Treatment The standard treatment modality for patients with FAP is prophylactic proctocolectomy with IPAA because it offers decreased morbidity rates, increased functionality, and favorable quality of life (QoL) compared with other procedures.7 Total colectomy with ileorectal anastamosis (IRA) has been used for patients with FAP, but it does not eliminate the risk of rectal cancer. Risk of rectal cancer 10 years after IRA is 4.5%; 30% of patients develop rectal cancer by age 60 years.8 The 5-year survival after IRA is only 68%; the primary cause of death is rectal cancer.8
QoL for patients remains favorable after proctocolectomy with IPAA. Delaney and colleagues followed 1,895 patients over a 10-year period to assess the QoL of those who had proctocolectomy with IPAA. Determination of QoL was established using the Cleveland Global Quality of Life score. Overall, 96% of study participants were satisfied with the results of the procedure, and only 4.1% of patients required excision or permanent diversion of the pouch because of pouch failure.9 Patients younger than 45 years had a slightly higher QoL because they were less likely to experience incontinence and nocturnal seepage.9
As with any surgical procedure, proctocolectomy with IPAA can be followed by complications. The most common complications are anastomotic leakage, pouchitis, small-bowel obstruction, and pouch failure.8-11 Other associated morbidities include pouch-anal fistula, incisional hernia, and pelvic cysts.8
Anastomotic leakage is a rare but serious complication that can be fatal if not recognized promptly. A study conducted by Walker and colleagues determined survival rates in patients with anastomotic leakage following resection of the colon. Of the 1,722 patients evaluated, 5.1% developed anastomotic leakage, and the 5-year survival rate with leakage was 44.3%.10 Leakage has also led to metastasis beyond the intestinal lumen.10
The manifestations of anastomotic leakage are similar to those of generalized peritonitis, but a subclinical leak may only be recognized by abdominal CT with contrast.10 Subclinical leaks have a favorable outcome and usually require only percutaneous drainage and frequent follow-up.10 General anastomotic leaks, however, are much more serious and require urgent abdominal closure of the dehiscence.10
Pouchitis, another postoperative complication of proctocolectomy with IPAA, occurs most commonly within 2 years after surgery and more often in patients with ulcerative colitis who have undergone the procedure. Clinical symptoms of pouchitis are stool frequency and urgency, rectal bleeding, abdominal cramping, and pelvic discomfort.11 Definitive diagnosis of pouchitis is made through endoscopic findings.
Metronidazole (MTZ, Flagyl) is the treatment of choice in acute pouchitis (duration less than 4 weeks), while MTZ plus ciprofloxacin (Cipro) is the standard therapy in chronic cases.11 Some sources suggest using probiotics, such as lactobacilli, but this has so far been ineffective as primary treatment in pouchitis.11 Overall, pouchitis has a good prognosis and is an infrequent cause of pouch failure.
Conclusion At her 30-day postoperative evaluation, our patient reported that she was tolerating the procedure well, with only limited pain at the incision site. A postoperative CT revealed no signs of pouch failure. The patient was attending regular physical therapy sessions and said her QoL had returned to its preoperative level. She and the members of her immediate family have an appointment with a genetic counselor.
Because the patient lacked a complete family history at presentation, the likelihood that her FAP would be missed was high. If the condition had been left undiagnosed, she stood a 100% chance of developing colon cancer by age 50 years. FAP is a devastating disease that requires prophylactic proctocolectomy with IPAA to avoid untimely colon cancer and possible death. With continued postoperative follow-up, the prognosis in this case is very good. The postoperative treatment plan for FAP patients should always include genetic counseling in order to avoid missed diagnoses and untreated FAP in future generations. JAAPA
Mike Deeter works at NextCare Urgent Care in Phoenix, Arizona. He has indicated no relationships to disclose relating to the content of this article.
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