VOLUME 32 - NUMBER 6-7 - 2011

Laparoscopic cholecystectomy in a patient with Steinert myotonic dystrophy. Case report

  • Agrusa A., Mularo S., Alessi R., Di Paola P., Mularo A., Amato G., Romano G.
  • Clinical practice, 320-321
  • Full text PDF

  • Myotonic dystrophy (MD) is a serious multi-systemic autosomal dominant disease. The estimated incidence is 1 in every 8000 births, with an estimated prevalence of between 2.1 and 14.3 cases per 100,000 inhabitants. Signs and symptoms vary from a severe form of congenital myopathy, present from birth and often fatal, to a classic form and a delayed form, which generally presents after the age of 50 and in which the only sign is a cataract and life expectancy is completely normal. We describe the clinical case of a 40-year-old woman with Steinert myotonic dystrophy who underwent laparoscopic cholecystectomy (under general anesthesia) for symptomatic gallbladder stones. The conduct of anesthesia in such patients must be carefully considered, as hypothermia, shivering, electrical and mechanical stimulation, and the drugs used can all trigger myotonia.

  • KEY WORDS: Laparoscopy - Cholecystectomy - Myotonic dystrophy - Steinert disease.