Pediatric neuromuscular disorders

Published on 07/02/2015 by admin

Filed under Anesthesiology

Last modified 07/02/2015

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Pediatric neuromuscular disorders

Andrea P. Dutoit, MD and Randall P. Flick, MD, MPH

Neuromuscular disorders (NMDs) affect a diverse group of pediatric patients with muscular and neurologic disease of varying complexity and origin. Perioperative concerns and management differ greatly depending on the specific cause of the disorder and the patient’s signs and symptoms, making it very important to be familiar with the precise underlying pathophysiology of the NMDs. This information is not always known, making anesthetic management of patients with NMDs challenging.

NMDs are often associated with other congenital disorders; pediatric patients with NMDs often present for elective surgical procedures to correct various deformities or for diagnostic purposes. The major and most common anesthetic concerns when caring for pediatric patients with NMDs are listed in Box 208-1. Several types of NMDs are briefly considered here, along with their major perioperative concerns.

Box 208-1 Anesthetic Considerations for Children with Neuromuscular Disorders

• Aspiration risk is increased due to decreased airway reflexes and increased oral secretions.

• The use of succinylcholine should be avoided, except in emergency situations, due to the increased risk of hyperkalemia developing.

• The risk of MH or rhabdomyolysis with MH-triggering anesthetic agents is unclear.

• Patients may have an increased resistance to the effects of nondepolarizing neuromuscular blocking agents.

• The MAC may be decreased.

• Patients with NMDs may have an increased sensitivity to the effects of opioids.

• Patients with NMDs may have an increased risk of experiencing perioperative blood loss, factor deficiency, or thrombocytopenia.

• Patients with NMDs may be more prone to developing hypothermia.

MAC, Minimum alveolar concentration; MH, malignant hyperthermia; NMD, neuromuscular disorders.

Cerebral palsy

Cerebral palsy (CP) and static encephalopathy are terms used for a collective group of nonprogressive disorders of movement involving abnormal development or prenatal injury to the brain. Although genetic abnormalities, perinatal anoxia, infection, and trauma have been proposed as etiologic factors in CP, no single cause has been identified. CP has a prevalence of 2 to 4 in 1000 live births, and patients with CP will have a variety of presentations, from near normal functional status to complete incapacitation. Clinical manifestations include disorders of posture due to spasticity or hypotonia of lower extremity or upper extremity muscle groups and abnormal speech or vision. Gastroesophageal reflux, behavior problems, mental retardation, and epilepsy can occur in some children with CP.

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