Pneumatosis intestinale in children with primary combined immunodeficiency.

We report six episodes of pneumatosis intestinale (PI) in children with primary combined immunodeficiency. PI was generally benign and responded to conservative therapy. Increased risk of infection or disruption of gastrointestinal mucosa are probable predisposing factors. A diagnosis of PI should be considered in children with primary combined immunodeficiency who experience otherwise unexplained gastrointestinal symptoms, particularly in the early post-transplant period.