Abstract
Purpose
To report the anesthetic management of a patient with carnitine palmitoyltransferase II deficiency who presented for labour and delivery.
Clinical features
A 30-yr-old primiparous woman with known carnitine palmitoyltransferase II deficiency and a past history of exercise induced muscle pain, weakness and myoglobinuria presented in active labour. Management consisted of an early epidural for labour and continuous dextrose infusion with frequent blood sugar monitoring. She had an uneventful spontaneous vaginal delivery. She experienced a single asymptomatic episode of hypoglycemia on the first postpartum day. Her serum creatine kinase was six times normal at 24 hr postdelivery and remained elevated for three days without evidence of rhabdomyolysis. The remainder of her postpartum course was uneventful.
Conclusion
Labour and delivery is a potential precipitant of rhabdomyolysis in patients with carnitine palmitoyltransferase II deficiency. The normal postpartum creatine kinase elevation (two to four times baseline at 24 hr) must be taken into account when monitoring these patients. On the basis of the physiologic principles, institution of early epidural analgesia to blunt the stress response to labour and delivery, continuous dextrose infusion and frequent glucose monitoring during labour and postpartum are the mainstays of management.
Résumé
Objectif
Présenter ľanesthésie ďune patiente atteinte ďun déficit en carnitine palmitoyltransférase II hospitalisée pour un accouchement.
Éléments cliniques
Une primipare de 30 ans, connue pour un déficit en carnitine palmitoyltransferase II et des douleurs musculaires induites par ľexercice, de la faiblesse et de la myoglobinurie, s’est présentée en travail actif. Une analgésie épidurale précoce pour le travail et une perfusion de dextrose continue sous vérification fréquente des glycémies ont été instituées. Ľaccouchement a été spontané, par voie vaginale. Elle n’a eu qu’un seul épisode ďhypoglycémie asymptomatique au premier jour du postpartum. La créatine-kinase sérique était six fois plus élevée que la normale à 24 h après ľaccouchement et est demeurée élevée pendant trois jours sans preuve de rhabdomyolyse. Le reste du postpartum a été sans incident.
Conclusion
Le travail et ľaccouchement peuvent précipiter une rhabdomyolyse en présence de déficit en carnitine palmitoyltransferase II. Ľélévation normale de la créatine-kinase au cours du postpartum (deux à trois fois les mesures de base à 24 h) doit être prise en compte pendant le monitorage de ces patientes. Partant des principes physiologiques, ľinstitution ďune analgésie épidurale précoce, pour réduire la réaction de stress du travail et de ľaccouchement, ďune perfusion de dextrose continue et du monitorage fréquent du glucose pendant le travail et le postpartum sont les principales mesures thérapeutiques.
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References
Deschauer M, Wieser T, Zierz S. Muscle carnitine palmitoyltransferase II deficiency: clinical and molecular genetic features and diagnostic aspects. Ach Neurol 2005; 62: 37–41.
Abramov Y, Abramov D, Abrahamov A, Durst R, Schenker J. Elevation of serum creatine phosphokinase and its MB isoenzyme during normal labor and early puerperium. Acta Obstet Gynecol Scand 1996; 75: 255–60.
Di Mauro S, Di Mauro PM. Muscle carnitine palmityltransferase deficiency and myoglobinuria. Science 1973; 182: 929–31.
Roe CR, Coates PM. Mitochondrial fatty acid oxidation disorders. In: Scriver CR, Beaudet AL, Sly WS, Valle D (Eds). The Metabolic and Molecular Basis of Inherited Disease, 7th ed. New York: McGraw-Hill; 2002: 1501–11.
Vladutiu GD, Bennett MJ, Fisher NM, et al. Phenotypic variability among first-degree relatives with carnitine palmitoyltransferase II deficiency. Muscle Nerve 2002; 26: 492–8.
Mardirosoff C, Dumont L, Cobin L, Massaut J. Labor analgesia in a patient with carnitine palmityl transferase deficiency and idiopathic thrombocytopenic purpura. Int J Obstet Anesth 1997; 7: 134–6.
Dreval, D, Bernstein D, Zakut, H. Carnitine palmitoyl transferase deficiency in pregnancy - a case report. Am J Obstet Gynecol 1994; 170: 1390–2.
Moundras JM, Wattrisse G, Leroy B, Decocq J, Krivosic- Horber R. Prise en charge anesthésique du travail obstétrical chez une parturiente atteinte ďun déficit musculaire en canitine palmitoyl transférase. Ann Fr Anesth Reanim 2000; 19: 611–6.
Sigauke, E, Rakheja D, Kitson K, Bennet MJ. Carnitine palmitoyltransferase II deficiency: a clinical, biochemical, and molecular review. Lab Invest 2003; 83: 1543–54.
Brownridge P. The nature and consequences of childbirth pain. Eur J Obstet Gynecol Reprod Biol 1995; 59(Suppl): S9–15.
Katsuya H, Misumi M, Ohtani Y, Miike T. Postanesthetic acute renal failure due to carnitine palmityl transferase deficiency. Anesthesiology 1988; 68: 945–8. 12Vladutiu GD, Taggart RT, Smail D, Lindsley HB, Hogan K. A carnitine palmitoyl transferase II (CPT2) Arg503Cys mutation confers malignant hyperther- mia and variable myopathy. Am J Hum Genet 1998; 63(Suppl): A5 (abstract). 13Vladutiu GD, Bennett MJ, Smail D, Wong LJ, Taggart RT, Lindsley HB. A variable myopathy associated with heterozygosity for the R503C mutation in the carnitine palmitoylatransferase II gene. Mol Genet Metab 2000; 70: 134-41.
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Lilker, S., Kasodekar, S. & Goldszmidt, E. Anesthetic management of a parturient with carnitine palmitoyltransferase ii deficiency. Can J Anesth 53, 482–486 (2006). https://doi.org/10.1007/BF03022621
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DOI: https://doi.org/10.1007/BF03022621