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.
Keywords
spinal muscular atrophy - SMA - pregnancy - restrictive lung disease
