PEDIATRICS Vol. 28 No. 4 October 1961, pp. 655-661
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GASTROINTESTINAL POLYPOSIS WITH MUCOCUTANEOUS PIGMENTATION IN CHILDREN (PEUTZ-JEGHERS SYNDROME)

James E. Wenzl M.D.1, Lloyd G. Bartholomew M.D.2, George A. Hallenbeck M.D.3, and Gunnar B. Stickler M.D.4

1 Mayo Foundation and Section of Pediatrics, Mayo Clinic, Rochester, Minnesota
2 Section of Medicine, Mayo Clinic, Rochester, Minnesota
3 Section of Surgery, Mayo Clinic, Rochester, Minnesota
4 Section of Pediatrics, Mayo Clinic, Rochester, Minnesota

A case is reported in which a child had gastrointestinal polyposis associated with mucocutaneous pigmentations (the Peutz-Jeghers syndrome). He experienced severe recurrent abdominal pain caused by intermittent intussusception. Removal of the grossly palpable polyps from the small intestine and stomach controlled his symptoms. On the basis of a review of the literature it has become apparent that this disease may first become manifest during childhood. The symptoms are primarily those of recurrent abdominal pain and gastrointestinal bleeding in the presence of mucocutaneous pigmentation. The prognosis associated with this disease is excellent, and the risk of malignant change, if it really exists, is minimal. For this reason, it is urged that surgical treatment be conservative rather than radical, to avoid unnecessary loss of intestine.


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