Another common presentation of small bowel CD is focal stricturing disease which leads to obstructive symptoms. There might be a single area or multiple areas along the length of the small bowel (Fig. 50.3). The strictures are the result of chronic inflammation in which the transmural scarring leads to compromise of the lumen. Previously, areas of multiple strictures were resected or by-passed. Both led to loss of functional bowel length which resulted in nutritional compromise. Furthermore, long segments of by-passed small bowel are associated with bacterial overgrowth and potential increased risk of malignancies in the by-passed segment. The use of strictureplasty in CD was first reported in 1982 [15]. The advantage of this technique is it can be repeated along the length of the bowel without the need for resection. The most commonly used technique is the Heineke–Mikulicz strictureplasty . This technique is useful for strictures up to 4–5 cm in length. The stricture is divided by making a longitudinal enterotomy along the antimesenteric border (Fig. 50.2). The enterotomy is then closed in a transverse fashion which reestablishes and widens the lumen of the bowel. Different types of strictureplasties have been developed for specific situations. For longer strictures (approximately 6–15 cm), the stricture can be folded upon itself and a side to side strictureplasty can be performed. In rare cases, a patient will have a long segment of bowel with multiple strictures or a single long stricture. Traditionally, this situation would have required a resection of the entire involved segment. To address this complex problem, Michellassi developed the long segment side-to-side isoperistaltic strictureplasty [16]. The segment of involved bowel is divided in the middle and then opened along the antimesenteric border. The bowel segments are overlapped in an isoperistaltic fashion and a side-to-side anastomosis is performed.

Strictureplasty is successful in relieving obstructive symptoms while preserving small bowel length. The Cleveland Clinic reported a large series of 698 strictureplasties performed in 162 patients without any septic complications or deaths [17]. The reoperation rate at 5 years was similar to patients who underwent formal bowel resections. For unclear reasons, in patients requiring reoperation for recurrent strictures, the majority of new strictures occurred at sites remote from the previous strictureplasties [18]. In a recent meta-analysis, strictureplasty is associated with an overall complication rate of 11 % with major complications occurring in 5 % of cases [19]. The median surgical recurrence rate was 24 % after a median follow-up of 46 months.

The transmural inflammation associated with CD results in the fistula formation. Fistulas can occur between different regions of the small and large bowel, adjacent organs, or the abdominal wall. Often, only one side of the fistula is involved with active CD and many entero-enteric fistulas may be asymptomatic. However, if a large segment of bowel is by-passed by the fistula either by connecting to a distal segment of bowel or to the abdominal wall, it may compromise nutrient and fluid absorption to such an extent that surgical division is required.

The initial treatment of a CD enteric fistula is similar to the management of any enteric fistula. Any septic focus needs to be controlled and the anatomy of the fistula delineated. An abdominal CT scan with oral water-soluble contrast is the test of choice because it is both diagnostic and possibly therapeutic. Ideally, any intra-abdominal fluid collection should be drained percutaneously. If the patient has any evidence of systemic infection, broad spectrum antibiotics should be started. Any fluid and electrolyte abnormalities need to be corrected and the patient should be placed on bowel rest. In patients with enterocutaneous fistulas, prompt consultation with a wound or enterostomal therapist is essential in order to avoid skin breakdown and to aid in determining the amount of fistula output with the bowel at rest. Early initiation of intravenous hyper-alimentation is important to prevent further nutritional deterioration. If the patient can be fed orally without significantly increasing the fistula output while maintaining their nutritional state that is preferred to chronic hyper-alimentation. An extended period of intense medical therapy and nutritional support should be attempted prior to surgery. The patient’s CD medication management needs to be optimized prior to surgery including the use of biologic therapies . The addition of infliximab to standard medical therapy for enterocutaneous fistulas has resulted in a 68 % closure rate compared to 13 % for placebo [20].

Surgery for CD fistulizing disease requires detailed counseling and operative planning. The patient should be seen and marked by a certified enterostomal therapist for a possible ostomy. At the time of surgery, it is important to distinguish between areas of active CD and “innocent by-stander” bowel. The fistula always originates from the active disease site and extends to non-diseased bowel. The principle in internal fistula surgery is to resect the active disease and repair the non-diseased bowel. If both ends of the fistula are involved with active disease then both segments of bowel are removed. The same strategy is used for fistulas to other organs or the skin. A recent review of the surgical treatment of enterocutaneous fistulas at the Cleveland Clinic reported a 90 day mortality of 3.5 % and a fistula recurrence rate of nearly 21 % over 3 months [21]. However, using the treatment algorithm of controlling sepsis, maximal nutritional and medical therapy and resection of active disease they achieved an 80 % closure rate.

In some CD patients, the disease is limited to the colon. However, CD is a disease of the entire bowel and patients may present with disease limited to the colon but later develop small bowel disease [22]. The colitis may demonstrate a chronic relapsing course or as a fulminant episode. Indications for surgery in Crohn’s colitis include intractable symptoms, fistula formation, stricture formation or severe perianal disease. Fulminant Crohn’s colitis may present with massive hemorrhage, free perforation, or systemic toxicity. The surgical management of these patients is similar to patients with fulminant ulcerative colitis. A subtotal colectomy with ileostomy and oversewing of the rectum is the preferred operation. Once the patient’s overall medical condition improves and there is no longer evidence of active disease, intestinal continuity can be reestablished by performing an ileorectostomy if the rectum is not involved with Crohn’s disease.

In CD patients with segmental involvement of the colon, limited resection is the preferred approach. The Mayo Clinic experience demonstrates that resection of the focal Crohn’s colitis leads to 86 % of patients remaining stoma free for more than 10 years [23]. However, nearly 50 % will experience a recurrence in the colon and nearly a third require a second operation for that recurrence. Due to the high recurrence rate, some prefer to offer total proctocolectomy with a permanent end ileostomy as the treatment of choice for segmental Crohn’s colitis. Certainly for patients with severe perianal fistulizing CD and segmental colitis a total proctocolectomy and ileostomy should be performed.

Another consideration in Crohn’s colitis is the possibility of colon cancer. A meta-analysis by Jess et al. reported nearly a doubling of the standardized incidence ratio for colorectal cancer in Crohn’s patients compared to the general public [24]. Any colonic stricture needs to be thoroughly evaluated for a potential malignancy. Furthermore, 10 % of CD patients with a colonic malignancy will have synchronous tumors. In this setting, the preferred operation is a total abdominal colectomy with ileorectostomy.

Perianal CD is a difficult management problem. Eleven percent of patients will present with perianal disease [22]. There are numerous manifestations of perianal disease including: hypertrophic anal skin tags, ulceration, fissures, anal stenosis, abscesses and fistulas. A patient may have one or multiple manifestations of perianal disease. Common presenting symptoms include severe pain, mucus discharge, bleeding, fecal urgency or incontinence. Surgery for perianal CD is directed at symptom management. Extensive anal operations should be avoided as scarring and recurrent disease may cause irreversible damage to the anal sphincter mechanism resulting in fecal incontinence. Procedures should be limited to controlling local sepsis and preventing recurrent sepsis.

Perianal abscesses need to be drained. Antibiotics alone are not adequate treatment. At the time of abscess drainage if an associated anal fistula is discovered, a loose draining Seton should be placed to provide long-term drainage. If no internal anal opening of a fistula is found, the abscess cavity can be packed as one would a routine perianal abscess. For larger abscess cavities, a mushroom tip rubber catheter can be secured into the cavity to provide long-term drainage.

In patients with complex perianal CD disease , especially fistula disease, imaging is very helpful in guiding management. The combination of endoanal ultrasounds, pelvic MRI and examination under anesthesia will correctly identify 100 % of pathology when at least two of the modalities are used [25]. While surgery is directed at controlling symptoms, newer medical therapies are directed at reducing the initiating inflammatory process and promote fistula healing. All the biologic agents tested to date have excellent efficacy at closing perianal fistulas with success rates between 30 and 100 % either when used alone or combined with surgery [26]. It should be noted that initial surgical control of local sepsis prior to initiation of biologic therapy leads to higher rates of closure. According to the a recent global consensus statement on the treatment of perianal fistulizing CD use of anti-tumor necrosis factor agents as first line therapy is the gold standard of treatment [27]. Once CD medical therapy is optimized and all local sepsis has resolved if there is not spontaneous closure of the fistula an attempt at surgical closure may be attempted.

Complex dissections and flap procedures should be avoided in CD patients with anal fistulas to minimize any unnecessary disruption of the sphincter mechanism. A surgical option for these CD fistulas is to fill the fistula track with a slowly absorbable material. Fibrin glue injected into the tract has been modestly effective in healing perianal fistula tracts [28, 29]. Another option is to place a plug of bio-absorbable material in the tract which permits slow scarring of the tract and eventual closure [30]. Success rates for these procedures are quite variable with most reports closure rates for all patients, not just CD, between 20 and 60 % [31]. The newer ligation of the intersphincteric fistula tract (LIFT) procedure has been shown to have consistently higher fistula closure rates, 70–80 %, in multiple studies of patients with and without CD [32, 33]. An exciting new approach is the use of adipose deprived stem cells as a treatment of Crohn’s perianal fistula. After a number of promising small series, recent 2 year follow-up of a phase II trial of 43 patients has demonstrated an 80 % closure rates [34]. Larger trials are now being undertaken to assess this treatment approach.

In patients with extensive medically refractory perianal CD the sphincter mechanism may become impaired leading to fecal incontinence or severe pain such that proctectomy is the only treatment option. Up to 25 % of patients with persistent perianal CD will eventually require proctectomy [35]. Fecal diversion often temporarily improves perianal disease but when reversed, active disease frequently returns. Surgery cannot cure perianal CD. However, conservative surgical management of perianal CD can greatly improve a patient’s quality of life.

Despite significant advances in the medical treatment of CD, surgery remains an integral aspect of CD treatment. Although biologic therapies are much more effective at symptom control and reducing the need for hospitalization in CD, population studies have not demonstrated a decrease in the need for surgery [36]. For the foreseeable future, surgery directed at treatment of medically refractory symptoms or complications of CD will continue to be performed ideally by specialist surgeons well versed in the newer aspects of CD care and working in collaboration with the treating gastroenterologist to optimize patient outcomes.

Chronic ulcerative colitis (CUC) is a recurring inflammatory condition limited to the colon and rectal mucosa. Since the disease is limited to colon, surgery is curative for the intestinal manifestations of the disease. By definition, the inflammation begins in the rectum and progresses uninterrupted for a variable distance into the colon (Fig. 50.4). The disease course is notable for low level chronic intestinal inflammation with intermittent worsening of the inflammation. These flares of disease activity result in worsening symptoms frequently manifested as by abdominal pain and bloody diarrhea. In a minority of patients, the initial presentation of CUC is a severe acute illness associated with high fever, tachycardia, abdominal pain, distension, and bloody diarrhea. Toxic, or fulminant, colitis is a medical emergency and requires rapid assessment and treatment [37]. Ideally, these patients should be managed by both a gastroenterologist and the surgeon experienced in the care of CUC.

Surgery in CUC is divided into two categories, emergent and elective. The operation performed is influenced by the setting in which it is performed. Emergency operations are performed for life threatening complications such as massive hemorrhage or toxic colitis or severe medically refractory disease requiring hospitalization. In this emergency setting, a definitive operation is not recommended. Elective surgery is undertaken to treat intractable symptoms of the disease or for dysplasia or malignancy in the setting of CUC. In appropriate patients a definitive operation can be performed. A definitive operation removes the entire colon and rectum. In many patients, a restorative operation can be performed avoiding the need for a permanent ileostomy.

In appropriately selected patients, the best surgical option is total proctocolectomy with ileal pouch-anal anastomosis (IPAA). IPAA avoids the need for a permanent stoma and maintains the normal route of defecation. This operation is technically demanding and should be performed by surgeons comfortable with the procedure. Long-term follow-up of IPAA patients has demonstrated durable functional results with a high degree of patient satisfaction.

Emergency surgery is performed in patients with fulminant disease, or toxic megacolon or rarely massive hemorrhage. In the emergency setting, the goal of the operation is to remove the abdominal colon, construct an ileostomy, and leave the rectum undisturbed. Preservation of the rectum maintains the option of a future restorative procedure, such as an ileal pouch anal anastomosis (IPAA). Using this “damage control” approach allows the patient to recover from their acute illness, discontinue any immunosuppressive medication, and improve their nutritional state before a definitive operation is undertaken.

Fulminant colitis is the initial presentation in 10 % of CUC patients [38, 39]. Truelove and Witts defined the clinical syndrome of fulminant colitis [40]. It is characterized by the sudden onset of severe bloody diarrhea (more than ten per day), abdominal pain, dehydration, and anemia. In addition, the patient must have at least two of the following: tachycardia, temperature greater than 38.6 °C, leukocytosis and hypoalbuminemia . These patients are extremely ill and require immediate medical attention. Initial therapy involves aggressive intravenous fluid resuscitation, correction of electrolyte abnormalities and anemia. If the patient has abdominal distension a nasogastric tube should be placed. Stool cultures should be obtained to rule out C. difficile or hemorrhagic infectious enteritis. A patient with known CUC may be started on intravenous steroids. In patients without a diagnosis of CUC, an endoscopic evaluation of the colon needs to be performed to help establish the diagnosis. A complete endoscopic evaluation of the colon is not required and may be unsafe in this setting. If the patient is clinically stable, there is no need for antibiotic therapy. However, in the presence of a fever or leukocytosis, broad-spectrum antibiotics should be initiated after cultures are obtained. Close observation for 24–48 h while on maximal medical therapy is crucial. If there is no improvement, or if the patient’s condition deteriorates, then surgery is advised. During this observation period, the development of peritonitis or hemodynamic instability requires immediate operation.

Other complications requiring immediate surgical evaluation are toxic megacolon and massive hemorrhage. Toxic megacolon may be seen in the setting of fulminant colitis or in isolation. Transverse colon dilatation greater than 5.5 cm defines the radiographic criteria of toxic megacolon. The entire colon or an isolated segment of the colon (usually transverse) is involved with gaseous distension. Clinically, the patients have significant abdominal distension and pain. They may also have fever and a leukocytosis. These patients are treated in a similar fashion to those with fulminant colitis. Operation is indicated if the patient’s clinical or radiographic status worsens, or if there is no improvement after 24–36 h of medical therapy. Profound intestinal hemorrhage is a rare complication of CUC. Aggressive fluid and blood-product resuscitation is required as well as correction of any electrolyte or clotting deficiencies. Ideally, upper intestinal endoscopy needs be performed to rule out a bleeding gastric or duodenal ulcer since these patients are often on steroids at the time of presentation. If the patient remains hemodynamically unstable even after resuscitation, then operation is indicated; medical therapy is too slow to reverse the mucosal inflammation responsible for the bleeding. If the patient responds to resuscitation, then a trial of intravenous steroids may be instituted. Persistent bleeding requiring transfusion after 48–72 h of therapy is an indication for surgery. Perforation outside the setting of toxic megacolon rarely occurs. A patient presenting with a perforation without megacolon should raise concern that the actual diagnosis is Crohn’s disease, or that there is another cause for the perforation, such as a gastric or duodenal ulcer. Whatever the cause, there is no role for conservative therapy, and the patient should immediately undergo exploration.

Regardless of the indication for an emergency CUC surgery, the operation of choice is a subtotal colectomy with an end ileostomy. A pelvic dissection is avoided so the planes in the pelvis are not disturbed, making future surgery easier. The rectum should be retained even in patients who are not candidate for a future restorative procedure. Performing the rectal dissection during emergency surgery increases the complexity of the case, lengthens the operation, and increases the risk of bleeding. After the patient recovers and their health status improves the retained rectum can be addressed at a future elective definitive procedure.

The most common indication for elective surgery is intractability, despite medical therapy. Other indications include: colonic dysplasia, a dysplasia associated lesion or mass (DALM) , malignancy, or side-effects of the medications. In children, stunting of normal growth is also an indication for surgery. Intractability is a clinical definition. In the chronic disease setting, it refers the inability to discontinue oral steroids completely or the development of severe drug-related side effects.

The presence of colonic dysplasia is an important consideration in CUC patients especially in those with long-standing disease. CUC patients are at high risk of developing colorectal cancer. The cancer risk increases with both duration and extent of the disease. The lifelong risk of colorectal cancer is estimated to be anywhere from 2 % at 20 years after onset of CUC to 43 % at 35 years [41]. The presence of colonic dysplasia on endoscopic biopsies is evidence of epithelial instability. This is considered a premalignant state. Initially reported by Taylor and colleagues and expanded upon recently by Gorfine et al. colon specimens with dysplasia of any grade were 36 times more likely to harbor a cancer [42, 43]. In the Mayo Clinic experience, 18 CUC patients with low-grade dysplasia were observed with serial colonoscopies for a median of 32 months [42]. Nine of the 18 patients developed advanced neoplastic lesions including a cancer. Ullman and colleagues reported a similar high rate of malignancy in the setting of low grade dysplasia without any associated masses [44]. While some recommend increased endoscopic surveillance for low grade dysplasia others consider its presence an indication for surgery. The presence of a polyp not associated with any surrounding dysplasia usually can be managed with endoscopic removal without any increased risk for malignancy [45]. While increased frequency of endoscopic surveillance for low grade dysplasia may be warranted, evidence of recurrent low grade dysplasia or any evidence of moderate or high grade dysplasia is an indication for surgery.

The two primary definitive operations for CUC are total proctocolectomy with end ileostomy (TPC) or total proctocolectomy with ileal-pouch anal anastomosis (IPAA). The TPC can be performed in a single stage with a relatively low morbidity. However, the patient is left with a permanent ileostomy. The IPAA is often a multiple stage procedure and is associated with a higher complication rate. The advantage of IPAA is that it avoids the need for a permanent ileostomy and preserves the normal route of defecation albeit with a higher number of daily bowel movements.

IPAA is the procedure of choice in appropriately selected patients who wish to avoid a permanent ostomy. Due to technical considerations, obese patients and extremely tall patients may not be good IPAA candidates. Our experience is that IPAA needs to be abandoned in less than 4 % of patients intraoperatively due to technical or anatomic problems [46]. Furthermore, advanced age which was once considered a contraindication to IPAA is now considered a relative contra-indication.

Whether IPAA is performed though a traditional open or a minimally invasive approach, the operation involves four steps: (1) removal of the intra-abdominal colon, (2) dissection and removal of the rectum sparing the pelvic nerves and the anal sphincter mechanism, (3) construction of an ileal reservoir, (4) anastomosis of the ileal reservoir to the anal canal. We have previously described our IPAA technique. [47] At our institution, nearly all patients also receive a diverting loop ileostomy at the time of IPAA.

Construction of the ileal pouch requires that the small bowel mesentery be completely mobilized from the retroperitoneum up to the inferior border of the pancreas to ensure there is adequate length mesenteric length to reach the anal canal. To achieve adequate length, it may be necessary to divide either the ileocolic vessel or one of the branches of the superior mesenteric artery. Once the mesentery has been mobilized, the pouch is fashioned. A J-shaped reservoir is constructed from the terminal 30–35 cm of the ileum. The pouch is constructed by folding the terminal ileum into a J-shape. The common wall between the two limbs is divided by repeated firings of a linear cutting stapling device from the apex of the pouch. The pouch is anastomosed to the anal canal by using a circular stapler or alternatively by a hand-sewn pouch to anal canal anastomosis. As mentioned previously, our practice is to construct a proximal diverting loop ileostomy at the time of the pouch operation. However, as will be discussed later, in some select patients omitting the ileostomy can be considered. Eight to twelve weeks after the operation if there is no evidence of a leak from the pouch on a contrast study performed through the anus, the ileostomy is reversed.

While the majority of the literature regarding complications, functional outcomes, and long-term durability of IPAA is based upon IPAAs constructed via an open laparotomy, the last decade has seen a significant transition to IPAA being performed using advanced minimally invasive techniques. In a case matched series reported by Dunker and colleagues, laparoscopic-assisted IPAA as compared to open IPAA resulted in similar functional results and quality of life outcome measurements [48]. Our initial experience at the Mayo Clinic with laparoscopic IPAA was similarly positive [49]. In a report using a large national surgical database, the percentage of minimally invasive IPAA increased from 18.5 % in 2005 to 41.3 % in 2008 and in some individual institution reports as high as 80 % minimally invasive IPAAs [50]. Early reports on minimally invasive IPAA focused on the improved cosmesis, reduced postoperative pain, and decreased length of stay; more recent national studies have also demonstrated significant reductions in major postoperative morbidities [50, 51].

A number of surgeons and institutions have reported their IPAA experience. Across these many studies, the functional results are quite similar [52–54]. The majority of patients report good to excellent function with their IPAA. In the Mayo Clinic IPAA experience, the average number of daytime bowel movements after ileostomy closure was six and one at night. [52] Incontinence was an unusual occurrence during the day with 79 % of patients reporting complete continence, 19 % occasional incontinence and 2 % frequent incontinence episodes. Nocturnal incontinence was more common with 59 % reporting no incontinence episodes and 49 % occasional incontinence episodes. Although long-term follow-up for patients who had minimally invasive IPAA is limited, the 1 and 5 year functional outcomes for 119 patients from the Cleveland Clinic is similar to matched patients who had open IPAA [55].

Thirty years after the introduction of IPAA, the long-term durability of the pouch has been assessed. Hahnloser et al., reported on the functional outcomes of IPAA patients who have had their pouch in place for up to 20 years [56]. Pouch failure is rare even in those who suffer postoperative complications. The overall pouch success rate is 92 % (Fig. 50.5). Most commonly, permanent diversion or pouch excision were performed for poor pouch function. The strongest association with pouch failure was with postoperative pelvic sepsis. Other reasons for pouch failure were chronic pouchitis and subsequent development of Crohn’s disease.

IPAA is associated with a number of early and late complications . Small bowel obstruction and pelvic abscesses are the most common early complications. Late complications include anastomotic stricture, pouch fistulas, pouchitis, and cuffitis. Delayed presentation (>1 year after reversal of the ileostomy) of a pelvic abscess or fistula raises the concern that the underlying diagnosis is not CUC but rather CD.

Numerous reports of IPAA experience have had similar postoperative complication rates [57–59]. The overall postoperative morbidity rate ranges between 25 % and 30 %. The most worrisome postoperative complication is a pouch leak and associated pelvic sepsis which occurs with a rate between 5–24 %. As discussed later, this rate of pelvis leak does not seem to be influenced by proximal diversion with an ileostomy at the time of pouch construction. Patients with a pelvic phlegmon CT scan respond to broad-spectrum antibiotics. If there is drainable fluid collection, percutaneous CT-guided drainage is the preferred approach. Rarely laparotomy with abdominal washout and drainage is required.

Small bowel obstruction is both a short and long term complication after IPAA. In the Mayo Clinic experience, perioperative small bowel obstruction was 15 % with nearly a quarter requiring operation [60]. The most common site of obstruction was in the pelvis. MacLean reviewed the literature and reported an average incidence of bowel obstruction as 18 % at 1 year, 27 % at 5 years, and 31 % at 10 years [61]. The impact of minimally invasive IPAA on the incidence of bowel obstruction has not been reported. However, in one small series where pelvic adhesions were assessed at the time of ileostomy closure there were significantly reduced [62]. Pouch-anal anastomotic stricture is another common complication after IPAA [60]. There is no correlation between stricture formation and anastomotic technique, hand-sewn versus stapled. Fortunately, these strictures are easily dilated either in the operating room or by the patient using soft dilators.

The most common long-term IPAA complication is pouchitis [63]. The reported incidence of pouchitis is heavily influenced by the diagnostic criteria used. Patients report symptoms similar to colitis including fever, anemia, and diarrhea [64]. An increased number of pouch leakage episodes or fecal incontinence is a common complaint. There are few reliable preoperative or postoperative risk factors that have been associated with development of pouchitis. Fortunately, chronic pouchitis is infrequent and often responds to therapy.

The most common treatment is a 2 week course of oral antibiotics either metronidazole or ciprofloxacin [65]. Patients who suffer frequent recurrent episodes or develop chronic pouchitis may require prolonged antibiotic therapy or even immunomodulator therapy. Rarely, chronic pouchitis requires permanent diversion or pouch excision. In long-term follow-up of IPAA patients, nearly 50 % of patients reported at least one episode at 10 years but it rose to 78 % after 20 years [56] (Fig. 50.6). In this cohort, chronic pouchitis developed in less than 5 % of patients and only 2 % required diversion or pouch removal.