List of Abbreviations
Anal transitional zone
Inflammatory bowel disease
Ileal pouch-anal anastomosis
Quality of life
Total abdominal colectomy
Ulcerative colitis (UC) is a chronic mucosal inflammatory disease of the colorectum that can have a widely varied presentation. Although many patients suffering from UC are successfully treated with various medical combinations that mitigate symptoms, there are several clinical scenarios in which the consideration of surgical intervention is warranted. 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).
There are several patient scenarios in which surgery is indicated. First, the patient presenting with peritonitis, massive uncontrolled hemorrhage, and/or visceral perforation warrant immediate surgical intervention. Next, the patient who fails to maintain acceptable QOL on aggressive medical therapy benefits from surgical discussion. Finally, the patient with good control of colitis-associated symptoms but who suffers from untoward side effects from medication use, or has findings of dysplasia on surveillance colonoscopy deserves the offerings of surgery. All of the above scenarios are best handled with a multidisciplinary approach, with early surgeon participation in the team.
Choosing the most appropriate surgical strategy should be individualized to each patient. Consideration is given to the patient’s overall quality of health, his clinical status, and his personal goals of surgery, that is, preservation of bowel function, minimal number of surgeries, avoidance of ostomy, and so forth. Surgical options for treatment of UC will be discussed in the following text, with emphasis on patient selection and preservation of patient QOL. In addition, both common and uncommon methods of restoration of intestinal continuity and continence will be described and discussed.
Total Proctocolectomy With Permanent Ileostomy
Prior to the development of the ileal pouch-anal anastomosis (IPAA) in the early 1980s, total proctocolectomy with end ileostomy (TPC) was the most effective definitive surgery for UC ( Fig. 23.1 ). Although restorative proctocolectomy with IPAA has become the most commonly performed surgery for patients with UC requiring surgery, there still remains a role for TPC in selected patients who desire a definitive operation and are accepting of a permanent stoma.
The clinician should consider TPC in patients who choose not to undergo IPAA or who are not good candidates for IPAA. These patients include those with impaired anal sphincter function or those with reduced mobility or comorbid disease. Typically these are elderly patients, and TPC should be strongly considered in this population.
TPC is a desirable option in selected cases for several reasons. Most importantly, it is a safe and curative operation that allows for complete removal of at-risk colorectal mucosa, thus preventing disease-associated dysplasia or cancer development. This is best facilitated by using an intersphincteric technique for proctectomy ( Fig. 23.2 ) rather than low stapling with preservation of anal transition zone. TPC can often be performed in a single surgical setting (one operation) with less technical challenge than that required for IPAA. Although some have shown a similar morbidity between TPC and IPAA, TPC is associated with less-severe complications, a characteristic ideal for elderly UC patients requiring a surgical cure. Certainly, young and fit UC patients who need surgery may also choose this pathway for the benefits outlined above and should never be discouraged from TPC or convinced to pursue IPAA.
TPC is certainly not without its drawbacks, and arguably the most significant is the requirement for a permanent ileostomy, which carries with it the associated risks for pouching difficulties, parastomal hernia, and stomal prolapse. Permanent ileostomy may negatively impact body image and thus QOL, a parameter that is meant to improve after surgery in these patients. There is evidence that IPAA is associated with better perception of body image than a permanent stoma, even though QOL is similar compared to TPC in a matched study. Others have shown that patient QOL is restored to that of the general population after TPC for UC or is not significantly affected. Patients often experience difficulty in healing the perineal wound even when intersphincteric approach is utilized, and delayed wound healing may occur in 18%–25% of this population, creating great morbidity that is difficult to treat. Since pelvic dissection is unavoidable to complete TPC, patients must accept a risk for pelvic nerve damage similar to that with IPAA, which may lead to irreversible sexual and urinary dysfunction.
Restorative Proctocolectomy With Ileal Pouch-Anal Anastomosis
Restorative proctocolectomy with IPAA has been an ideal surgical option for patients with UC for nearly 4 decades. In most cases, patients enjoy excellent QOL with a durable surgical and functional result, avoiding the need for a lifelong ileostomy. IPAA has undergone several modifications in its approach since it was popularized in the early 1980s. Over this time, contemporary approaches have been applied to IPAA surgery, functional outcomes have improved, and pouch survival has remained high as described in a high-volume IPAA center study.
IPAA surgery consists of removal of the colorectum and creation of an ileal reservoir, which is constructed from varying lengths of distal ileum. The reservoir is subsequently joined, using varying methods, to the anorectal ring to recreate intestinal continuity. In patients with severe fulminant colitis or who have poor health, the procedure is performed in multiple stages. This typically involves colectomy with end ileostomy, followed by proctectomy with diverted IPAA when health is restored, usually after a waiting period >6 months. In very carefully selected patients who are otherwise fit and have no risk factors for poor healing, a single-stage IPAA may be a safe option.
The first step in proceeding with restorative pouch surgery is the addition of the surgeon to the multidisciplinary team. This should occur early in the patient’s disease course to allow for thorough discussion of surgical options, and time for the patient to consider options and discuss with family members or other patients who have undergone the procedure. Next, a full evaluation of the patient’s disease, diagnosis, and health status is necessary. Appropriate testing to rule out Crohn’s disease (CD), cancer, or dysplasia is important as surgical decision-making and options for surgery may be affected. The final decision regarding timing and extent of surgery must be individualized and consider the patient’s desires and goals for surgery in addition to clinical status. A preoperative visit by an experienced enterostomal therapist is helpful for preoperative stoma site marking and orientation of the patient to living with an ostomy. A patient who is a candidate for IPAA, but does not have a desire for such, and is accepting of a permanent ileostomy, should never be convinced otherwise. If a patient is unsure of this decision, a staged approach is ideal, in that the disease is controlled but all surgical options (TPC, total abdominal colectomy [TAC], IPAA) are still available.
Deciding Among Pouch Configurations
The first reports of IPAA decades ago described an IPAA using an S-shaped ileal pouch in combination with a handsewn anastomosis ( Fig. 23.3 ). A variety of configurations have been considered over time, including the S, J, W, and H configurations ( Figs. 23.3 and 23.4 ). Of these, the J pouch is the easiest to construct, facilitated by stapling devices, and has become the most commonly used configuration to date. The S and W pouches require a longer segment of distal ileum and typically necessitate a handsewn approach, thus are more time-consuming and technically challenging to create. The J pouch configuration is most commonly used unless adequate mesenteric length is not available, as creating a tension-free pouch-anal anastomosis is the most critical step to successful pouch surgery. In the case where a J pouch will not reach without tension, an S pouch may be helpful as its configuration allows for a longer reach (2–4 cm longer than J pouch) into the pelvis ( Fig. 23.5 ).
A metaanalysis including over 1500 patients with the three main pouch configurations (S, J, W) showed no significant difference between the groups in terms of postoperative complications including leak, stricture, pouchitis, sepsis, and pouch failure. With respect to function, the J configuration was associated with more frequent bowel motions than either S or W pouch, and patients with a J pouch reported more use of antidiarrheal medications. However, those with an S or W pouch were more likely to have difficult pouch evacuation requiring per anal intubation. Seepage and incontinence were similar among all three groups. Overall, the decision regarding pouch configuration must be individualized to the characteristics of the patient, with a J configuration typically considered the best overall choice in terms of ease of creation and acceptable functional results.
Stapled Versus Handsewn (Mucosectomy) Anastomosis
Stapled IPAA has been the preferred method over handsewn IPAA since the introduction of stapling devices several decades ago. It is less-time-consuming and associated with better outcomes than handsewn IPAA. In addition, patients with UC undergoing a stapled IPAA rarely develop cancer in the preserved anal transition zone (ATZ). The stapled IPAA is carried out with either a single- or double-stapled approach, and the IPAA is joined to the ATZ, thus preserving anal sensory epithelium ( Fig. 23.6 ). Conversely, a handsewn IPAA is performed by first removing all anorectal mucosa from the dentate line proximally to the anorectal transection ( Fig. 23.7 ). The IPAA is then delivered into the pelvis and sutured to the internal sphincter in a radial fashion. If properly performed, the anal sensory epithelium and all rectal mucosa are removed in this method.
Pooled data comparing handsewn to stapled IPAA have shown similar outcomes in terms of postoperative complications, sexual function, QOL, and rate of ATZ dysplasia. Bowel frequency was also similar among groups. However, nighttime seepage, incontinence to liquid stool, and nighttime use of perineal pad were more frequent in patients who underwent handsewn IPAA. A more recent single institution study of over 3000 IPAA patients similarly suggested worsened incontinence, seepage and pad use in handsewn group, and also reported a higher QOL and happiness after surgery in the stapled IPAA group versus handsewn IPAA group. Overall, a stapled approach appears to be the preferred method of IPAA creation with less complications, ease of creation, and better functional results over handsewn IPAA. One may argue that there is a role for each method, therefore, final decision-making must be individualized with specific patient factors being considered.
Revisional Pouch Surgery
Patients and clinicians alike have enjoyed the overall good results of IPAA creation in those suffering from colitis. Morbidity and pouch failure rates are low in high-volume centers, and most patients report high satisfaction with good QOL. However, there are instances in which IPAA surgery is unsuccessful in providing acceptable bowel function and QOL due to complications after surgery. In these cases, carefully selected patients may benefit from salvage surgery to revise or recreate the IPAA.
Revisional IPAA surgery is commonly performed via a combined transabdominal/transanal approach. The pathology is identified and corrected, which may involve disconnection of the pouch-anal anastomosis followed by recreation of the anastomosis to a revised or newly created IPAA. Repeat pouch surgery is a major endeavor and requires a motivated patient and experienced IPAA center team.
Studies evaluating the success of revisional pouch surgery have been promising and insightful. The most common indication for revisional pouch surgery was pelvic sepsis, with several studies reporting a pouch survival as high as 85%–89% after 5 years, depending on etiology of pouch failure. The largest and most recent study from the Cleveland Clinic Foundation reported on 502 patients undergoing redo IPAA surgery. Over 50% of these patients suffered from IPAA failure due to septic complications. The authors reported a pouch survival of 80% (median follow up 7 years) with favorable bowel function and QOL reported.
IPAA failure in the setting of suspected CD requires special attention, as technical failure after IPAA and subsequent pelvic sepsis may mimic signs and symptoms of CD. Garrett et al. reported on patients with a diagnosis of CD following failed IPAA who were referred to a high-volume IPAA center. Seventy-nine percent of these patients were misdiagnosed and actually suffered from septic complications unrelated to CD. This subset of patients underwent redo IPAA with 84% retaining a functional IPAA and reporting favorable functional outcomes.
IPAA failure treated with revisional salvage surgery can be successful and result in favorable outcomes in very carefully selected patients. For greatest success, an experienced, multidisciplinary clinician team and a very motivated patient are imperative.