Colonic surgery in patients with juvenile polyposis syndrome: a case series.

PURPOSE: Juvenile polyposis syndrome is characterized by multiple hamartomatous polyps in the large intestine. When indicated, the surgical choices in symptomatic juvenile polyposis syndrome patients are colectomy with ileorectal anastomosis or proctocolectomy with pouch. The aim of this study was to evaluate the long-term outcomes of the surgical options in juvenile polyposis syndrome patients who present with symptomatic colonic polyps.
METHODS: The charts of all juvenile polyposis syndrome patients who had had at least one colonic operation since 1953 in our institution were reviewed. The following data were abstracted: demographics, the number and site of the polyps, symptoms, the intervals and types of the colonic operation, follow-up, and the patients' current status.
RESULTS: There were 13 patients (6 males) with a median age of 10 years (range, 1-50 years) at the time of diagnosis. Patients had colonic (n = 13), rectal (n = 12), and gastric (n = 6) polyps. Rectal bleeding (n = 11) was the most common presenting symptom. Three patients underwent proctectomy as the initial operation. Although a rectum-preserving operation was initially performed in ten patients, a subsequent proctectomy was required in five of them within a median of 9 years (range, 6-34 years). Therefore, eight patients had their rectum removed during the study period; five had an ileal pouch-anal anastomosis, one had a Koch pouch as a restorative surgery, and two had an end ileostomy. No relation was observed between the number of colonic and rectal polyps and the type of surgery or the need for proctectomy. Patients were followed up a median of 3 years (range, 2-24 years) after their ultimate operations. During this period, one patient (20 percent) who underwent restorative proctectomy and 4 patients (80 percent) whose rectums were preserved required multiple endoscopic polypectomies for recurrent polyps in the pouch (first patient) or their rectums (the other four patients). The patient who underwent the Koch procedure required surgery for recurrent polyps in her pouch.
CONCLUSIONS: One-half of the patients who initially underwent rectal preservation required subsequent proctectomy. The number of colonic or rectal polyps does not influence the choice of the surgical procedure. Both restorative proctocolectomy and subtotal colectomy with ileorectal anastomosis need endoscopic follow-up because of the high recurrence rates of juvenile polyps in the remnant rectum or pouch.