Ce. Scottconner et al., FAMILIAL JUVENILE - POLYPOSIS PATTERNS OF RECURRENCE AND IMPLICATIONSFOR SURGICAL-MANAGEMENT, Journal of the American College of Surgeons, 181(5), 1995, pp. 407-413
BACKGROUND: Familial juvenile polyposis predisposes to the development
of carcinoma of the colon, Optimum surgical management and recommende
d surveillance of affected individuals are still being defined. STUDY
DESIGN: A retrospective review of experience with a kindred identified
in 1988 was carried out. RESULTS: Of 34 living members, 15 have been
investigated, and histologically typical juvenile polyps were found in
11, In each instance, polyps were most numerous in the right colon, w
ith few polyps in the descending colon and none in the rectum, Eight p
atients have had subtotal colectomies with ileorectal anastomoses; the
remaining patients were managed by polypectomy (with one recurrence a
fter ten years), In addition to juvenile polyps, polyps with adenomato
us or villous elements were identified in three patients, One of these
patients had invasive adenocarcinoma in a large mixed polyp of the ce
cum. Two patients with polyps had coexisting carcinoma of the stomach.
All patients have been followed up with periodic upper and lower gast
rointestinal endoscopy. Polyps have recurred in the rectal remnants of
three patients at a mean of 36 months after subtotal colectomy, Two p
atients have undergone conversion to total proctocolectomy with ileoan
al anastomosis and J pouch; one patient was found to have juvenile pol
yps in the pouch 40 months after surgery. CONCLUSIONS: Despite the pre
ponderance of right-sided polyps at initial diagnosis, the rapid recur
rence of polyps after subtotal colectomy argues in favor of performing
proctocolectomy with preservation of anal sphincter function (restora
tive proctocolectomy) at the time of initial surgery Patients with a s
mall number of polyps may choose instead to undergo periodic colonosco
py with colonoscopic polypectomy, An algorithm for surveillance and fo
llow-up is proposed.