Surgical Treatment of FAP

As FAP is 100% precancerous, colectomy is recommended. Treatment of FAP is influenced by the nat ural history of the disease, which is variable. If patients are left long enough without colectomy, they will all develop carcinoma. The sooner is patient diagnosed, the better prognosis could be if colectomy is performed. There are three possibilities of surgical treatment. Each has its pros and cons:

• Total colectomy with permanent ileostomy (in cases of malignancy in the lower rectum)

• Total colectomy with ileoanal anastomosis (IAA) (poor functional results)

• Subtotal colectomy with ileorectal anastomosis (IRA) (good functional results but risk of recurrence in the rectal stump)

The procedure of choice is the subject of much debate, particularly since restorative proctocolecto-my became feasible [21]. The other options are colec-tomy and IRA, proctocolectomy or colectomy and rectal mucosectomy without restoration of GI continuity. Conventional panproctocolectomy has the advantage of eliminating all colorectal polyps and virtually eliminating the risk of carcinoma of the large bowel. This operation does not protect against ampullary or small-bowel malignancy. In addition, there is the burden of an ileostomy in a condition where 50% of family members are at risk of disease, and the patient is exposed to the small risk of pelvic nerve damage with resulting bladder and sexual problems. Also, there is a perineal wound, which although less likely to break down than after procto-colectomy for inflammatory bowel disease, may leave the patient with a persistent sinus.

Intersphincteric excision of the rectum reduces risk of pelvic nerve injury, and rectal mucosectomy eliminates problems with the perineal wound. Risk associated with a long rectal mucosectomy is that all the diseased mucosa may not be removed, and there is then a slight risk of carcinoma developing in any remaining remnant. The overwhelming disadvantage, however, of any procedure that leaves a permanent ileostomy, is that it is a poor advertisement of treatment for other members of the family. Although a patient with an ileostomy can lead a full and active life, it is difficult to convince a young and active family member, who may well be asymptomatic, to undergo such a procedure. It is for this reason that sphincter-saving procedures such as colectomy with IRA or restorative proctocolectomy have become popular [22].

The procedure of colectomy and IRA has the advantage that GI continuity is restored and bowel function is reasonable. Its disadvantage is that polyps are left in the rectum, with the potential of malignant change. To prevent this unfortunate outcome, patient need to have repeated fulguration of residual or newly formed polyps [23]. Not only can this procedure be uncomfortable, it also requires the patient to return repeatedly for rectoscopic examination. In addition, although it would seem logical, if the adenoma-carcinoma sequence is accepted - that removal of polyps removes the risk of carcinoma - this does not necessarily follow. There is evidence to suggest that carcinoma can develop de novo in rectal mucosa not occupied by polyps.

Mucosal proctectomy and pelvic ileal reservoir, now generally termed restorative proctocolectomy, has the theoretical advantage that the disease is eradicated, GI continuity is restored and continence is maintained. It appears, unfortunately, from some results reported, that bowel function is not always as satisfactory as after IRA. However, these results have in the main been described in patients who have suffered from ulcerative colitis. There is now substantial evidence either from our own experience or from others that the clinical results of restorative procto-colectomy for polyposis are far superior to those reported in patients with colitis. Nevertheless, restorative proctocolectomy is a complex procedure and although mortality is very low, morbidity can be high. Although results are steadily improving, the procedure is still developing, and the long-term results in FAP are unknown, perhaps due to the small number of patients. Further modifications may be desirable before it can be categorically accepted as the operation of first choice for all patients with FAP.

The conventional policy in many units dealing with these patients is still to perform a colectomy and ileorectal anastomosis in the first instance, provided patients are likely to be reliable in attending for follow-up. Follow-up at regular 6-month intervals is usually necessary so that rectal polyps, if present, can be destroyed by fulguration. In order to determine if this is still a reasonable policy, it is necessary to access the risk of developing a rectal carcinoma after IRA.

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