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Familial Congenital Muscular Dystrophy and Pregnancy
Abstract Number: T 53
Abstract Type: Case Report/Case Series
INTRODUCTION: Familial Congenital Muscular Dystrophy is a heterogenous group of disorders involving progressive muscle weakness secondary to atrophy without evidence of denervation. Clinical and genetic variability occurs among patients with these disorders. Progressive muscle weakness and joint contractures lead to impaired mobility and respiratory compromise. The disease also affects cardiac muscle, resulting in a dilated cardiomyopathy and conduction abnormalities. We present a case of a parturient with familial congenital muscular dystrophy who required a cesarean delivery due to progressive respiratory compromise and discuss the implications for the anesthesiologist caring for such patients.
CASE REPORT: A 25yo 57kg G3P2 with muscular dystrophy, severe scoliosis, Harrington rods, restrictive pulmonary disease, pulmonary hypertension, and cardiomyopathy presented at 18 wks gestation. She was noted to have features of a difficult intubation (small mouth opening, limited neck mobility, short thyromental distance, and Mallampati Score of 3). PFTs confirmed restrictive lung disease (FEV1 11% FVC 19% Ratio 97%) and an echocardiogram showed diastolic dysfunction with preserved LV function, enlarged RV and a RVSP of 45 mmHg. At 24 wks, she developed acute hypercarbic respiratory failure with mental status changes and sustained CO2 elevations despite BiPap. She was fiberoptically intubated in the ICU and subsequently underwent tracheostomy. One week later, she developed elevated peak airway pressures (low 50s) and inadequate oxygenation and ventilation despite maximal ventilatory support. ABG results were 7.35/57/64/92% on 100% FiO2. With the fetal heart tracing showing decreased variability and maternal clinical status deteriorating, the decision was made to proceed with cesarean delivery at 25 2/7 wks. She was transported to the OR on the ICU ventilator after receiving midazolam. A TIVA technique with propofol and remifentanil was begun upon arrival in the OR and rocronium was administered. Upon delivery of the baby, PIPs decreased to the low 40s and oxygen saturation improved to 100%. Weeks later the patient was transferred to a long term care facility as she was unable to be weaned from the ventilator.
CONCLUSION: Women with progressive myopathies, including familial muscular dystrophy, often experience an exacerbation of symptoms during pregnancy. The increased minute ventilation and decreased FRC of pregnancy may lead to clinical deterioration of a patient’s respiratory status. Aspiration may be a concern due to dysphagia and mental status changes. Contractures lead to difficulty with line placement and positioning. Muscular dystrophy patients may be susceptible to a hypermetabolic syndrome similar to malignant hyperthermia, so triggering agents (succinylcholine and volatile agents) should be avoided. A TIVA technique is commonly used. If neuromuscular blocking drugs are used, documentation of adequate reversal is essential.