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Clinical Management Based on Long-term Observations

Jay L. Grosfeld, MD; Karen W. West, MD

Arch Surg. 1986;121(5):530-534.

Abstract
• Generalized juvenile polyposis occurred in five patients (age range, 18 months to 16 years). Clinical findings included abdominal pain, weakness, rectal bleeding, diarrhea, rectal prolapse, intussusception, clubbing, and failure to thrive. Laboratory findings included anemia, hypoalbuminemia, hypokalemia, and skin test anergy. Diagnosis is achieved by double contrast enema, endoscopy, and biopsy. Unlike patients with solitary juvenile polyps, patients with generalized involvement require surgical intervention. Subtotal colectomy and ileoproctostomy are the procedures of choice, and we performed them in four cases. An ileoanal-endorectal pull-through procedure was required in one patient with continued rectal disease. All five patients are currently alive and well. Long-term follow-up is important as polyps may persist into adult life. Family members are at risk for developing gastrointestinal tract tumors and should be screened.

(Arch Surg 1986;121:530-534)

Author Affiliations
From the Section of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine and the James Whitcomb Riley Hospital for Children, Indianapolis.

Footnotes
Accepted for publication Feb 13, 1986.

Read before the 93rd Annual Meeting of the Western Surgical Association, Rochester, Minn, Nov 19, 1985.

Reprint requests to Department of Surgery, James Whitcomb Riley Hospital for Children, 702 Barnhill Dr (K21), Indianapolis, IN 46223 (Dr Grosfeld).

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