Conclusions

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M. C. Turner and J. Migaly

Evaluation for stricture with gastrografn enema is prudent prior to take down of diverting ileostomy.

(e) Fistula Formation: Pouch-vaginal stu-

las occur at a rate of 3–15%. Increased vaginal discharge is the typical presenting symptom, or stulae may be found during evaluation for ileostomy take down. Stepwise management can be with seton placement, endoanal ileal advancement ap, gracilis muscle interposition, or require pouch revision, depending on severity and previous failed closure attempts. Proximal diversion alone is typically insufcient for closure of the stula tract. Evaluation for stula with gastrografn enema is prudent prior to diverting ileostomy take down.

(f) Sexual Complications: Women with UC

of childbearing age who undergo proctocolectomy have decreased fertility compared to women who elect for medical management. This is postulated to be the result of pelvic adhesive disease obstructing the fallopian tubes. Mitigation of this risk includes medical management of UC, TAC with ileorectal anastomosis, or TAC with end ileostomy, with plan for subsequent IPAA when childbearing is complete. There is no impact on male fertility. Men with UC report improved sexual quality of life following IPAA. While data regarding rates of erectile dysfunction and retrograde ejaculation are mixed, overall sexual satisfaction compared to preoperative function is preserved to improved. Women report increase in dyspareunia, but overall unchanged coital or orgasm frequency following IPAA.

(g) Pouchitis: Pouchitis, presenting as

increased stool frequency, bleeding, abdominal pain, incontinence and fever, approaches a 50% occurrence rate 10 years postoperatively from IPAA. It occurs as a result of bacterial overgrowth, and is treated with aerobic and anaerobic antibiotic coverage. Refractory symptoms may be allevi-

ated with topical steroids or aminosalicylates. Furthermore, chronic or cyclic antibiotics may also be required. Chronic pouchitis requires reevaluation for Crohn’s disease. Proximal diversion typically does not resolve symptoms, and excision and creation of a new pouch will likely result in recurrent symptoms of the new pouch. Pouch excision and end-ileostomy are rarely required. Finally, it is important to ensure that pouchitis is not a manifestation of “cuftis” where there is residual tissue between the dentate line and pouch anastomosis that is chronically inamed. While this may respond to topical therapy, mucosal stripping may be required.

(h) Re-do IPAA: Pouch failure requiring

excision and end ileostomy versus re-do occurs between 3–15% of patients, with sepsis being the driving cause. Re-do IPAAs can be performed transanally or transabdominally and are associated with similar quality of life relative to primary IPAA. Re-do surgery is associated with increased risk to nerve, ureter, and vessel injury.

X. Functional Outcomes: Following IPAA and

diversion take down, patients typically experience six bowel movements in a 24h period. The long-term patient satisfaction with symptom improvement is remarkable, with high quality of life scores that persist over time.

Y. Stoma Complications: While most stomas

are tolerated well, several complications can occur, and up to 30% of patients with end ileostomy will require re-intervention on their ostomy. These complications include retraction, stenosis, prolapse, and parastomal hernia. Skin excoriation is common and can be mitigated with appropriately sized appliances.

Z. Continent Ileostomy: The continent ileos-

tomy, Kock pouch, is largely of historical signicance secondary to its high rate of complications and the improved techniques for sphincter preserving operations. Candidates are highly motivated patients

53 Colonic Conditions: Ulcerative Colitis

415

who are unable to undergo IPAA/ IRA. Following total abdominal colectomy an ileal pouch is created with 45cm of small bowel folded into an ‘S’ formation. The distal outow is congured into intussusception which serves as an in situ valve. As stool builds in the pouch, the pressure occludes the valve preventing evacuation. The pouch is initially cannulated to gravity drainage, and is matured overtime with increasing volumes of distention for extended periods of catheter clamp time. Ultimately, the goal is to cannulate the pouch for evacuation several times per day. Postoperative complications are common: in addition to complications seen with loop and end ileostomy, herniation, stricture, prolapse, and excoriation, the Kock pouch can be complicated by stula formation, subluxation of the nipple valve, and perforation with cannulation. These complications carry signicant morbidity and risk of sepsis, and ultimately many are converted to traditional end ileostomy.

AA. Ileorectal Anastomosis: Ileorectal anasto-

mosis (IRA), while having the benet of preserving the reservoir function of the rectum, is infrequently performed in the setting of UC.This is secondary to continued inammatory symptoms of the rectum, and the annual rectal mucosal surveillance required. For elderly patients, those who have a limited life span, and who have minimal symptoms at the rectum, without evidence of dysplastic changes, an IRA may be appropriate. If refractory symptoms occur, or neoplasia is identied in the rectum, these patients are candidates for IPAA or completion proctectomy with end ileostomy. Strict surveillance for dysplasia of the rectal tissue is required. Additionally, chronic medical management strategies must be employed to mitigate proctitis symptoms.

BB. Indeterminate Colitis: Approximately 10%

of patients affected with colitis will not t diagnostic criteria for either Crohn’s disease or ulcerative colitis. Previously, this entity was referred to indeterminate colitis (IC) which is now a designation reserved following conrmation with specimen

pathology, and the term inammatory bowel disease unspecied (IBDU) is the preferred preoperative distinction. In this scenario, the most appropriate surgical management can be difcult to discern, especially in the setting of fulminant colitis. Recent studies demonstrate equivalent outcomes between patients with UC, IC and IBDU following IPAA.

CC. Postoperative Care: Historically, postopera-

tive management for colonic resection included nil per os until return of bowel function, and serial monitoring of postoperative laboratories. In the modern era, enhanced recovery protocols (ERAS) are found to be safe, effective, and practical. At our institution, for appropriately selected patients, we use the following ERAS protocol. Preoperatively, patients undergo a mechanical and non-absorbable enteral antibiotic preparation. An epidural pain catheter is placed preoperatively, and managed by our anesthesia colleagues both intra- and postoperatively. Intraoperative goal directed uid resuscitation is used following hemodynamic and urine output metrics. A regular diet is resumed on postoperative day zero. Postoperative day one, the urinary catheter is removed, and a stoma nurse works with the patient on ostomy care. The expectation is that the patient will be out of bed, and physical therapy consulted for those who require it. Postoperative day 2 brings transition to oral pain medication and removal of epidural catheter if a diet has been tolerated. Once the patient is comfortable with their pain management, understands how to manage their ostomy, is tolerating a diet, and able to complete their activities of daily living, they are discharged from the hospital.

Conclusions

While UC is managed with improving medical therapies all patients require endoscopic oncologic surveillance, and nearly half will require operative intervention for sequela of the disease. Improved resection techniques that are

416

M. C. Turner and J. Migaly

sphincter preserving and minimally invasive in nature have led to improved quality of life for patients with UC while mitigating, eliminating, or treating oncologic risk.

Colonic Conditions: Indeterminate Colitis

JonD.Vogel andMarianaBerho

See Algorithm inFig. 54.1

A. Similar to ulcerative colitis or Crohn’s coli-

tis, patients with IC or IBDU typically have symptoms of diarrhea, blood in the stool, abdominal pain, fecal urgency, and tenesmus. With each of these colitides, severe or fulminant presentations may occur.

B. A detailed medical and family history is per-

formed to search for clues that implicate Crohn’s disease as the underlying pathology. Prior small bowel obstruction, obstructive symptoms, or perianal disease are suggestive of Crohn’s disease. Symptoms of extraintestinal disease and family history of IBD are also assessed. Colitis disease severity may be estimated by stool frequency, blood in the stool, weight loss, anemia, or signs of “toxicity” such as fever, tachycardia, or hypotension.

C. The physical examination is used to assess

colitis severity (e.g. abdominal distension or tenderness), and to look for clues of Crohn’s disease such as palpable abdominal mass, edematous anal skin tags, perianal abscess or

J. D. Vogel (*) University ofColorado, Aurora, CO, USA e-mail: jon.vogel@ucdenver.edu

M. Berho Department ofPathology, Cleveland Clinic Florida, Weston, FL, USA

stula, anal stenosis, or scars from prior anorectal surgery.

D. A complete blood count and serum albumin

measurement are helpful to determine the severity and chronicity of IBD-UC/IC. Stool testing is performed to exclude Clostridium difcile infection or other infectious etiologies of colitis. Serological biomarkers, such as p-ANCA and ASCA, and genetic markers, such as NOD2/CARD 15, are generally not useful to establish the specic type of IBD.

E. CT or MR enterography are selectively used

to exclude the presence of small bowel disease or segmental colorectal disease that are suggestive of CD. In acutely ill patients, plain abdominal radiographs are used to exclude megacolon or pneumoperitoneum.

F. Colonoscopy is used to evaluate the severity

and extent of colitis. Rectal sparing, segmental inammation, deep or linear ulcers, ileocecal valve or terminal ileal ulceration, erythema, edema, or strictures are endoscopic ndings indicative of Crohn’s disease. Alternatively, gross inammation that starts in the rectum and extends proximally in a continuous pattern and then transitions to normal appearing mucosa is characteristic of ulcerative colitis. Backwash ileitis, or continuous inammation of the terminal ileum without focal ulceration or strictures, may occur with ulcerative colitis. A discontinuous “cecal patch” of inammation, that is limited

https://doi.org/10.1007/978-3-319-65942-8_54

417

418

J. D. Vogel and M. Berho

Presentation

A. Bloody diarrhea

Abdominal pain

Fig. 54.1 Algorithm for indeterminate colitis

Initial

Evaluation

B. History

C. Physical examination

Evaluation

D. Lab testing

E. Imaging

F. Endoscopy

G. Gross pathology

H. Histopathology

to the mucosa immediately surrounding the appendiceal orice, may also be seen in UC.It is important to also note that medical treatment of UC may result in segmental colorectal mucosal changes and the false impression that CD is the underlying disease. Mucosal biopsies are obtained for histologic assessment of inammation, cytomegalovirus (CMV) infection, dysplasia, or cancer.

G. Inammatory bowel disease—unclassied

(IBD-U) is the term to describe biopsy specimens with inammatory bowel disease in which a denitive diagnosis of Crohn’s disease or ulcerative colitis cannot be made with certainty. A diagnosis of indeterminate colitis (IC) is based on the pathological analysis of a colectomy specimen. Macroscopic pathological features of Crohn’s disease include mesenteric fat wrapping of the bowel wall, “skip areas” of inamed and normalappearing mucosa, segmental rather than continuous mucosal inammation, small bowel involvement beyond what is typical for “backwash ileitis” (continuous inammation of the terminal ileal mucosa without stricture or deep ulceration), stula, stricture, cobblestone appearance of the intestinal mucosa, transmural inammation, and perianal disease. Grossly, ulcerative colitis is characterized by continuous mucosal inam-

Acute

Treatment

I. Medical treatment

J. Urgent surgery

K. Elective surgery

Outcome

L. Short-term

outcomes

M. Long-term

outcomes

N. Functional

outcomes

mation that begins in the rectum and “spreads like a sheet” into the more proximal colon. Mesenteric fat wrapping or segmental colonic mucosal inammation, in the absence of other distinct features Crohn’s disease or ulcerative colitis, raise the specter of indeterminate colitis (Table54.1, Fig.54.2).

H. Histological features of Crohn’s disease

include non-caseating granulomas (identied in 20% of biopsy specimens and 50% colectomy specimens), and aphthous ulcers. These ndings are inconsistent with a diagnosis of ulcerative colitis or indeterminate colitis. Nerve cell hyperplasia and vasculitis may be seen in Crohn’s disease or ulcerative colitis but are more common in Crohn’s disease. Transmural inammation occurs in Crohn’s disease but may also be present with severe colitis due to ulcerative or indeterminate colitis (Table54.2, Fig.54.3).

I. The medical treatment of UC, CD, and

IBD-U/IC are similar and typically include the 5-ASA drugs, glucocorticoids, azathioprine, and biologic therapies targeted at TNF-alpha or intestinal mucosal integrin. Supportive therapy with intravenous uids, antibiotic treatment of clostridium difcile as needed, and the use of antibiotics to treat suspected or proven infectious processes that complicate IBD colitis may also be required.

54 Colonic Conditions: Indeterminate Colitis

Table 54.1 Macroscopic features useful to distinguish ulcerative colitis from Crohn’s disease

Ulcerative colitis

Fat wrapping No Yes Occasionally in areas

Segmental involvement/rectal sparing

Small intestinal involvement

Fistulas No Ye s No Cobblestone

appearance Predominantly distal

disease Anal/perianal disease No Ye s

A diagnosis of backwash ileitis should be limited to those cases with a mild inammation limited to the distal 5cm of

the terminal ileum, the presence of deep ulceration should raise the possibility of Crohn’s disease

The presence of anal/perianal disease should strongly raise the possibility of Crohn’s disease even when the rest of the

pathological changes are equivocal

No Ye s Common – Medical treatment in UC may lead to

No Ye s No – UC may display “backwash ileitis”

No Ye s No

Yes No No – UC may show discontinuous

Crohn’s disease Indeterminate colitis Caveats

of deep ulceration

– Severe cases of UC with deep ulcers may display fat wrapping

uneven healing and areas of “pseudosparing”

– Up to 20% of cases of CD present with colonic involvement only

involvement of the cecum (cecal patch)

419

simplies subsequent proctectomy via preservation natural tissue planes. Rectal resection is usually avoided in the acute setting to minimize potential complications of surgery and to allow pathology review of the resected colon to help solidify the diagnosis.

K. Proctocolectomy with ileal pouch anal anas-

tomosis (IPAA) may be performed in carefully selected patients with IBD colitis in whom the diagnosis of CD, particularly small bowel or perianal CD, has been excluded.

Fig. 54.2 Indeterminate colitis

Open or minimally invasive surgical techniques may be used. The decision to perform

Venous thromboembolism prophylaxis is also generally recommended in hospitalized patients with IBD.

J. Urgent subtotal or total abdominal colectomy

with ileostomy is generally indicated for IBD-U complicated by severe colitis refractory to medical therapy, megacolon, colonic perforation, or hemorrhage. Open or minimally invasive surgical techniques may be used. Preservation of the inferior mesenteric artery pedicle and the distal sigmoid colon facilitates subcutaneous implantation of the stapled sigmoid stump or creation of a sigmoid mucous stula when necessary (e.g. severely edematous distal colon) and also

a one- or multi-staged proctocolectomy should be an individualized decision with consideration of the risks and benets associated with each approach. In general, staged IPAA procedures, with initial subtotal colectomy and end ileostomy, are performed in patients who are malnourished, on highdoses of steroids, or have ndings at surgery, such as extraordinary fragility of the tissues and bleeding during the colectomy. Subtotal or total abdominal colectomy with end ileostomy is also recommended when the operative ndings are suspicious for Crohn’s disease. In this situation, pathology review of the colectomy specimen will often help guide

420

J. D. Vogel and M. Berho

Table 54.2 Microscopic features useful to distinguish ulcerative colitis from Crohn’s disease

Ulcerative colitis Crohn’s disease

Granulomas No Yes

Transmural inammation

Aphthous ulcers

Nerve cell hyperplasia

Vasculitis + +++ No

No Yes Common Severe UC may display transmural

No Yes No

+ +++ No

Only 20% of biopsies and approximately 50 to 60% of surgical specimens

Indeterminate colitis Caveats

No Ruptured crypts secondary to

inammation may show granulomatous reaction in ulcerative colitis

inammation in areas of deep ulcers

patients. In Dayton’s series, there were no differences in early postoperative IPAA complications

M. Long-term outcomes of IPAA for IC com-

pared to UC vary and include an eventual change in the diagnosis to Crohn’s disease in 1–15% of patients and pouch failure (requiring diverting ileostomy or pouch excision) in 2–23% of patients. With 10-year follow-up, Yu reported signicantly more long-term complications in IC patients compared to UC including pelvic sepsis, pouch stula, and pouch failure (23% vs. 9%). More recently,

Fig. 54.3 Deep ulceration

Murrell and colleagues reported similar

IPAA outcomes for their IC and UC patients future surgical decisions. Proctolectomy with permanent ileostomy may be appropriate for elderly patients, or those who have impaired anal sphincter function, or for patients who have other reasons for which an ileo-anal reservoir is a poor choice. Total abdominal colectomy with ileorectal anastomosis may also be considered in select patients with a grossly normal and dysplasia-free rectum who accept the risk of proctitis, the need for subsequent medical or surgical therapy for the rectum, and who are compliant with rectal mucosa surveillance.

L. In the series reported by Yu, Brown, and

Delaney, early postoperative complications of IPPA surgery, including pelvic sepsis and stula formation, but not anastomotic leak, occurred more often in IC compared to UC

with conversion to a diagnosis of CD in 14

(14%) of 98 patients with IC, and 29 (11%)

of 236 patients with UC (Table 54.3).

Pouchitis occurs with similar frequency

among patients with UC and IC (Yu, Dayton,

Murrell). In patients with an IPAA who con-

vert from a diagnosis of IC to CD, pelvic

perineal sepsis is treated with antibiotics,

incision and drainage, and liberal use of

draining setons. Mucosal inammation or

stulizing disease is initially treated with the

full spectrum of Crohn’s disease medical

therapy. In patients in whom the above inter-

ventions fail, the use of a diverting ileostomy

or excision of the ileal pouch with permanent

ileostomy must be considered.

N. In a study from the Cleveland Clinic

(Delaney), in which 115 IC and 1399 UC

54 Colonic Conditions: Indeterminate Colitis

Table 54.3 Published series on indeterminate colitis

Author Year IC patients (N) IC→CD (%) Marcello 1997 53 13 12 499 3 2 Yu 2000 82 15 23 1437 2 9 Delaney 2002 115 6 3 1399 1.3 3.5 Dayton 2002 79 1 2.5 565 0.7 1.2 Brown 2005 21 0 10 1135 NA 6 Murrell 2009 97 14 NA 237 11 NA

Table modied from C.Delaney etal. 2002

IC patients indeterminate colitis patients, IC→ CD IC patients subsequently diagnosed with Crohn’s Disease, IC w/ IPAA failure patients with IC and IPAA with subsequent IPAA excision or permanent ileostomy, UC patients ulcerative

colitis patients, UC→CD UC patients subsequently diagnosed with Crohn’s disease, UC w/IPAA failure patients with UC and IPAA with subsequent IPAA excision or permanent ileostomy

IC w/IPAA failure (%)

UC patients (N) UC→CD (%)

UC w/IPAA failure (%)

421

patients who underwent IPAA, the functional outcomes were largely similar for the two diagnoses. Daytime bowel movements numbered 4–8 and nighttime were 0–2. The majority (70–74%) reported rare or no fecal incontinence and nighttime stool seepage occurred in about one-third of IC and UC patients. Similar functional results for IPAA in UC and IC have also been reported by surgeons from the University of Toronto (Brown), Louisville (Rudolph), Utah (Dayton) and the Mayo Clinic (Yu).

Colonic Conditions: Toxic Colitis

CarmenFong andBenjaminAbbadessa

Refer toAlgorithm in Fig.55.1

A. History and Physical

1. Clostridium difcile Infection (CDI, Pseudomembranous colitis) is the leading cause of infectious diarrhea in the world, and has become increasingly more prevalent and severe. Two prevalent theories behind C. difcile-associated toxic colitis are that C. difcile exotoxin causes either inammatory inltrates in the myenteric plexus which lead to smooth muscle damage, or inammatory mediators such as nitric oxide cause smooth muscle relaxation, both resulting in dilation and distension of the large bowel.

CDI may present with abdominal pain, bloating, diarrhea (occasionally bloody), and subjective fever and chills. However, one study quotes up to 40% of patients may present with the obstipation form of CDI and may not have diarrhea. On physical exam, the patient may have marked abdominal tenderness and distension. In the most severe cases, the patient may also be febrile, dehydrated and septic with profound leukocytosis. One report found that 96% of cases of the disease are associated

C. Fong · B. Abbadessa (*) Department ofSurgery, Mount Sinai Beth Israel, New York, NY, USA e-mail: babbadessa@ucsd.edu

with use of antibiotics such as ciprooxacin or clindamycin within the last 14days. Symptoms usually appear 2–3 days after initiation of antibiotic therapy. Other risk factors include IBD, recent travel, HIV seropositivity, malnutrition, advanced age and acid suppression with a proton pump inhibitor.

2. Ulcerative Colitis (UC) is an autoimmune disease characterized by chronic mucosalbased inammation of the colon. The disease begins in the rectum and may extend proximally in a continuous manner to involve all or part of the colon. The small bowel and terminal ileum are not involved but may show patterns of mucosal inammation due to local inammatory factors associated with backwash ileitis. Patients typically present with bloody diarrheal stools. Abdominal pain is most common with moderate and severe UC.On physical exam, patients with severe UC may present with fever, leukocytosis, abdominal tenderness and distension. Most patients’ disease course will involve remissions and exacerbations.

3. Crohn’s Disease (CD) is a trans-mural, autoimmune process that can affect the entire length of the intestinal tract in a discontinuous manner. The most common involved locations include the terminal ileum (30%), ano-rectum and colon (20%) and combined small bowel and colon

https://doi.org/10.1007/978-3-319-65942-8_55

423

424

Fig. 55.1 Algorithm for treatment of toxic colitis. DLI diverting loop ileostomy, ICL intraoperative colonic lavage

C. Fong and B. Abbadessa

disease (50%). CD has an incidence of 2–4 patients per 100,000in the Caucasian population. It is more common in Ashkenazi Jews, and 20 times more prevalent in patients with a family history. Patients are more likely to be female and tend to have a bimodal distribution of age at presentation. The typical presentation includes abdominal pain that is vague, crampy and intermittent. There may be hematochezia. Severe colitis may present as, or progress to, major lower GI bleeding, perforation, obstruction, or fulminant colitis. Extra- intestinal manifestations are more frequent in Crohn’s disease than Ulcerative colitis and include ocular, bone, hepatobiliary and cutaneous manifestations. On physical exam, the patient may look toxic, with fever, leukocytosis, abdominal tenderness and distention.

B. Laboratory Testing

A complete blood count as well as a complete metabolic panel should be obtained, as well as inammatory markers such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). An elevated ESR and CRP can signify disease.

Stool studies including tests for C. dif- cile, cytomegalovirus, ova and parasites, and stool leukocytes are important tools in diag-

nosis, and help differentiate between infectious and non-infectious causes of toxic colitis. Within stool testing for C. difcile, there are various modalities. A positive stool culture indicates the presence of C. difcile bacterium, whereas a positive stool toxin indicates clinically signicant disease. Cell cytotoxicity assays, which test for toxins A&B, have sensitivities between 60% and 100%. The US Department of Health recommends a 2-stage test approach involving enzyme immunoassay (EIA) glutamate dehydrogenase testing followed by cell cytotoxicity or cultures. This approach has >90% sensitivity and specicity. A relatively new modality, based on polymerase chain reaction (PCR) testing of toxin genes, called nucleic acid amplication tests (NAAT) is gaining popularity as a faster and more reliable method of diagnosis which may replace the two- step method, as it has a 94–99% sensitivity rate.

Lastly, PCR for CMV has a sensitivity and specicity in the blood of 45% and 87%, a mucosal biopsy sensitivity of 90%, and sensitivity in stool of 67%, but nonetheless remains an important differential diagnosis.

C. Imaging

A CT scan is a useful tool in evaluation of abdominal pain (Fig. 55.2). While

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Conclusions

Suggested Reading

Colonic Conditions: Indeterminate Colitis

Suggested Reading

Colonic Conditions: Toxic Colitis