Surgical treatment of familial adenomatous polyposis: dilemmas and current recommendations

Abstract

Familial adenomatous polyposis (FAP) is an autosomal dominant inherited syndrome characterized by multiple adenomatous polyps (predisposing to colorectal cancer development) and numerous extracolonic manifestations. The underlying genetic burden generates variable clinical features that may influence operative management. As a precancerous hereditary condition, the rationale of performing a prophylactic surgery is a mainstay of FAP management. The purpose of the present paper is to bring up many controversial aspects regarding surgical treatment for FAP, and to discuss the results and perspectives of the operative choices and approaches. Preferably, the decision-making process should not be limited to the conventional confrontation of pros and cons of ileorectal anastomosis or restorative proctocolectomy. A wide discussion with the patient may evaluate issues such as age, genotype, family history, sphincter function, the presence or risk of desmoid disease, potential complications of each procedure and chances of postoperative surveillance. Therefore, the definition of the best moment and the choice of appropriate procedure constitute an individual decision that must take into consideration patient's preferences and full information about the complex nature of the disease. All these facts reinforce the idea that FAP patients should be managed by experienced surgeons working in specialized centers to achieve the best immediate and long-term results.

Keywords: Adenocarcinomas; Familial adenomatous polyposis; Ileal pouch-anal anastomosis; Ileorectal anastomosis; Restorative proctocolectomy; Surgical treatment.

Figure 1

British surgeon John Percy Lockhart-Mummery (1875-1957) who established the famous Polyposis Registry in St. Marks Hospital (London).

Figure 2

PAF specimen showing numerous polyps along the colon and at least seven simultaneous malignant lesions.