The prognosis of clinical course and the analysis of the frequency of the inflammation and dysplasia in the intestinal J-pouch at the patients after restorative proctocolectomy due to FAP

Abstract

PurposeThe main operative method in familial adenomatous polyposis (FAP) patients is restorative proctocolectomy with “J”-shaped pouch and temporary loop ileostomy. The aim of the study was the analysis of the frequency of the dysplasia and inflammation in the intestinal pouch and prognosis of the clinical course in FAP patients after restorative proctocolectomy.MethodsA group of 165 FAP patients (86 females and 79 males, mean age 22.49 ± 12) subjected to a restorative proctocolectomy in the years 1985–2009 was analyzed. Clinical data coming from follow-up observation in the period of 2004–2009 were evaluated. In all patients, clinical examination and endoscopy with polypectomy and/or biopsy of pouch mucosa were done.ResultsThe mean time of pouchitis occurrence after an ileal pouch-anal anastomosis was 6 months. Mean time for low-grade dysplasia was 14 months. The time difference of low-grade dysplasia after the above procedure as compared to pouchitis alone was substantial. Mean time for high-grade dysplasia was 16 months and for neoplasia even 19 months. It was estimated that early pouchitis happening within the first year after surgery occurs in 5% of patients, low-grade dysplasia 4 years later in 7% of cases, high-grade dysplasia 7 years later in around 10% of patients and neoplasia 14 years after surgery in 15% of cases.ConclusionsIn conclusion, the Polyposis Registry encompassing whole country is the best way of controlling FAP patients. The regular lifelong endoscopic monitoring gives the opportunity of the early detection of the dysplasia and can protect against neoplasia.

Lower fecal pH may be a novel indicator of pouchitis after IPAA in patients with FAP or metachronous Lynch syndrome

“…Pouchitis is a high‐incidence complication after IPAA, and approximately half of all patients with inflammatory bowel disease suffer from pouchitis; however, the incidence of pouchitis is much lower in patients with hereditary CRC than in those with inflammatory bowel disease . The pouchitis rate in this study was not much lower than the rates reported in studies on hereditary CRC; nonetheless, the rate in this study was lower than the rates reported in studies on ulcerative colitis, which may be a reflection of the surgical technique and the particularities of ulcerative colitis.…”

Prophylactic surgery in familial adenomatous polyposis (FAP)—a single surgeon’s short- and long-term experience with hand-assisted proctocolectomy and smaller J-pouches

“…The incidence of pouchitis in FAP patients varies widely in literature from about 5 % [52] to more than 20 % [53], and the definition of pouchitis is controversial. Furthermore, pouchitis is normally dealt with by general practitioners, so therefore data regarding the incidence of pouchitis in this series has not systematically been documented.…”

Chemoprevention in patients with genetic risk of colorectal cancers

“…FAP patients are also at risk for extracolonic manifestations including gastric and duodenual polyps, osteomas and dental anomalies, congenital hypertrophy of the retinal pigment epithelium, desmoids and other soft tissue tumors. The current standard of treatment includes intensive monitoring with prophylactic surgery (either restorative proctocolectomy or ileorectal anastomosis [IRA]) when the adenoma burden becomes unmanageable by endoscopy, often by 20 years of age [20,21]. While surgery is life-saving [22], reported postoperative quality-of-life outcomes are highly variable [23–26] and suggest that surgery may increase the risk of infertility [27,28].…”