Pregnancy in a Patient with Spinal Muscular Atrophy and Severe Restrictive Lung Disease.

ABSTRACT

Spinal muscular atrophy (SMA) is a rare autosomal recessive neuromuscular disease that is often associated with chronic respiratory failure. Few cases have described the antepartum and postpartum course in patients with severely compromised respiratory status. We present a case of a 24-year-old nullipara with a history of SMA type II complicated by surgically corrected kyphoscoliosis and severe restrictive lung disease. Her pregnancy was complicated by progressively worsening dyspnea resulting in increased use of noninvasive positive pressure ventilation, ultimately leading to indicated premature delivery at 28 weeks' gestation via cesarean section under general anesthesia. Women with SMA and severe restrictive lung disease are at high risk of premature delivery secondary to worsening respiratory status. A multidisciplinary approach is vital in treating these patients.