Long-term home mechanical ventilation using a noninvasive ventilator via tracheotomy in patients with myasthenia gravis: a case report and literature review

Abstract

Neuromuscular diseases (NMD) are indications for long-term home mechanical ventilation (HMV). Noninvasive ventilation is preferred to HMV. However, invasive mechanical ventilation (IMV) is more appropriate if the patient has uncontrollable airway secretions, the possibility of aspiration, failure to wean, or severe weakness of the respiratory muscles. But if the patient undergoes multiple intubation or tracheotomy, it will be more painful and unbearable. For some end-stage NMD patients who need long-term tracheostomy, HMV using noninvasive ventilator via tracheotomy may be a conservative care option. An 87-year-old male with myasthenia gravis underwent repeated IMV and failed to wean. We used a noninvasive ventilator connected to a tracheostomy tube for mechanical ventilation. One and a half years later, the patient weaned successfully. However, there was a lack of evidence-based medicine and standardized guidelines in such areas as indications, contraindications, and ventilator parameter setting. For the systematic review, a literature search was performed in PubMed, Embase, Cochrane, and CNKI (China National Knowledge Infrastructure) to identify reported cases of using noninvasive ventilator in patients undergoing tracheostomy. A total of 72 cases who performed ventilation via tracheotomy tube were identified. The main diagnoses included NMD, chronic obstructive pulmonary disease (COPD), pneumonia, and congenital central hypoventilation syndrome (CCHS). Indications included dysfunctional ventilatory weaning response (DVWR), apnea and cyanosis. Clinical outcome was as follows: 33 patients were weaned, and 24 patients underwent HMV. A total of 288 cases who performed ventilation through the mask after blocking the tracheostomy tube were identified. The primary diagnoses included COPD, NMD, thoracic restriction, spinal cord injured (SCI), and CCHS. Indications included DVWR, apnea and cyanosis, routine weaning. Clinical outcome was as follows: successful tracheostomy tube decannulations were performed in 254 patients and failed in 33 patients. So, in patients requiring HMV, selection of noninvasive ventilation (NIV) or IMV should be individualized. Tracheostomy preservation should be considered in some patients with advanced NMD if there is respiratory muscle weakness or the risk of aspiration. And attempts can be made to use a noninvasive ventilator because of its advantages of portability, ease of operation, and low cost. Noninvasive ventilators can be used in patients with tracheotomy, whether direct connection tracheotomy or mask ventilation after the tube is capped, especially in weaning and tracheostomy tube decannulation.

Decision for early tracheostomy in respiratory failure of a paralyzed myasthenia gravis patient with sepsis-induced pneumonia: A case report

“…MG is more frequent in women under the age of 40 years and in men over the age of 60 years [ 7 ]. However, no correlation exists between age and indication for early tracheostomy placement [ [2] , [3] , [4] , 8 ].…”

“…Conditions that do not justify early tracheotomy are medical or surgical patients on a ventilator without lung infections (assessed by a Clinical Pulmonary Infection Score of <6) and without serious systemic problems (assessed by a Sequential Organ Failure Assessment score of ≥5) [ 9 ]. The clinician in charge subjectively judged which patients to tracheostomize earlier [ 1 , 2 , 4 ]. If the prediction of prolonged ventilation is inaccurate, an early tracheostomy strategy may result in some patients undergoing tracheostomy unnecessarily.…”

“…This is particularly beneficial for individuals experiencing MC. Patient demographics and baseline characteristics [ 2 , 4 ], comorbidities (such as diabetes mellitus, previous stroke, hypertension, chronic obstructive pulmonary disease, poor cardiac function, renal impairment, and ischemic heart disease [ [10] , [11] , [12] ]), time since MG onset, and complications such as pneumonia and cardiopulmonary resuscitation have been linked to a longer duration of mechanical ventilation [ 2 , 4 ]. An early tracheostomy may be required when medical therapy fails and patients continue to worsen despite intubation.…”

“…An early tracheostomy may be required when medical therapy fails and patients continue to worsen despite intubation. Given the combination of neuromuscular illness, respiratory insufficiency, dysphagia, and the significant likelihood of extubation failure, this might be explained by tracheostomy reducing anatomical dead space volume and improving breathing [ 1 , 4 ].…”

“…In MG, 15 to 20 % of patients develop a potentially life-threatening myasthenic crisis (MC), defined as respiratory failure requiring mechanical ventilation due to respiratory muscle weakness or bulbar dysfunction. The most common preceding factor is respiratory infection leading to sepsis [ 4 ].…”