///2019 Abstract Details
2019 Abstract Details2019-07-13T07:45:15-05:00

Successful Perinatal Management of a Woman with McArdle’s Disease: A Case Report

Abstract Number: FCA-379
Abstract Type: Case Report Case Series

Chad T Dean MD1 ; Lauri Adler MD2; Susan McElroy DO3; Grace Lim MD MS4

McArdle’s disease (type V glycogen storage disease) is a rare (incidence 1:100,000), autosomal recessive, metabolic disease resulting in myophosphorylase enzyme deficiency. This enzyme is present in skeletal muscle and is involved in glycogen breakdown into glucose for use in muscle. Exacerbations present as muscle pain/cramping, early fatigue, and myoglobinuria. With muscle inability to use glucose for energy, alternative energy sources (proteins and fatty acids) are used. Protein catabolism leads to rhabdomyolysis which can progress to renal failure. There are few reported cases of anesthetic management in pregnant patients with McArdle’s disease. We report perinatal management of a woman with this rare disease.

A 24-year-old G1P0 with McArdle’s disease and anxiety presented at 37 weeks for elective cesarean delivery. Pre-operative optimization included clear fluids until two hours before surgery followed by initiation of D10 ½ NS intravenous infusion at 1.5 times maintenance rate, to prevent hypoglycemia and protein catabolism. She received midazolam for anxiolysis and uneventful spinal anesthesia with bupivacaine 12mg and duramorph 150mcg. Intraoperative course was uncomplicated. The dextrose infusion was continued throughout the case, with lactated ringers for fluid boluses to maintain intravascular volume. Adequate urine output and appearance was closely monitored. Normothermia was maintained with an upper body forced air warmer. To prevent physiologic stresses from nausea and vomiting, she was prophylactically treated with phenylephrine infusion, ondansetron and dexamethasone prior to delivery. Postoperatively, the dextrose solution continued for 24 hours until a full diet was tolerated.

Inborn errors of metabolism can pose significant problems during the peri-operative period if physiologic stressors, temperature, euglycemia, and protein catabolism are not addressed. Perioperative risks include fatigue, respiratory compromise, hypoglycemia, rhabdomyolysis, myoglobinuria, renal failure, and potentially malignant hyperthermia (MH). Although no known link between MH and McArdle’s exists, there have been two case reports suggesting a possible connection. Patients often have chronically elevated creatine phosphokinase (CPK), which is released from muscle catabolism. Careful monitoring of CPK levels can provide early evidence of stressors and inadequate homeostasis maintenance. Spontaneous compartment syndrome can potentially arise as well [1]. For obstetric patients, special concerns include anxiolysis, pain management, and candidacy for the physiologic stresses of labor and delivery; successful vaginal delivery has been described [2]. In conclusion, with careful attention to maintaining adequate blood glucose levels and fluid management, normothermia maintenance, blood pressure monitoring, and maintaining MH precautions, safe anesthesia can be provided to these patients.

1. Bollig G. 2013;23:817

2. Giles W. 2011;4:120

SOAP 2019