a non-invasive imaging technique enabling frame rates high enough to capture transient events such as evoked muscle contractions. This study investigated relationships between indices derived from ultrafast ultrasound and Pdi,tw , and how these indices might be used to estimate Pdi,tw . CMS was performed in 13 healthy volunteers from 30% to 100% of maximal stimulator intensity in units of 10% in a randomized order. Pdi,tw was measured and the right hemidiaphragm was imaged using a custom ultrafast ultrasound sequence with 1 kHz framerate. Maximal diaphragm axial velocity (Vdi ,max ) and diaphragm thickening fraction (TFdi,tw ) were computed. Intra-session reliability was assessed. Repeated-measures correlation (R) and Spearman correlation coefficients (ρ) were used to assess relationships between variables. Intra-session reliability was strong for Pdi,tw and Vdi,max and moderate for TFdi,tw . Vdi,max correlated with Pdi,tw in all subjects (0.64 < ρ < 1.00, R = 0.75; all P < 0.05). TFdi,tw correlated with Pdi,tw in eight subjects only (0.85 < ρ < 0.93, R = 0.69; all P < 0.05). Coupling ultrafast ultrasound and CMS shows promise for the non-invasive and fully non-volitional assessment of diaphragm contractility. This approach opens up the prospect of both diagnosis and follow-up of diaphragm contractility in clinical populations.