Chronic Disease of Childhood

Abstract

Cerebral Palsy describes a spectrum of neurological motor disorders that can be associated with other conditions such as seizures and intellectual impairment. Most children have increased muscle tone or spasticity in one or more muscle groups or limbs. There is a broad range in the severity of problems in these children, and children with more severe forms present a number of challenges for anesthesia. These include communication, positioning, hypothermia, respiratory function and pain management. Myopathies are uncommon conditions that have important implications for anesthesia. Most young children with muscle diseases undergo anesthesia without problem. There are, however, three specific risks—the risk of rhabdomyolysis from suxamethonium in any child with a myopathy; the risk of rhabdomyolysis from volatile agents in a child with muscular dystrophy, and finally the risk of malignant hyperthermia in some children with specific, rare muscle disorders. Children with mucopolysaccharidosis can be among the most difficult to anesthetize because they can have a very difficult airway, poor cardiac function, and developmental issues affecting behavior. The incidence of Type I diabetes in children is increasing. Many of these children now have insulin pumps and continuous glucose monitoring. Autism spectrum disorder is a group of disorders characterized by deficits in social interaction and communication with repetitive or unusual behaviors. Anxiety and behavior are the main issues for anesthesia, for which there are many practical and pharmacological strategies to help with their management.

Review Questions

• 1. Why is suxamethonium contraindicated in patients with muscular dystrophy?

• 2. How would you initiate treatment for suspected MH? What is the dose of Dantrolene?

• 3. Why do patients with Hurler’s syndrome have difficult airways?

• 4. Why might a child with Trisomy 21 have a difficult airway?