Open
Matthew Z. Wilson
David B. Stewart Sr
INDICATIONS/CONTRAINDICATIONS
Total proctocolectomy with end ileostomy (TPCI) is an extensive surgery that involves the removal of the entire large intestine (colon, rectum, and anus). Owing to the radical and irreversible nature of this procedure with a resultant life-long ileostomy, the indications for TPCI are limited and only used in instances that leave no feasible alternative when considering both disease- and patient-related factors.
Crohn’s Colitis with or Without Involvement of the Anoperineum
Since there is no medical or surgical cure for Crohn’s disease (CD), surgery in this setting is only indicated for disease-related complications that cannot be addressed by medical or endoscopic therapies. Given the chronic and recurrent nature of CD, and considering the high incidence of clinical recurrence rates following partial colectomies in Crohn’s of the large intestine, decisions regarding sparing the large intestine as well as decisions regarding constructing an anastomosis must be tailored to the patient. This chain of thought needs to take into account the particular disease complication requiring surgery (inflammation and related manifestations vs. dysplasia/cancer), the patient’s overall health status, the patient’s anticipated postoperative fecal continence, and the risk associated with the potential need for additional major surgery in the setting of future CD recurrences.
In general, TPCI for CD is reserved for the following scenarios. Patients with pan-proctocolitis who are either medically refractory to appropriate therapy or prove to be non compliant with treatment regimens are considered for this surgery. Such patients would have no feasible alternative surgery which would both remove their diseased intestine while providing them with an anastomosis with acceptable continence. As a subcategory of this group are patients who might otherwise have been offered an ileorectal anastomosis but who because of age, continence, and/or health-related reasons are felt to be better served with an end ileostomy. In this scenario, a TPCI removes the rectum that might otherwise have been stapled and left in discontinuity, which depending on the patient’s age would pose a risk for the subsequent development of rectal cancer. Patients who have undergone a prior partial colectomy for CD, but who now have a clinical recurrence should be considered for a completion proctocolectomy with an end ileostomy, depending on the involvement of their rectum, their length of remaining colon, and their current continence and frequency of bowel movements. Although the management options for low-grade dysplasia have been recently revisited in an effort to avoid radical surgery, patients with high-grade dysplasia or histologically proven adenocarcinoma of the large intestine in the setting of CD should undergo TPCI. Dysplasia and cancer are often multifocal in these patients, and since surveillance for colorectal cancer is more difficult in patients with inflammatory bowel disease (IBD), a TPCI will remove all of the at-risk tissue for cancer in the large intestine.
CD can concomitantly affect the perineum in the setting of Crohn’s proctocolitis, and the severity of perineal involvement should be taken into account in surgical planning. Although complex enough to warrant its own chapter, as a general principle of management, active perineal sepsis is a contraindication to a TPCI because of the need to operate in the infected field of the perineum. In this scenario, a total abdominal colectomy with an end ileostomy and a stapled rectal stump should be selected, allowing for resolution of perineal sepsis, with a subsequent completion proctectomy when the patient’s perineum poses a less challenging surgical field. This approach has the benefit of taking into account the additional operative time and blood loss associated with the need for soft tissue coverage of a perineum with this extent of disease, which often requires a rotational or pedicled myocutaneous flap.
Ulcerative Colitis in a Patient for Whom a Restorative Procedure Is Contraindicated or Not Desired
Unlike CD, ulcerative colitis (UC) has a putative surgical cure that involves the removal of the colon and rectum. For younger, healthier patients, a restorative procedure with an ileal pouch-anal anastomosis (IPAA) provides an option that, when properly performed, removes the symptoms of UC but without the requirement of a permanent stoma. Since an IPAA results in stools of a more frequent and more diarrheal character, the decision to construct an IPAA must take into account the patient’s preoperative continence, making it a less appropriate surgical option for older patients, for patients with limited mobility who cannot, when needed, proceed quickly to a bathroom facility, or for patients with preoperative incontinence. In addition, many surgeons divert patients undergoing an IPAA, which then requires a second surgery to close the diverting stoma; this should be taken into account for older and other higher risk patients. The indications for a TPCI in UC include medically refractory disease as well as the diagnosis of dysplasia or adenocarcinoma, when the considerations listed earlier are also present. Some patients may request surgery for UC because of dissatisfaction with the cost, the side effects, and the time commitment associated with medical therapy.
Synchronous Malignancies of the Large Intestine, with at Least One Malignancy Involving the Rectum
Although all patients with synchronous colorectal cancers do not require a TPCI, when one of these malignancies involves the rectum, the decision to spare the anus must be carefully weighed against the risks of metachronous cancers and poor postoperative continence. Patients with synchronous colorectal cancers have a significant risk for metachronous polyps (often advanced adenomas) and cancers, which may require future surgery of a greater difficulty given the postoperative state of the abdomen. Further, because some rectal cancers require chemoradiotherapy, this has implications for both postoperative continence as well as the risk of future surgeries if the large intestine is spared. These considerations carry even greater force for older patients, or patients with greater surgical risk, who would be better served with one major surgery and not two.
Familial Adenomatous Polyposis in a Patient for Whom a Restorative Procedure Is Contraindicated or Not Desired
Attenuated forms of familial adenomatous polyposis (FAP) spare the rectum to a degree that a total colectomy with an ileorectal anastomosis is a surgical option. In FAP that presents with carpeting of the entire colon and rectum with polyps, removal of the entire large intestine is required to diagnose and to prevent adenocarcinoma of the large intestine. The decision to perform a TPCI as opposed to a restorative procedure shares similar decision making in many respects to the scenario described for UC.
Contraindications and Final Comments
A TPCI is a lengthy procedure with two fields of operation (abdominal and perineal). It is not an appropriate surgery in an emergent setting, where a total colectomy with an end ileostomy and a stapled rectal stump should instead be selected. In an emergent setting, or in an elective setting with perineal sepsis, a total colectomy allows for resolution of these mitigating factors, with an elective completion proctectomy at a more opportune time. The added operative time and blood loss associated with resection of the anorectum is neither needed nor warranted in an emergent surgery. In addition, because a perineal surgical site is associated with a high incidence of infectious and wound-healing complications, it should be avoided in patients with moderate or severe protein-calorie malnutrition.
PREOPERATIVE PLANNING
Preoperative evaluation of the patient begins with a colonoscopy to confirm the diagnosis and the need for a proctocolectomy. Patients with a malignancy should have a preoperative carcinoembryonic antigen level obtained, as well as clinical staging with either a dual contrast-enhanced computed tomography (CT) scan of the chest, abdomen, and pelvis, or with magnetic resonance (MR) imaging if iodinated contrast is contraindicated. For CD, owing to its multifocal distribution, CT or MR imaging is critical to identify every site of the affected gut, especially within the small intestine. For patients with CD and UC, every effort should be made to discontinue or limit steroid use. The use of immunomodulators can be associated with decreased blood counts, including neutropenia, and the use of biologic agents can increase the risk of postoperative infections; these medications should be discontinued, and, if possible, a suitable time should be provided to allow the effects of these medications to resolve before surgery. The timing of surgery should balance the desire to limit or discontinue immunosuppression while not leaving the now untreated patient at risk for a flare of IBD.
In addition to obtaining preoperative laboratory tests such as a complete blood count, electrolytes, and renal and liver function tests, for IBD patients with chronic symptoms, albumin and, possibly, prealbumin levels should be measured to identify patients with protein-calorie malnutrition. A perineal dissection should be avoided in moderately or severely malnourished patients.
Although the benefits and risks of a mechanical bowel preparation have been debated by surgeons, the authors prefer its use for TPCI to avoid contamination of the pelvis or perineum during a difficult dissection where a proctotomy might be committed.
Because a TPCI will result in a permanent stoma, the proper siting of the ileostomy is extremely important, especially in obese patients or in those who have prior surgical scarring of the abdomen. Before surgery, a patient should undergo stoma-site marking by a stoma therapist. If possible, patients who have given consent for a TPCI should also undergo preoperative counseling and education regarding life with a stoma and proper stoma care. A group setting with other ileostomates is often helpful for prospective patients to avoid feeling isolated or unique in their need for an ileostomy, as well as providing the opportunity for patients to hear encouraging reports from those who have already had this surgery performed.
SURGERY
Venous Thromboembolic Prophylaxis
IBD, cancer, and lengthy operations are associated with a high risk for a perioperative venous thromboembolic event. At minimum, the use of chemoprophylaxis beginning on the day of the procedure (and before the induction of general anesthesia) should be part of routine patient care. Initiation of lower extremity pneumatic compression is required before the patient is anesthetized. It is the authors’ practice to also provide a dose of chemoprophylaxis at noon the day before surgery, and to prescribe 2 weeks (benign disease) or 4 weeks (cancer) of outpatient chemoprophylaxis following discharge for benign or malignant disease, respectively.