Anesthetic Considerations in Congenital Myasthenic Syndrome: A Case Report

TL;DR

This case report highlights anesthetic challenges in congenital myasthenic syndrome and suggests safe practices for managing these patients during surgery.

Contribution

The report raises concerns about sensitivity to neuromuscular blocking agents and suggests safety of dilute local anesthetics in this patient group.

Findings

Patients with congenital myasthenic syndrome may show heightened sensitivity to non-depolarizing neuromuscular blocking agents.

Caudal administration of dilute local anesthetics appears safe for neuraxial analgesia in these patients.

Abstract

Congenital myasthenic syndrome poses unique anesthetic challenges. Most notably, this rare disease is heterogeneous. Affected individuals’ responses to depolarizing and non-depolarizing neuromuscular blocking agents are poorly understood. Additionally, while neuraxial techniques may confer respiratory benefits due to their opioid-sparing profiles, it is unclear to what extent local anesthetics may impair signal transmission at the neuromuscular junction. This case report raises concern for heightened sensitivity to non-depolarizing neuromuscular blocking agents in patients with congenital myasthenic syndrome. Neuraxial analgesia with caudal administration of dilute local anesthetics appears to be safe.

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