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Management of carnitine palmitoyltransferase (CPT) II deficiency during labor: a case series
Abstract Number: S-35
Abstract Type: Case Report/Case Series
Introduction: CPT II deficiency is characterized by an enzyme defect preventing long chain fatty acid use for energy. The myopathic form presents with weakness, myalgias, and fatigue during periods of increased metabolic demand with possible progression to rhabdomyolysis, renal failure, cardiac arrhythmias, and coma. We present a case series of two parturients with CPT II deficiency both of whom had transient myalgias after vaginal delivery and mild elevations of CPK levels irrespective of assisted or unassisted second stage.
Case Reports: A 20 y/o G2P0 diagnosed with CPT II deficiency at age 16 and a history of exercise-induced myalgias presented in spontaneous labor at 40 wks. A D10 infusion was started and an early epidural was placed to minimize labor stress. She pushed for 30 minutes and delivered via low forceps vaginal delivery. She complained of abdominal cramping post-delivery. Her baseline CPK was 48 U/L, peaked to 531 U/L post-delivery and decreased to 347 U/L at 24 hours post-partum. Her urine was negative for myoglobin.
A 28 y/o G1P0 was initially diagnosed with CPT II deficiency at age 2 but became aware of the diagnosis one year ago after an episode of hypothermia led to prolonged weakness and myalgias. She presented in spontaneous labor at 39.2 wks. A D10 infusion was started and an early epidural was placed. The patient labored down and after 35 minutes of pushing had an unassisted vaginal delivery. After delivery, she complained of right arm cramping. Her baseline CPK was 51 U/L, peaked to 268 U/L post-delivery and decreased to 162 U/L at 24 hours post-partum.
Discussion: CPT II deficiency can present unique challenges during labor due to the possibility of precipitating severe symptoms of this disease. The peripartum management of these patients focuses on maintaining glucose substrates and avoiding metabolic stress.1 These patients should receive an early labor epidural to minimize labor stress. To avoid lactic acidosis from hypoglycemia, D10 should be infused and glucose levels checked regularly. While previous literature supports checking CK levels at baseline and 24 hrs post-partum2, we also recommend checking CK levels soon after delivery. Both of our patients had CK levels peak within hours of delivery, indicating that this time period could confer the greatest risk for rhabdomyolysis. Although previous studies demonstrate lower overall CK values post-cesarean delivery3, this clinically insignificant difference in CK does not warrant elective surgery in these patients.
Although these patients have developed rhabdomyolysis and renal failure under general anesthesia, there is no evidence they are MH-susceptible. If propofol is used, the dose and duration should be minimized since mitochondrial disease predisposes patients to propofol infusion syndrome.4
1. Anaesth Intensive Care. 2009;37:305-308.
2. Can J Anesth. 2006;53:482-486.
3. Acta Anaesthesiol Scand. 1978;22:491-6.
4. Anesth Analg. 2009;109:1049-53.