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DP08: LABOR ANALGESIA IN A PARTURIENT WITH SUSPECTED CPT II DEFICIENCY
Karina Oganezova, MD; Justin Scuorzo, DO; Emily King, MD
Memorial Healthcare System
Background: Carnitine palmitoyltransferase II (CPT II) is an enzyme within the carnitine shuttle system that enables long-chain fatty acids to enter mitochondria for energy production. CPT I converts long-chain fatty acids into acylcarnitines, which are transported into the mitochondrial matrix, where CPT II converts them into acyl-CoA for β-oxidation. The resulting acetyl-CoA enters the citric acid cycle or is used for ketone production. CPT II deficiency is the most common inherited disorder of long-chain fatty acid oxidation affecting skeletal muscle, manifesting with exercise-induced myalgia and recurrent rhabdomyolysis. Because this deficiency, as well as other mitochondrial disorders, results in the decrease of ketones and ATP, parturients with these conditions are at increased risk of hypoglycemia, rhabdomyolysis, and kidney failure.
Case Report: A 31-year-old G1P0 woman with a BMI of 46 presented at 34w1d for induction of labor due to preeclampsia with severe features. During the initial anesthesia evaluation, a significant history of recurrent rhabdomyolysis was identified. In 2018, the patient was hospitalized for rhabdomyolysis complicated by posterior reversible encephalopathy syndrome and seizure activity. After a brief course of antiepileptic therapy, she has remained seizure-free.
Following that hospitalization, the patient underwent a skeletal muscle biopsy demonstrating increased cytoplasmic lipid droplets within otherwise morphologically normal muscle fibers. Although these findings were nonspecific, in the context of her clinical history, CPT II deficiency was proposed as a possible diagnosis, though confirmatory genetic testing was never completed. The patient had no prior exposure to general anesthesia and no family history of anesthetic complications. No formal preanesthetic consultation occurred before admission.
Given concern for an underlying fatty acid oxidation disorder, extensive peripartum planning was undertaken to minimize metabolic stress and avoid triggering agents (Figure 1). The obstetric team aimed to reduce labor-associated stressors, including prolonged pushing. Dextrose-containing intravenous fluids were administered while the patient was nil per os, with frequent glucose monitoring to prevent hypoglycemia-induced rhabdomyolysis. Creatine kinase levels were monitored every six hours for early detection of muscle injury.
Early labor analgesia was provided via epidural infusion with fentanyl and ropivacaine, with frequent reassessment to reduce the likelihood of conversion to general anesthesia. Bupivacaine was avoided due to concern for potential cardiac toxicity in suspected CPT II deficiency. A dedicated operating room was prepared for emergent cesarean delivery. Ultimately, the patient delivered an infant via vacuum-assisted vaginal delivery with APGAR scores of 1, 3, and 8 requiring NICU care. The patient is now undergoing evaluation to confirm the suspected diagnosis.
Discussion: As the anesthetic implications of CPT II deficiency are significant, and the spectrum of potential triggers is complex, this case underscores the importance of advanced planning and careful multidisciplinary coordination to ensure parturient safety. Avoidance of hypoglycemia was critical to prevent metabolic stress–induced attacks. Volatile anesthetics were removed from the operating room, and agents such as propofol and succinylcholine were avoided due to their association with rhabdomyolysis. With careful coordination between anesthesiology and obstetrics, the patient had an uncomplicated labor, delivery, and postpartum course, highlighting the need for further guidelines in this population.
