Pyloromyotomy in a patient with paramyotonia congenita.
A 2-mo-old infant with paramyotonia congenita was scheduled for pyloromyotomy and repair of inguinal hernia. Diagnosis of paramyotonia congenita was done with positive family history, myotonia at eyelids, provocation by cold, and electromyogram analysis. Anesthesia was induced via face mask with sevoflurane at 4 minimum alveolar anesthetic concentration in oxygen. Tracheal intubation was attempted without a neuromuscular relaxant. Anesthesia was maintained with sevoflurane at 0.5 minimum alveolar anesthetic concentration in oxygen and remifentanil infusion at a rate of 0.2 micro g. kg(-1). min(-1). After discontinuation of sevoflurane and remifentanil, the patient was awake and had full recovery of muscle activity.


CONCLUSIONS
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.
Ay
Binnaz
B
Departments of *Anesthesiology and Reanimation and †Pediatric Surgery, Marmara University Medical School, Istanbul, Turkey.
Gerçek
Arzu
A
Doğan
Varlk I
VI
Kyan
Gürsu
G
Göğüş
Ylmaz F
YF
D000769:Anesthesia, Inhalation; D018685:Anesthetics, Inhalation; D004576:Electromyography; D006552:Hernia, Inguinal / Q000150:complications / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D008738:Methyl Ethers; D020967:Myotonic Disorders / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D010375:Pedigree; D011708:Pylorus / Q000601:surgery*; D000077149:Sevoflurane
D018685:Anesthetics, Inhalation; D008738:Methyl Ethers; D000077149:Sevoflurane