Peripartum Management of Congenital Fiber Type Disproportion Myopathy With Severe Restrictive Lung Disease

Congenital myopathies raise unique challenges for anesthesiologists during labor and delivery. Apart from having a risk for malignant hyperthermia, this patient population can present with severe restrictive lung disease in the third trimester. Scoliosis and weak pelvic muscles could make regional anesthesia difficult. The common complications in pregnancy include premature labor, preterm delivery, spontaneous abortion, a prolonged first stage of labor, and uterine atony. We report a case of 28-year-old primigravida of 37 weeks gestation diagnosed with congenital fiber type disproportion successfully managed by a team of high-risk obstetricians, anesthesiologists, and pulmonologists. The patient was closely monitored with serial arterial blood gas to determine carbon dioxide retention in a high-risk labor floor with a backup operating room for cesarean delivery. We reserved a malignant hyperthermia cart and a postpartum hemorrhage cart for emergencies. Our patient was able to deliver vaginally with the help of ultrasound-guided regional anesthesia despite having severe restrictive lung disease and scoliosis. We emphasize a multi-disciplinary team approach for a successful outcome for this patient population.