Pyloromyotomy in a Patient with Paramyotonia Congenita

Abstract

The literature concerning general anesthesia in paramyotonic patients is limited. We report a case of paramyotonia congenita in a 2-mo-old male infant undergoing surgery for pyloric stenosis and inguinal hernia after an uneventful anesthesia.

Repeat episodes of severe muscle rigidity in a child receiving sevoflurane

“…Mutated sodium channels which result in an increased tendency to generate repetitive action potentials and muscle fibers depolarization, have been described (12). An abnormal gene on chromosome 17q, which encodes the α ‐subunit of human skeletal muscle sodium channel, may be responsible for these diseases (13). The likelihood of these disorders in this patient is quite small since family history is negative, exposure to cold temperatures did not occur, and rigidity was not reported with succinylcholine.…”

Pathophysiologic and anesthetic considerations for patients with myotonia congenita or periodic paralyses

“…Later in life, these individuals may also elicit transient disabling paralysis following such episodes of myotonia . It has been reported that volatile anesthesia using sevoflurane has been used repeatedly in PC patients without any incident and the risk of susceptibility to MH was considered to be that of the general population . In both reported cases of anesthetic induction for the PC patient, the protocols did not use muscle relaxants.…”

“…In both reported cases of anesthetic induction for the PC patient, the protocols did not use muscle relaxants. It should be noted that especially the use of succinylcholine is contraindicated in such cases, as potential increases in serum potassium concentrations could trigger myotonic symptoms . Furthermore, one should refrain from administering anticholinesterase agents or supplementing potassium, as these may provoke severe myotonia.…”

Anesthetic management for subtotal gastrectomy in a patient with paramyotonia congenita

“…Mutant channels exhibit sustained sodium currents that lead to prolonged membrane depolarization, causing myotonia, followed by membrane desensitization (or inactivation), resulting in paralysis. Clinically, this disorder is characterized by transient stiffness (myotonia) precipitated by exposure to such factors as cold temperatures or physical exertion.Several case reports of anesthesia employed for patients with PC have been published [1][2][3], and below we describe our experiences with anesthesia employed for a PC patient who underwent a subtotal gastrectomy for treatment of a gastric carcinoma. …”

“…Several case reports of anesthesia employed for patients with PC have been published [1][2][3], and below we describe our experiences with anesthesia employed for a PC patient who underwent a subtotal gastrectomy for treatment of a gastric carcinoma.…”