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.
Meredith
J T
JT
Department of Pathology, Orlando Regional Medical Center, FL 32806.
Cerezo
L
L
Alvarez
M
M
Price
G
G
Bourgeois
S
S
D000293:Adolescent; D001157:Arterial Occlusive Diseases / Q000473:pathology*; D003937:Diagnosis, Differential; D004064:Digestive System / Q000098:blood supply*; D005260:Female; D005352:Fibromuscular Dysplasia / Q000473:pathology*; D006801:Humans; D012595:Scleroderma, Systemic / Q000473:pathology