Clinical History.—This 12-year-old girl was referred to Childrens Hospital of Los Angeles for evaluation of abdominal pain and anemia. At 1½ years of age, she passed a polyp per rectum. Sigmoidoscopy at that time showed numerous rectal polyps. She was well in the interim until one year ago when she began to experience intermittent episodes of generalized crampy abdominal pain and vomiting. The symptoms became more frequent and severe in the past three weeks. She obtained some relief by leaning far forward in a knee-chest position. Her bowel movements were normal. Her parents and two older brothers are alive and well.
Physical Examination.—The patient was an extremely pale girl with numerous pigmented spots on the skin around her mouth and on the mucous membrane of her lips and mouth. She was alert, oriented, and in no acute distress. Her vital signs were normal. A grade II/VI systolic ejection