Gastrointestinal arterial fibromuscular dysplasia of childhood

An unusual case of a progressive, noninflammatory stenosing vasculopathy, arterial fibromuscular dysplasia is presented. The distinctive features of this particular case include onset in early childhood with a predominant involvement of the gastrointestinal system, sparing of the renal arteries, lack of hypertension, and no cutaneous features of progressive systemic sclerosis. We discuss the clinical history over a decade and the pathologic features, including routine and electron microscopic findings of biopsy and autopsy tissues, and review the literature.