Multidisciplinary Perioperative Care for Children with Neuromuscular Disorders

Kynes, J. Matthew; Blakely, Martin L.; Furman, Kevin C.; Burnette, W. Bryan; Modes, Katharina

doi:10.3390/children5090126

Cited by 11 publications

(3 citation statements)

References 33 publications

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“…Cardiac involvement is of particular concern in all muscular dystrophies and is thought to be present in up to 20% of cases. 34 The absence of skeletal symptoms does not preclude cardiac abnormalities. Other NMDs such as Friedreich’s ataxia can be associated with left ventricular hypertrophy.…”

Section: Resultsmentioning

confidence: 99%

Perioperative Optimization of Patients With Neuromuscular Disorders Undergoing Scoliosis Corrective Surgery: A Multidisciplinary Team Approach

Sedra

Shafafy

Sadek

et al. 2020

Global Spine Journal

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Study Design: Narrative review. Objective: The high rate of complications associated with the surgical management of neuromuscular spinal deformities is well documented in the literature. This is attributed to attenuated protective physiological responses in multiple organ systems. Methods: Review and synthesis of the literature pertaining to optimization of patients with neuromuscular scoliosis undergoing surgery. Our institutional practice in the perioperative assessment and management of neuromuscular scoliosis is also described along with a clinical vignette. Results: Respiratory complications are the most common to occur following surgery for neuromuscular disorders. Other categories include gastrointestinal, cardiac, genitourinary, blood loss, and wound complications. A multidisciplinary approach is required for perioperative optimization of these patients and numerous strategies are described, including respiratory management. Conclusion: Perioperative optimization for patients with neuromuscular disorders undergoing corrective surgery for spinal deformity is multifaceted and complex. It requires a multidisciplinary evidence-based approach. Preadmission of patients in advance of surgery for assessment and optimization may be required in certain instances to identify key concerns and formulate a tailored treatment plan.

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Section: Resultsmentioning

confidence: 99%

Perioperative Optimization of Patients With Neuromuscular Disorders Undergoing Scoliosis Corrective Surgery: A Multidisciplinary Team Approach

Sedra

Shafafy

Sadek

et al. 2020

Global Spine Journal

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“…Succinylcholine, a depolarizing muscle relaxant, should be avoided in patients with muscular dystrophies, motor neuron diseases, and intrinsic muscle disease because of the risk of malignant hyperthermia, fatal hyperkalemia, and rhabdomyolysis ( 35 – 37 ). Nondepolarizing muscle relaxants should be reduced dosage and titrated carefully in some categories of NMD, including myotonic muscular dystrophy, MG, congenital myasthenic syndrome, SMA, polymyositis, and dysimmune neuropathies ( 36 , 37 ). In addition, due to the advantages of easy controlled dosage and shorter onset time, intravenous anesthetics are preferable to volatile agents in most patients of NMD ( 37 ).…”

Section: Management Of Nmd Patients With Covid-19 Infectionmentioning

confidence: 99%

Care for Patients With Neuromuscular Disorders in the COVID-19 Pandemic Era

Tseng

Chen

2021

Front. Neurol.

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The coronavirus disease 2019 (COVID-19) pandemic has prompted a rapid and unprecedented reorganization of medical institutions, affecting clinical care for patients with chronic neurological diseases. Although there is no evidence that patients with neuromuscular disorders (NMD) confer a higher infection risk of COVID-19, NMD and its associated therapies may affect the patient's ability to cope with infection or its systemic effects. Moreover, there is a concern that patients with chronic NMD may be at increased risk of manifesting severe symptoms of COVID-19. In particular, as respiratory compromises account for the major cause of mortality and morbidity in NMD patients, newly emerging data also show that the risk of exacerbation caused by COVID-19 accumulates in this particular patient group. For example, patients with motor neuron disease and dystrophinopathies often have ventilatory muscle weakness or cardiomyopathy, which may increase the risk of severe COVID-19 infection. Thus, the COVID-19 pandemic may severely affect NMD patients. Several neurological associations and neuromuscular networks have recently guided the impact of COVID-19 on patients with NMD, especially in managing cardiopulmonary involvements. It is recommended that patients with moderate- to high-risk NMD be sophisticatedly monitored to reduce the risk of rapid decline in cardiopulmonary function or potential deterioration of the underlying NMD. However, limited neuromuscular-specific recommendations for NMD patients who contract COVID-19 and outcome data are lacking. There is an urgent need to properly modify the respiratory care method for NMD patients, especially during the COVID-19 pandemic. Conclusively, COVID-19 is a rapidly evolving field, and the practical guidelines for the management of NMD patients are frequently revised. There must be a close collaboration in a multidisciplinary care team that should support their hospital to define a standardized care method for NMD patients during the COVID pandemic. This article reviews evidence-based practical guidelines regarding care delivery, modification, and education, highlighting the need for team-based and interspecialty collaboration.

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“…Alternatives such as ketamine, propofol, opioids, barbiturates and benzodiazepines each have their specific disadvantages and risks in patients with Becker’s and Duchenne’s dystrophies. 35–37…”

Section: Anaesthesia-induced Rhabdomyolysismentioning

confidence: 99%

Anaesthesia and neuromuscular disorders: what a neurologist needs to know

et al. 2020

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Neurologists are often asked for specific advice regarding patients with neuromuscular disease who require general anaesthesia. However, guidelines on specific neuromuscular disorders do not usually include specific guidelines or pragmatic advice regarding (regional and/or general) anaesthesia or procedural sedation. Furthermore, the medical literature on this subject is mostly limited to publications in anaesthesiology journals. We therefore summarise general recommendations and specific advice for anaesthesia in different neuromuscular disorders to provide a comprehensive and accessible overview of the knowledge on this topic essential for clinical neurologists. A preoperative multidisciplinary approach involving anaesthesiologists, cardiologists, chest physicians, surgeons and neurologists is crucial. Depolarising muscle relaxants (succinylcholine) should be avoided at all times. The dose of non-depolarising muscle relaxants must be reduced and their effect monitored. Patients with specific mutations in RYR1 (ryanodine receptor 1) and less frequently in CACNA1S (calcium channel, voltage-dependent, L type, alpha 1S subunit) and STAC3 (SH3 and cysteine rich domain 3) are at risk of developing a life-threatening malignant hyperthermia reaction.

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Multidisciplinary Perioperative Care for Children with Neuromuscular Disorders

Cited by 11 publications

References 33 publications

Perioperative Optimization of Patients With Neuromuscular Disorders Undergoing Scoliosis Corrective Surgery: A Multidisciplinary Team Approach

Perioperative Optimization of Patients With Neuromuscular Disorders Undergoing Scoliosis Corrective Surgery: A Multidisciplinary Team Approach

Care for Patients With Neuromuscular Disorders in the COVID-19 Pandemic Era

Anaesthesia and neuromuscular disorders: what a neurologist needs to know

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