Vascular Perfusion Abnormalities in Infants with Spinal Muscular Atrophy

Spinal muscular atrophy (SMA) is an important cause of death in children and SMA type 1, also known as Werdnig-Hoffman disease, is the most severe form of this disease. We report 2 cases of infants with SMA I in whom a distal necrosis developed, a feature not previously reported. Poor perfusion, autonomic dysfunction, and position-dependent factors may all play a role in the development of this complication. (J Pediatr 2009,155:292-4)