Abstract
Friedreich’s ataxia is an inherited neuromuscular disorder often associated with significant cardiac disease. We report a case of Friedreich’s ataxia in a 13-year-old girl with ulcerative colitis and hypertrophic cardiomyopathy who was successfully managed for subtotal colectomy with general anaesthesia and epidural narcotic. Anaesthetic considerations included the maintenance of fluid volume and stable cardiovascular variables in the intra- and postoperative periods.
Résumé
Ľataxie de Friedreich est une maladie neuromusculaire familiale souvent associée à des troubles cardiaques significatifs. On rapporte le cas ďune jeune fille de 13 ans atteinte ďataxie de Friedreich, de colite ulcéreuse et ďune cardiomyopathie hypertrophique ayant subi avec succès une colectomie sub-totale sous anesthésie générale et analgésie épidurale aux narcotiques. Les considérations anesthésiques comprennent le maintien ďune volémie et ďun état hémodynamique stable dans la période per et postopératoire.
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References
Pratt RTC. The Genetics of Neurological Disorders. London: Oxford University Press. 1967. 34–5.
Bell CF, Kelly JM, Jones RS. Anaesthesia for Friedreich’s ataxia. Case report and review of the literature. Anaesthesia 1986; 41: 296–301.
Child JS, Perloff JK, Bach PM, Wolfe AD, Perlman S, Kark P. Cardiac involvement in Friedreich’s ataxia. A study of 75 patients. J Am Coll Cardiol 1986; 7: 1370–8.
Alboliras ET, Shub C, Gomez MR et al. Spectrum of cardiac involvement in Friedreich’s ataxia: clinical, electrocardiographic and echocardiographic observations. Am J Cardiol 1986; 58: 518–24.
Friedreich N. Über degenerative Atrophie der spinalen Hinterstränge. Arch Anat Physiol 1863; 26: 391–419; 433-59.
Hartman JM, Booth RW. Friedreich’s ataxia: a neurocardiac disease. Am Heart J 1960; 60: 716–20.
O’Brien ET, Dajee H, Ward OC. Friedreich’s disease — a family study. Europ J Cardiol 1977; 6: 15–24.
Schilero AJ, Antzis E, Dunn J. Friedreich’s ataxia and its cardiac manifestations. Am Heart J 1952; 44: 805–22.
Thorén C. Cardiomyopathy in Friedreich’s ataxia. Europ J Cardiol 1977; 5: 282.
Berg RA, Kaplan AM, Jarrett PB, Molthan ME. Friedreich’s ataxia with acute cardiomyopathy. Am J Dis Child 1980; 134: 390–3.
Coté M, Davignon A, Pecko-Drouin K et al. Cardiological signs and symptoms in Friedreich’s ataxia. Can J Neurol Sci 1976; 3: 319–21.
Gottdiener JS, Hawley RJ, Maron BJ, Bertorini TF, Engle WK. Characteristics of the cardiac hypertrophy in Friedreich’s ataxia. Am Heart J 1982; 103: 525–31.
Bromage PR. Epidural Analgesia. Philadelphia: WB Saunders 1978: 707.
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Campbell, A.M., Finley, G.A. Anaesthesia for a patient with Friedreich’s ataxia and cardiomyopathy. Can J Anaesth 36, 89–93 (1989). https://doi.org/10.1007/BF03010895
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DOI: https://doi.org/10.1007/BF03010895