INTRODUCTION

Recent decades have witnessed rapid progress in the medical treatment of ulcerative colitis (UC), with available biological agents and small-molecule drugs. Some patients with UC, however, still require colectomy for their medically refractory disease or colitis-associated neoplasia (CAN). A variety of surgical strategies exist for the treatment of UC, and all options must share a common goal: to alleviate symptoms of the disease and to reduce the risk for cancer development, all the while maintaining the highest patient quality of life (QoL). A meta-analysis of population-based studies reported a cumulative risk of colectomy in adult patients with UC of 4.4%, 10.1%, and 14.6%, respectively, with a trend of a decreased surgical rate as compared with the “prebiological era.” With better control of inflammation of UC with potent medications, the indication for colectomy is changing over time.

Patients with UC who require colectomy typically have the following scenarios. The patient presenting with peritonitis, massive uncontrolled hemorrhage, and/or visceral perforation warrants immediate surgical intervention. Next, the patient who fails to maintain QoL on advanced medical therapy benefits from a discussion of surgical options. Finally, the patient with good control of colitis-associated symptoms but who suffers from untoward side effects from medications or has findings of CAN deserves the offering of surgery. All of the above scenarios are best handled with a multidisciplinary approach, with early surgeon participation in the team. In a population study of Olmsted County, 90% of colectomies for UC were performed for medically refractory disease, 5% for fulminant colitis, and 5% for CAN before 1990; the numbers were 56%, 26%, and 12%, respectively from 1990 to 2004. The study also reported that total proctocolectomy (TPC) with ileal pouch-anal anastomosis (IPAA) was performed in 54%, TPC with end-ileostomy in 33%, and subtotal colectomy (STC) and ileostomy in 12%.

Common surgical treatment modalities in patients with UC requiring colectomy include TPC and end ileostomy (EI), RPC and IPAA, colectomy with ileal rectal anastomosis (IRA), and continent ileostomy. Continent ileostomies, mainly the Kock pouch, are discussed in separate chapters. The selection of an appropriate surgical strategy should be individualized for each patient. Consideration is given to the patient’s overall quality of health, his or her clinical status, comorbidities, and personal goals of surgery, for example, preservation of bowel function, minimal number of surgeries, and avoidance of ostomy. Surgical options for the treatment of UC will be discussed in the following text, with emphasis on patient selection and preservation of QoL, common and uncommon methods of restoration of intestinal continuity and continence.

Despite careful planning and execution, complications of RPC and IPAA are common. Pouch-associated structural, inflammatory, functional, and neoplastic complications and metabolic morbidities are recently summarized in a recent consensus guideline from the International Ileal Pouch Consortium. Some patients with structural or neoplastic complications may require surgical pouch revision or pouch redo.

TOTAL PROCTOCOLECTOMY WITH PERMANENT ILEOSTOMY

Before the popularization of the IPAA in the early 1980s, TPC with a conventional Brooke’s EI was the most effective definitive surgery for UC. ^, Although RPC with IPAA has become the most commonly performed surgery in patients with UC following colectomy, there remains a role for TPC and EI in selected patients who desire a definitive operation accepting a permanent stoma. ^, The clinician should consider TPC with EI in those who do not desire an IPAA or who are not good candidates for an IPAA. These patients include those with impaired anal sphincter structure and/or pelvic floor function or those with reduced mobility or comorbidities. Typically, but not always, these are elderly patients and TPC with EI should be strongly considered in this population, although IPAA may be fitting in certain circumstances. ^,

Total proctocolectomy with EI is a desirable option in selected patients for several reasons. TPC with primary EI is a safe and curative operation that allows for the complete removal of at-risk colorectal mucosa, thus preventing disease-associated neoplasia. The procedure is best facilitated by using an intersphincteric approach for proctectomy rather than low stapling of the rectum with preservation of the anal transition zone (ATZ). TPC with EI can often be performed in a single operation without the need for staging, as it has fewer technical challenges than that required for RPC and IPAA. Although some have shown similar morbidity between RPC and IPAA, TPC with EI is associated with a reduced risk for severe complications, a characteristic ideal for elderly or frail UC patients requiring a surgical cure. Certainly, young and fit UC patients requiring surgery may also elect to pursue this approach for the benefits outlined above, and should not be discouraged from TPC or talked into pursuing an IPAA instead.

End ileostomy after TPC for UC without trying IPAA is referred to as primary ileostomy , as opposed to the term secondary ileostomy after pouch failure and subsequent fecal diversion. The primary EI with TPC carries a significantly lower risk of stoma-associated complications than the secondary ileostomy. TPC and Brooke’s ileostomy is certainly not without its drawbacks, and arguably the most significant is the permanent maintenance, care, and management of a lifelong ileostomy and stoma-associated complications. A permanent ileostomy can adversely affect body image and thus QoL, a parameter that is meant to improve after surgery in these patients. The patient may have pouching difficulties, stoma retraction or prolapse, stoma stenosis, and parastomal hernia, all of which may increase morbidity and reduce QoL to an even higher degree. Patients often experience difficulty in healing the perineal wound after proctectomy (e.g., persistent perineal sinus) even when an intersphincteric approach is performed, which occurs in at least 18% to 25% of this patient population. Since pelvic dissection is unavoidable to complete TPC, patients must accept a risk for pelvic nerve damage similar to that with IPAA, potentially leading to sexual and urinary dysfunction.

RESTORATIVE PROCTOCOLECTOMY WITH ILEAL POUCH-ANAL ANASTOMOSIS

Restorative proctocolectomy with IPAA has been a preferred surgical option for patients with UC for the last four decades. Most patients enjoy excellent QoL with a durable surgical and functional result, preserved natural route of defecation, and avoidance of a permanent conventional ileostomy. This technique has undergone modifications in its approach since its initial introduction in 1978. Contemporary principles and techniques have been applied to IPAA surgery, functional outcomes have improved, and pouch survival has remained high as described in a high-volume pouch center study.

Restorative proctocolectomy and IPAA consist of the removal of the colorectum and the creation of a pelvic ileal reservoir, which is constructed from varying lengths of the distal ileum. The reservoir is subsequently joined with varying methods, to the anorectal ring or a short rectal stump to recreate intestinal continuity. In patients with fulminant colitis, megacolon, or severe comorbidities, the procedure is performed in multiple stages. This typically involves STC with EI, followed by completion proctectomy, IPAA, and diverted loop ileostomy when health is restored, usually after a waiting period of >3 to 6 months. In highly selected patients who are otherwise fit and have no risk factors for poor healing (such as those with UC in remission and with CAN, or FAP), a single-stage IPAA may be a reasonable option to consider.

The first step in proceeding with restorative pouch surgery is the integration of the surgeon into the multidisciplinary team. This should occur early in the patient’s disease course to allow for thorough discussion of surgical options, and timing and anticipation of surgery with patient and family members. The discussion may be initiated anytime and is particularly important to begin when patients develop steroids-dependent or steroid-refractory UC while pursuing therapy with biologics or small molecule agents. Next, a full evaluation of the patient’s disease status, diagnosis, and health and functional status is necessary. Appropriate endoscopic, histologic, and radiographic testing to rule out Crohn’s disease (CD) or neoplasia is important as this may affect decision-making and options for surgery. The final decision regarding the timing and modality of surgery must be individualized and considerate of the patient’s desires and goals for surgery and clinical status. A preoperative visit by an experienced enterostomal therapist (ET) is needed for stoma site marking and orientation of the patient to living with an ostomy. A patient who is a candidate for RPC and IPAA, but does not desire such and is accepting permanent ileostomy, should never be convinced otherwise. If a patient is unsure of this decision, a staged approach is preferred, in that the disease is controlled with initial colectomy but all surgical options are still available.

SUBTOTAL COLECTOMY WITH END ILEOSTOMY/HARTMANN POUCH

Crile and Thomasin 1951 recommended STC or total abdominal colectomy (TAC) with ileostomy as an alternative to ileostomy in the setting of toxic megacolon to reduce mortality in patients. TAC has evolved into the procedure of choice for most patients with severe or fulminant UC. TAC can be performed with an open or laparoscopic approach, and careful handling of the colon is critical with either method to reduce morbidity and mortality associated with intraoperative fecal spillage. In the case of toxic megacolon with concern for friability of the colon wall and subsequent iatrogenic perforation, the surgeon is best to construct a skin-level blowhole colostomy with diverting loop ileostomy to decompress the colon and divert the fecal stream.

Several methods are used to deal with the distal bowel after TAC. One recommendation is to transect the bowel with a stapler at the level of the rectum (the confluence of the Tenia coli), but some propose that this makes subsequent pelvic surgery more difficult and may increase the risk for leak and pelvic sepsis with an internal exposed staple line across diseased tissue if rectal stump blowout occurs. Therefore, a better approach is to transect at the rectosigmoid colon, deliver the cut edge into the distal aspect of the abdominal wound, and mature it into a mucous fistula or staple it closed and implant it in the subcutaneous space. Both methods reduce the morbidity associated with a leak at the closure site, providing a sepsis-free pelvic inlet that is more amenable to subsequent surgery.

Perhaps one of the greatest benefits of TAC in patients with severe or fulminant UC is the management of a severely diseased colon while avoiding the morbidity associated with pelvic dissection, preserving future pelvic surgical options. With TAC, the patient easily regains health and nutrition to a normal state while discontinuing all UC medications despite retaining a diseased rectal remnant. Achieving good health (normally 3–6 months) in these patients is mandatory to mitigate risk in subsequent pelvic surgeries.

TOTAL COLECTOMY WITH ILEORECTAL ANASTOMOSIS

Total colectomy with IRA has taken a lesser role in the surgical treatment of UC with the popularization of IPAA. Total colectomy and IRA were used as the only restorative alternatives to permanent EI in patients with UC requiring surgery. Total colectomy with IRA is a less complex procedure with lower morbidity than either TPC or IPAA. However, poor bowel function due to a rigid, noncompliant rectum, proctitis, and fear of cancer in the rectum give pause to the surgeon considering this option.

Patient selection is again an important component of preoperative decision-making to ensure favorable outcomes after IRA. While IRA is a thoughtful treatment option in selected cases of relative rectal sparing UC or CD, this procedure must be tempered with the likelihood of having good functional and oncological outcomes. Reported overall morbidities ranged from 8% to 28%, with anastomotic leak rates from 2% to 7%. For this reason, diverting ileostomy should be considered in high-risk cases. One of the main advantages of IRA over IPAA is that IRA does not require deep pelvic dissection and thus theoretically minimizes the risk of adhesions, pelvic nerve, and organ damage. For the same reason, the negative impact of pelvic dissection on fertility, often encountered after RPC and IPAA, is thought to be less after IRA. Therefore, IRA may be considered in female patients desiring to conceive as an interim procedure until child-bearing is complete if the rectum is usable and the risk for malignancy is low.

To select patients with UC for IRA, one must evaluate the structural and functional quality of the remaining rectum (i.e., the impact of disease on an elastic, expandable rectum), the integrity of the anal sphincters, and the risk for neoplasia.

Neoplastic transformation after the IRA has been of great interest to this population. The rate of CAN in patients with UC increases with time, and maintaining the rectum increases this risk. The risk for rectal dysplasia in the setting of an IRA increases from 9% at 10 years to 25% after 20 years. Reported overall rate of rectal cancer after IRA ranges from 0% to 8% depending on sample size and follow-up time, with risk factors being a longer duration of UC and lack of compliance with postoperative surveillance. ^, Therefore, patients with long-standing UC who are not compliant with routine surveillance, or who already have dysplasia or cancer, may be better off avoiding IRA. Rectal cancer in IRA appears to have more aggressive tumor biology and often presents in more advanced stages. A “reactionary” approach to cancer after IRA is ill-advised.

With careful patient selection and close postoperative surveillance, some investigators demonstrated that the probability of having a functional IRA is 74% to 84% at 10 years and 46% to 69% at 20 years. ^, One of the largest studies in IRA for UC showed comparable functional outcomes between IRA and IPAA patients after careful case matching. Nonetheless, IRA is not a definitive operation for many UC patients who may require completion proctectomy for refractory UC proctitis, ^, rectal neoplasia, or CD of the rectum/anoperineum. If IRA fails to provide a durable surgical result, other surgical options may be available to preserve continence and/or QoL, including TPC, IPAA, or continent ileostomy.

OVERVIEW OF REVISIONAL POUCH SURGERY

Morbidity and pouch failure after RPC with IPAA are low in high-volume IBD centers, and most patients report high satisfaction with good QoL. However, poor function can occur due to a variety of structural, inflammatory, functional, or neoplastic complications that contribute to pouch dysfunction or even failure. Pouch failure is defined as a permanent diversion with or without pouch excision or pouch reconstruction. In selected cases of pouch failure, salvage surgery to revise or recreate the IPAA may be performed with good results.

The etiology of pouch failure should be carefully determined using historical data, abdominopelvic imaging, flexible pouchoscopy, histology, and examination under anesthesia. Pouch dysfunction due to structural, inflammatory, functional, or even neoplastic complications may be remedied with corrective pouch surgery before the declaration of true pouch failure and acceptance of permanent conventional ileostomy. For example, a persistent presacral sinus that fails endoscopic sinusotomy may respond to more invasive corrective or revisional surgical techniques. These techniques are commonly performed via a combined transbdominal/transanal approach. After identifying the cause of pouch function (as much as possible), the colorectal surgeon experienced in corrective IPAA surgery should discuss options extensively with the patient and outline all possible risks of intervention. The surgical approach will depend on the reason for failure, which highlights the critical importance of preoperative assessment/testing and experience with the numerous pouch pathologies that exist. The procedure may involve removing or recreating a new pouch, revising or recreating a new pouch-anal anastomosis, or adjusting the afferent or efferent limb of the pouch. Regardless of technique, corrective pouch surgery is a major endeavor requiring a highly motivated patient, an experienced surgeon, and a multidisciplinary team, and comes with it the possibility for great success but also pouch loss.

Outcomes after pouch revision surgery have been promising, with reported pouch survival after the corrective surgery as high as 85% to 89% after 5 years, depending on the etiology of pouch failure. A large study from the Cleveland Clinic of 502 patients undergoing redo IPAA surgery reported pouch survival of 80% (median follow-up of 7 years; range: 0.1–31 years) with favorable bowel function and QoL. The causes of failure of the original pouch leading to pouch redo are leaks/fistula (including pouch vaginal fistula) (N = 263; 52%), obstruction (N = 116; 23%), dysfunction (N = 45; 10%), pelvic/perianal abscesses (N = 43; 9%), pouchitis (N = 14; 3%), prolapse (N = 11; 2%), and neoplasia (N = 10; 2%). The underlying diseases at the time of failure of the original pouch included UC or indeterminate colitis (N = 419; 84%), CD (N = 32; 6%), and FAP (N = 41; 8%). Of the 502 cases, 207 (41%) had the construction of new pouches. In addition, 295 patients had redo or revision procedures on the existing pouches, including pouch repair (N = 160; 32%), partial ileal pouch resection (N = 80; 16%), pouch augmentation (N = 38; 8%), and pouch mobilization (N = 17; 3%). Other corrective surgeries for pouch complication or pouch failure which are not included in the above study are strictureplasty of pouch stricture and pouch pexy surgery for pouch prolapse. Complications, however, can occur after redo pouch surgery, including pelvic sepsis (N = 50; 10%), ileus/bowel obstruction (N = 81; 16%), anastomotic leak (N = 38; 8%), wound infection (N = 41; 8%), hemorrhage (N = 13; 3%), fistula (N = 13; 3%), stoma complications (N = 6; 1%), and bowel perforation (N = 2; 0.4%). Patients undergoing pouch revision surgery must be made aware and fully understand the risks of surgery and possible functional and QoL changes they may experience. Since QoL is one of the most important outcome measures after this surgery, it must be kept as a central focus when proceeding through this process, remembering that QoL is very subjective, with different patients defining a high QoL in very different ways.

TECHNICAL DETAILS OF REVISIONAL POUCH SURGERY

As the pathologies leading to pouch failure are better described and understood, the surgical approaches used to salvage dysfunctional pouches are expanded, refined, and tailored to address these pathologies. The most common cause of pouch failure is anastomotic leak from the pouch-anal anastomosis causing pelvic sepsis and, in some cases, sinus and fistula formation. However, patients may suffer other types of problems originating from the superior and inferior regions of the pouch (afferent and efferent limbs) or the pouch body itself, of inflammatory, structural, functional, or neoplastic origins. The purpose of this section is to discuss the surgical fundamentals of corrective pouch surgery, including some newer strategies employed to correct problems that have been identified or become better understood in the recent past. In general, pouch revision surgery is preceded by initial loop ileostomy creation and cooling off at least 6 months to rest the pouch and allow the patient to regain physical and mental health.

Redo Pouch-Anal Hand-Sewn Anastomosis for Pouch-Anal Anastomotic Leak

Pelvic sepsis from the pouch-anal anastomotic leak, if not immediately addressed, results in chronic inflammation, sinus and/or fistula formation, and pelvic fibrosis. During corrective surgery, the retained inflammatory tissue, fibrosis, or chronic abscess cavities in the deep pelvis must be incised and drained or excised. The pouch is mobilized to the pelvic floor to expose the anastomotic defect and associated fibrotic tissue is completely excised. Any residual abscess cavities are drained. In cases of severe sepsis, the distal pouch exhibits a loss of integrity during this dissection., and the posterior wall of the pouch seemingly disintegrates. There is often a thick rind of fibrotic tissue here that requires sharp removal ( Fig. 29.1 ). After the complete removal of affected tissue in the deep pelvis, an anal canal mucosectomy is then performed to remove residual anoderm from the dentate line to the top of the anorectal ring. The existing pouch is examined, and if it is otherwise healthy, can be gently debrided and reused. If not usable, it is excised and a new pouch is created if enough length is present. Mesenteric lengthening procedures are sometimes necessary to facilitate this process ( Fig. 29.2 ), including relaxing incisions in the visceral peritoneum, and complete removal of residual colonic mesentery from the sacral promontory to allow a straight axis for the pouch to reach the distal pelvis without tension. The distal pouch is then sewn to the internal sphincter at the dentate line with circumferential transanal sutures ( Fig. 29.3 ).

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