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Acs0511 Crohn Disease 2004
1. © 2004 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 5 GASTROINTESTINAL TRACT AND ABDOMEN 11 CROHN DISEASE — 1 11 CROHN DISEASE Susan Galandiuk, M.D., F.A.C.S., F.A.S.C.R.S. The role of surgery in the management of Crohn disease has the known risk of disease recurrence after surgical treatment of undergone a dramatic evolution over the past 50 years. Crohn disease and the signiﬁcant associated operative morbidity. In Currently, surgical treatment of Crohn disease is seldom per- one single-center study, the reoperation rate for Crohn disease was formed in the emergency setting; it is nearly always performed 34% at 10 years.3 The agents used to treat Crohn disease can be di- after failed medical therapy.The decision to proceed with opera- vided into several broad groups: probiotics, antibiotics, anti-inﬂam- tive management is based on careful patient evaluation, with full matory drugs, immunosuppressive drugs, and biologic agents.These awareness of the potential complications and ramiﬁcations of can be used alone or in combination to treat disease, as well as to treatment. In particular, attention must be paid to the risk of maintain remission [see Table 1]. recurrent disease, the possible surgical sequelae, and the side Few good studies have been done on the cost-effectiveness of effects of medical therapy. medical or surgical therapy4,5 versus that of timely surgery fol- lowed by maintenance medical therapy.There is clearly a need for such studies.The use of potent and expensive immunomodulator Classiﬁcation therapy (e.g., maintenance inﬂiximab) for simple ileocolic disease There are many systems for classifying Crohn disease. One of is questionable, especially in the light of studies indicating that the simplest is the classiﬁcation developed by Farmer and associ- such treatment is not at all innocuous.6,7 ates,1 which categorizes the disease on the basis of disease location CHANGING CONCEPTS IN SURGERY FOR CROHN DISEASE alone (ileocolic, purely colonic, small bowel, and perianal). A more elaborate system is the Vienna classiﬁcation, which categorizes the Although ﬁrst described in the beginning of the 19th century, disease on the basis not only of location but also of age of onset Crohn disease was not recognized as a discrete clinical entity until and disease behavior.2 In this system, there are four categories for the ﬁrst part of the 20th century.8 At one point, it was treated sur- disease location: terminal ileum (L1), colon (L2), terminal ileum gically in much the same way as cancer, with frozen-section mar- and colon (L3), and any location proximal to the terminal ileum gins obtained at the time of resection.This approach did not yield (L4). There are two categories for age of onset: less than 40 years any substantial reduction in the recurrence rate.9 In fact, overzeal- of age (A1) and 40 years of age or older (A2). Finally, there are ous resections often resulted in Crohn patients’ requiring lifelong three categories for disease behavior: nonstricturing and nonpen- parenteral nutritional support.10 Accordingly, conservative surgery etrating (B1), stricturing (B2), and penetrating (B3). is now the rule: only gross macroscopic disease is resected into pal- Given that there are as many types and combinations of Crohn pably normal margins (in particular, a palpably normal mesenteric disease as there are patients with this condition, the most sensible border of the bowel). approach is probably to use some combination of these two clas- siﬁcation schemes. Careful evaluation of the speciﬁcs of each case will yield the best treatment results; however, general classiﬁcation General Indications for Surgical Treatment of the disease can help guide therapy. Broadly speaking, Crohn SIDE EFFECTS OF MEDICAL THERAPY disease of the small bowel has the highest recurrence rate. Because of the important function of the small bowel in digestion, surgeons Signiﬁcant side effects of medical therapy include those associ- tend to emphasize conserving small bowel length during operative ated with failure to wean from prednisone (e.g., cataract forma- treatment of Crohn disease. Currently, however, there is an in- creasing focus on colon conservation with the aims of maintaining water absorption in patients and delaying (or perhaps eliminating) Table 1 Medical Treatment of Crohn Disease the need for a stoma. Category Example Application Form Expense Roles of Medical Therapy and Surgical Therapy Probiotics Food, capsules, Lactobacillus $–$$ In planning treatment of Crohn disease, it is important not to pills, powders make the use of medical therapy or surgical therapy an either-or Metronidazole, Antibiotics p.o., I.V. $–$$ issue. Just as one tool cannot be expected to ﬁll every household ciprofloxacin need, operative management cannot be expected to solve every p.o. $ problem related to Crohn disease. Overall, careful use of medical Sulfasalazine Anti-inflammatories p.o., suppositories, $$ therapy, appropriately combined with surgical therapy, provides 5-ASA products enemas the best treatment of Crohn disease. Single-minded reliance on Conventional steroids p.o., I.V. $ either therapy to the exclusion of the other often leads to inade- Budesonide p.o. $$ quate patient care. Immunosuppressives Antimetabolites p.o. $$ Generally speaking, except in the case of a free perforation, can- Methotrexate p.o. or I.M. $ cer, or dysplasia, one should not operate on a patient with Crohn Cyclosporine p.o. or I.V. $$ disease without ﬁrst attempting medical therapy.With the dramati- Infliximab $$$$ cally improved medical treatment options currently available, surgery Biologics — Investigational agents NA can be avoided in many cases.This is often a desirable result, given
© 2004 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 5 GASTROINTESTINAL TRACT AND ABDOMEN 11 CROHN DISEASE — 2 Abscess Formation Abscesses are particularly common with ileocolic Crohn dis- ease. If they cannot be controlled by means of computed tomog- raphy–guided drainage, surgical therapy may be indicated. Cancer or Dysplasia The risk of colorectal cancer is approximately three times higher in patients with Crohn disease than in the general population.11-13 Failure to Grow In children, failure to grow and develop normally is one of the main indications that medical therapy for Crohn disease has been unsuccessful.Timely surgical therapy will permit normal develop- ment. On occasion, when bone age lags signiﬁcantly behind chronological age, treatment with recombinant human growth hor- mone is required. Figure 1 Shown is an example of stenotic ileocolic Crohn dis- Special Considerations ease resulting in obstructive symptoms that were not relieved by medical therapy. PREGNANCY Persons who have Crohn disease may be less fertile than healthy tion, aseptic necrosis of the femoral head, and weight gain). Side age-matched persons. One possible explanation for this difference is effects of antimetabolite therapy include pancreatitis, neutrope- that feeling ill may result in reduced sexual desire or decreased sexu- nia, and opportunistic infections. al activity. Another is that pelvic inﬂammation caused by Crohn dis- ease or by scarring and adhesion formation resulting from surgery COMPLICATIONS OF DISEASE may impair fertility.To reduce the chances of the latter, hyaluronic acid sheets may be placed around the tubes and ovaries; alternative- Lack of Response to Medical Therapy ly, the ovaries may be tacked to the undersurface of the anterior ab- Many patients with so-called toxic colitis do not respond satis- dominal wall with absorbable sutures and thereby prevented from factorily to medical treatment. In severe cases of refractory dis- entering the pelvis. ease, if surgery is not performed, colonic perforation, peritonitis, and multiple organ failure may ensue. Such cases are much less frequent now than they once were. Obstruction In many patients with Crohn disease, the behavior of the dis- ease changes over time, from a more inﬂammatory and edema- tous process to one characterized more by ﬁbrosis and scarring. Whereas anti-inﬂammatory drugs are ideal for treating the for- mer, surgery is frequently necessary for the latter. Failure to refer for surgical treatment of obstruction is, unfortunately, a common error among gastroenterologists. Severe abdominal pain is always a warning sign of obstruction and should be taken seriously [see 5:1 Acute Abdominal Pain and 5:4 Intestinal Obstruction]. The im- portance of this point is illustrated by a case from my experience, involving a patient who had obstructing ileocolic Crohn disease with gross proximal distention of the terminal ileum [see Figure 1]. This patient lost 20 lb, was experiencing severe abdominal pain, and was treated for more than a year with 6-mercaptopurine before being referred for operative management. Ileocolic resection led to rapid resolution of the symptoms. Symptomatic Fistulas Enteroenteric ﬁstulas, by themselves, are no longer considered an absolute indication for operation in the absence of other com- plicating factors. Symptomatic ﬁstulas, such as those associated with obstruction or those associated with disabling symptoms (e.g., rectovaginal ﬁstulas or enterocutaneous ﬁstulas [see Figure 2]), may have to be treated surgically. Ileosigmoid ﬁstulas, which Figure 2 Shown is an enterocutaneous ﬁstula that persisted for effectively bypass the entire colon, may be associated with pro- more than 1 year after an ileocolic resection (arrow). The choice found and refractory diarrhea (i.e., ≥ 20 bowel movements/day) of parallel incisions by the previous surgeon made selection of a and may also have to be treated operatively. temporary stoma site much more difﬁcult.
© 2004 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 5 GASTROINTESTINAL TRACT AND ABDOMEN 11 CROHN DISEASE — 3 There is no evidence that pregnancy exacerbates Crohn disease; not hesitate to convert to an open procedure.Typically, most areas however, there are some speciﬁc concerns that apply to pregnant that feel ﬁbrotic or contain ﬁbrotic adhesions are actually areas of patients with this condition. Because patients with Crohn disease ﬁstulizing disease and should be treated as such until proved oth- often have more-liquid bowel movements, they have a particular erwise. In one study, patients with recurrent disease, those older need for a well-functioning anal sphincter. If there is any chance of than 40 years, and those with an abdominal mass were more like- an obstetrics-related injury (e.g., from a large baby in a prima- ly to require conversion to an open procedure.16 gravida or from a breech presentation), a cesarean section is advis- able to minimize the risk of sphincter trauma.The same is true in the presence of severe perianal Crohn disease. During pregnancy, Surgical Management of Crohn Disease at Speciﬁc Sites prednisone and 5-aminosalicylic acid (5-ASA) medications are ESOPHAGEAL, GASTRIC, AND DUODENAL DISEASE safe, whereas drugs such as metronidazole are not. If imaging studies are needed, magnetic resonance imaging and ultrasonog- Crohn disease of the upper alimentary tract can be difﬁcult to raphy are the modalities of choice. diagnose, largely because it is relatively uncommon. Obstructing strictures due to Crohn disease in this area are unusual; the unsus- MARKING OF STOMA SITES AND CHOICE OF INCISION pected ﬁnding of noncaseating granulomas in biopsies of erythem- When a patient with Crohn disease is expected to need an atous areas in a patient with Crohn disease in other locations is ileostomy [see 5:30 Intestinal Stomas], it is extremely important to diagnostic. mark the site preoperatively.What looks ﬂat when the patient is on Occasionally, a patient with Crohn disease of the distal esopha- the operating table may not be ﬂat when he or she is upright.The gus requires dilatations, but this is uncommon. Surgical treatment patient must be asked to sit and lean over to conﬁrm that the for Crohn disease of the upper alimentary tract is almost exclu- marked stoma site is in an area without folds, creases, or previous sively reserved for disease affecting the duodenum. Diagnosis of incisions. Stoma appliances do not adhere well to areas of previ- duodenal Crohn disease can be difﬁcult and requires a certain ous scarring, and these should be avoided whenever possible. amount of suspicion. Frequently, the diagnosis is not made until Patients with Crohn disease do not react to intra-abdominal relatively late, because diagnostic imaging tends to focus on infection in a typical fashion. It is not unusual to ﬁnd unsuspect- endoscopy and because the degree of duodenal obstruction is ed abscesses that were not revealed by preoperative CT scans and often not evident except on barium studies.The rigidity and lumi- other imaging studies. If there is even a remote chance of an nal narrowing of the second portion of the duodenum is typically unsuspected abscess (particularly in cases of obstructing ileocolic much more readily apparent on contrast studies than on endos- Crohn disease), the possibility of a temporary stoma should be copy. Duodenal Crohn disease can lead to gastric outlet obstruc- raised with the patient and the proposed stoma site marked pre- tion. In children, it can be mistaken for annular pancreas. operatively. When duodenal Crohn disease does not respond to medical A key point is the necessity of planning for the future. Many therapy, gastrojejunostomy with vagotomy is the preferred surgical patients with Crohn disease will eventually require a stoma. Oper- treatment.17,18 Failure to perform a vagotomy may result in mar- ating through a midline abdominal incision preserves all four quad- ginal ulcer formation and obstruction. Some surgeons have per- rants for possible future stoma sites (if needed). formed duodenal strictureplasty to treat duodenal Crohn disease. The results have been conﬂicting19,20; the feasibility of this opera- LAPAROSCOPY tive approach is limited by the pliability of the duodenum. Many Laparoscopic surgical techniques have gained acceptance in the patients experience prompt and full recovery of normal gastric treatment of Crohn disease. In performing a laparoscopic opera- emptying after operation, but some patients with long-standing tion for Crohn disease, it is essential to adhere to the same techni- gastric outlet obstruction continue to experience impaired empty- cal standards that apply to corresponding open procedures. ing. The latter may beneﬁt from administration of a prokinetic Careful intraoperative exploration of the abdomen is important, in agent (e.g., metoclopramide or erythromycin). that many patients have multifocal disease. Without such explo- JEJUNOILEAL DISEASE ration, patients may experience persistent postoperative symptoms as a consequence of persistent proximal pathologic states that were not addressed. As with other treatment modalities, there are some Short Bowel Syndrome circumstances in which laparoscopy is particularly useful and oth- Although Crohn disease of the small bowel is not common and ers in which it should not be used. For example, a laparoscopic accounts for a relatively small proportion of all cases, disease in approach is ideal for fecal diversion in patients with perianal this area is associated with one of the highest overall recurrence Crohn disease. rates. Resection of large portions of the small bowel can result in Ileocolic resection for Crohn disease also lends itself well to a short bowel syndrome. For this reason, before proceeding with any laparoscopic-assisted approach; compared with open resection, type of small bowel or ileocolic resection, one should measure the laparoscopic resection has been reported to result in shorter hos- length of the existing small bowel to determine the patient’s pital stays and reduced costs.14,15 The ileocolic vessels originate “bowel resource.” One naturally would more readily perform a centrally, and they only lie over the retroperitoneum. Once the lat- resection in a patient who has 400 cm of normal small bowel than eral peritoneal attachments are divided, the colon and the small in one who has only 200 cm. bowel mesentery can be exteriorized, and the mesentery can be divided and the anastomosis performed extracorporeally. Resection versus Strictureplasty Many studies have shown that even ﬁstulizing Crohn disease The major advance in the surgical treatment of Crohn disease can be safely addressed laparoscopically, depending on the skill of over the past quarter-century has been the technique of small the surgeon. A hand-assisted approach is often useful with cases of bowel strictureplasty, ﬁrst proposed by Lee and subsequently pop- dense ﬁxation, in which ﬁstulas are common and ﬁnger dissection ularized by Williams, Fazio, and others.17,18 Currently, the two may facilitate deﬁnition of the anatomy. If in doubt, one should most prevalent strictureplasty techniques are Heineke-Mikulicz
© 2004 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 5 GASTROINTESTINAL TRACT AND ABDOMEN 11 CROHN DISEASE — 4 strictureplasty [see Figure 3] and Finney strictureplasty [see Figure a 4].The former is best suited for strictures up to 5 to 7 cm long [see Figure 5], the latter for strictures up to 10 to 15 cm long.The side- to-side strictureplasty described by Michelassi21 is suitable for longer areas of stricture; however, this technique involves longer suture lines and is mainly considered for patients who already have, or are at high risk for, short bowel syndrome. The short, isolated strictures characteristic of diffuse jejunoileal Crohn disease are more frequently described in patients with long- b standing Crohn disease. It has been postulated that over time, Crohn disease progresses from an edematous condition to a more ﬁbrotic, stricturing condition.22 It is the ﬁbrotic strictures charac- teristic of the later stage of the disease that are amenable to treat- ment with strictureplasty. Patients with these short ﬁbrotic stric- tures typically have obstructive symptoms and often are unable to tolerate solid food, experiencing dramatic weight loss as a result. Although strictureplasty leaves active disease in situ, it usually leads to prompt resolution of obstructive symptoms, regaining of lost body weight, and restoration of normal nutritional status. c a b Figure 4 Finney strictureplasty. (a) This procedure is suitable for longer areas of stricture (up to 10 to 15 cm). (b) The strictured bowel is bent into the shape of an inverted U. Stay sutures are placed at the apex of the U, which is at the midpoint of the stric- ture, and at the far ends, which lie 1 to 2 cm proximal and distal to the stricture. A longitudinal enterotomy is made on the antimesenteric border of the bowel with the electrocautery. A side-to-side anastomosis is then performed, with the posterior wall done ﬁrst. (c) Shown is the completed anastomosis. A signiﬁcant concern with strictureplasty is the possibility that small bowel adenocarcinoma may develop; several cases have been c reported.23,24 I have treated a patient in whom a poorly differenti- ated jejunal adenocarcinoma developed at the site of a stricture- plasty that had been performed 10 years earlier. Accordingly, many surgeons advocate routine biopsy of the active ulcer on the mesen- teric side of the bowel at the time of strictureplasty [see Figure 6]. Another concern has to do with the number of strictureplasties that can safely be performed in a single patient in the course of a single operation. As many as 19 strictureplasties have been per- formed during one procedure without increased morbidity.25 Figure 3 Heineke-Mikulicz strictureplasty. Stay sutures are Strictureplasty can be performed with either a single-layer or a placed parallel to each other on the antimesenteric border of the double-layer anastomosis. It should not be performed in the pres- bowel over the area of the stricture. (a) The antimesenteric bor- der of the bowel is then opened with the electrocautery over the ence of an abscess, a phlegmon, or a ﬁstula; and like any other area of the stricture, and the opening is extended for approxi- anastomosis, it should not be performed proximal to an existing mately 1 to 2 cm on either side of the stricture. (b, c) Traction is obstruction that is not treated at the time of operation. placed on the stay sutures, and the original longitudinal enteroto- Areas of small bowel Crohn disease that are too long to be treat- my is closed in a horizontal fashion in one or two layers. ed with strictureplasty can be treated with segmental resection.
© 2004 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 5 GASTROINTESTINAL TRACT AND ABDOMEN 11 CROHN DISEASE — 5 erature to suggest that the postoperative recurrence rate may be low- er with a wider anastomosis.27 The anastomosis can be performed in either one or two layers. If the bowel is thicker, a handsewn anasto- mosis is preferred to a stapled one. The incidence of reoperation for recurrent disease after ileocol- ic resection is high and increases with the number of resections.28 Postoperative chemoprophylaxis with mesalamine can signiﬁcant- ly reduce the recurrence rate.29 Patients who smoke should be strongly encouraged to stop: the rate and severity of recurrence are increased in smokers.20 Special Circumstances Ileocolic Crohn disease is often associated with intra-abdominal abscesses or ﬁstulas. If an associated abscess is known to be pres- ent, CT-guided drainage should be done preoperatively so that a single-stage procedure can then be performed. If an unsuspected abscess is identiﬁed at the time of operation, the safest approach is to proceed with bowel resection, perform the posterior wall of the Figure 5 Shown is a short ﬁbrotic stricture that is ideally suited anastomosis, and exteriorize the anastomosis as a loop ileostomy. to treatment with Heineke-Mikulicz strictureplasty. This loop ileostomy can then be safely closed, often without a for- mal laparotomy, 8 weeks after operation if there are no signs of The area to be resected should be as short as possible.There is no ongoing sepsis. If the abscess or the terminal ileal loop is adherent need to obtain frozen-section margins to determine the extent of to the sigmoid colon, an ileosigmoid ﬁstula may be present. The resection; doing so leads to unnecessary loss of small bowel decision whether to resect the sigmoid colon is dictated by the length.26 The resection should extend into palpably normal areas appearance and feel of the sigmoid in the involved areas. If only a of small bowel. The easiest way of determining the area to be portion of the anterior colon wall is involved, that portion can be resected is to feel the mesenteric margin of the bowel until palpa- excised in a wedgelike fashion and the excision site closed primar- bly normal tissue is reached. Because Crohn disease is generally ily. If the entire circumference of the sigmoid colon at that point is more severe on the mesenteric side of the bowel, palpation in this indurated and woody feeling, a short segmental resection with area gives the most accurate impression of the intraluminal char- anastomosis is the best option. acter of the bowel. Because it is not uncommon for patients to have multifocal Crohn disease, the entire small bowel should al- ways be inspected at the time of operation. Operating on one area of disease while failing to treat a more proximal lesion is clearly not in the patient’s interest. Because of the high rate of recurrence in patients with isolated small bowel disease, postoperative chemoprophylaxis should be strongly considered. In these patients, I prefer to use a more potent agent, such as an antimetabolite, rather than a 5-ASA agent. ILEOCOLIC DISEASE Approximately half of those diagnosed with Crohn disease have ileocolic disease. Ileocolic resection is, in fact, the operation most frequently performed to treat Crohn disease. Currently, there is a trend toward more aggressive medical management of Crohn dis- ease; at the same time, surgeons are seeing more complicated dis- ease at the time of operation. These developments have implica- tions for management. An easy ileocolic resection is an experience that a patient generally tolerates well and recovers from very quick- ly; however, delaying operative management with years of aggres- sive medical therapy can lead to more complicated disease associ- ated with enteroenteric ﬁstulas, which can be difﬁcult to treat. Ileosigmoid ﬁstulas are among the most common ﬁstulas associ- ated with ileocolic Crohn disease, along with ﬁstulas between the terminal ileum and the ascending colon and ﬁstulas between the terminal ileum and adjacent loops of small bowel. Disease recurrence is common after ileocolic resection. Colonos- copy is the most accurate modality for postoperative surveillance and the easiest to use; it is more sensitive than either small bowel follow- through or air-contrast barium enema. For this reason, I favor an end-to-end anastomosis after ileocolic resection. In the event of re- current disease, an end-to-side, side-to-end, or side-to-side anasto- Figure 6 A large ulcer is nearly always present on the mosis may be difﬁcult to intubate.There is some evidence in the lit- mesenteric luminal border of small bowel strictures.
© 2004 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 5 GASTROINTESTINAL TRACT AND ABDOMEN 11 CROHN DISEASE — 6 COLONIC DISEASE colonic mucosa; such sloughing permits endotoxins to enter the Colonic involvement is present in 29% to 44% of patients with circulatory system and evoke a septic response. The signs and Crohn disease.30 One of the challenges in treating colonic Crohn symptoms of toxic megacolon include those characteristic of sep- disease is obtaining the correct diagnosis. Whereas Crohn disease sis—leukocytosis, fever, tachycardia, and hypoalbuminemia.These of the small bowel is fairly easy to diagnose, colonic disease often patients are very ill and often manifest ileus, which is an ominous is not. Because granulomas are not present in most cases of development that frequently signals impending perforation. Emer- colonic Crohn disease and because this condition can look very gency surgical intervention is required. At operation, the colon is similar to ulcerative colitis both endoscopically and macroscopi- often distended, and when the specimen is opened, the colon may cally, differentiation between Crohn colitis and ulcerative colitis appear almost autolytic [see Figure 9b]. In this state, the bowel fre- can be difﬁcult in the absence of small bowel or anal disease. quently does not hold staples well; accordingly, it is often helpful Colonic Crohn disease appears to be more frequently associated to sew the distal Hartmann stump between the left and right with cutaneous manifestations (e.g., pyoderma gangrenosum) [see halves of the anterior inferior rectus fascia at the lower abdominal Figure 7]. incision and then to close the skin over it.31 Thus, if the staple line is disrupted, the result is essentially a surgical site infection that Indications for Surgical Treatment can be opened and drained, rather than the pelvic abscess [see The main indications for operative management of colonic Figure 9c] that could develop if the rectal stump were located deep Crohn disease are stricture [see Figure 8], malignancy, side effects within the pelvis. of medical therapy, and failure of medical therapy. In children, fail- Types of Disease ure to recognize and treat this condition promptly may result in growth retardation. It is important to monitor both bone age and Segmental disease In a 2003 review of 92 consecutive cases insulinlike growth factor–1 levels. If these are abnormal, timely of patients with Crohn colitis, the number of patients with seg- institution of human growth hormone therapy, operative manage- mental colonic Crohn disease and the number of those with pan- ment of inﬂammatory bowel disease, or both may still permit nor- colonic disease were nearly equal.30 Approximately 63% of those mal growth and development. with segmental colitis had other disease involvement as well (e.g., Side effects of medical therapy can be substantial. They may in- jejunoileal, ileocolic, or perianal), compared with only 12% of clude such varied complications as aseptic necrosis of the femoral those with pancolitis. The recurrence rate, however, was higher in head and cataract formation (both related to steroid use), as well as patients with segmental colitis than in those with pancolitis. In an increased incidence of opportunistic infections (from immuno- addition, the risk of recurrence was higher in patients who had suppression secondary to antimetabolite therapy). granulomatous disease than in those who did not. Failure of medical therapy can refer to continuing severe disease activity or, at worst, to so-called toxic megacolon. The term toxic Pancolonic disease In cases of pancolonic Crohn disease megacolon is actually a misnomer, in that not all patients with this with associated perianal, jejunoileal, or ileocolic involvement, diag- condition actually have a true megacolon [see Figure 9a]. In com- nosis is not difﬁcult. However, most patients with Crohn pancoli- mon usage, the term toxic megacolon refers to any condition asso- tis do not have other sites of disease involvement, nor do they have ciated with colitis that is severe enough to result in sloughing of the granulomas.30 Consequently, differentiation of Crohn pancolitis a b Figure 7 (a) Shown is pyoderma gangrenosum affecting the peristomal and incisional area 6 months after creation of a loop ileostomy in a 16-year-old girl who had undergone colectomy with IPAA for a presumed initial diagnosis of ulcerative colitis. (b) Shown is peristomal gangrenosum of the breast in an otherwise asymptomatic patient with Crohn colitis and perianal Crohn disease who had a diverting loop ileostomy.
© 2004 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 5 GASTROINTESTINAL TRACT AND ABDOMEN 11 CROHN DISEASE — 7 a b Figure 8 Shown is a sigmoid colon stricture secondary to Crohn disease that caused obstructing symptoms refractory to medical therapy. from ulcerative colitis can be very difﬁcult. Many patients with Crohn disease have been inappropriately subjected to colectomy with ileal pouch–anal anastomosis (IPAA) because they were ini- tially presumed to have ulcerative colitis. Operative Procedures Total proctocolectomy with end ileostomy The tradition- al procedure for colonic Crohn disease is total proctocolectomy with end ileostomy, which is associated with an 8% to 15% rate of recurrence in the bowel proximal to the stoma.32-34 This operation remains the best choice in patients with severe rectal and anal c Crohn disease (e.g., those with so-called watering-can perineum [see Figure 10]) and carries the lowest risk of disease recurrence. In contrast to the approach taken in patients with rectal cancer, which involves excising the external anal sphincter and a large por- tion of the levator muscles, the approach taken in those with colonic Crohn disease is intersphincteric, with dissection per- formed in the plane between the internal and external anal sphincters to reduce the size of the perineal wound and facilitate healing. Even with the intersphincteric approach, delayed healing of the perineal wound is common, occurring in as many as 30% of patients. Subtotal colectomy with ileorectal or ileosigmoid anas- tomosis Because many patients with Crohn disease are young, surgeons have long been interested in operations that do not involve an ileostomy. In the absence of signiﬁcant rectal and anal disease, subtotal colectomy with ileorectal or ileosigmoid anasto- mosis is an option. Unfortunately, this operation is associated with high recurrence rates (up to 70%)35; however, with the advent of more effective immunosuppressive and biologic therapy, it is hoped that these rates can be reduced. As much palpably normal distal rectum and colon as possible should be spared. The anastomosis Figure 9 (a) Shown is toxic megacolon in a 17-year-old girl with Crohn colitis. The colon is massively distended and near perforation can be stapled, though if the bowel wall is thickened, many sur- at the time of operation. (b) When the specimen is opened, it is geons would feel more secure with a handsewn anastomosis in apparent that large segments of the mucosa have sloughed off, leav- either one or two layers. ing denuded muscle wall. (c) The distal rectosigmoid is incorporated between the left and right halves of the anterior inferior rectus fas- Segmental resection Currently, more surgeons are advo- cia at the lower abdominal incision and placed underneath the skin, cating colon-sparing procedures [see 5:34 Segmental Colon Resection] which is then closed over it. Thus, in the event of disruption of the for Crohn disease. Although this is a relatively new approach, distal stump, the contents drain harmlessly through the wound.
© 2004 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 5 GASTROINTESTINAL TRACT AND ABDOMEN 11 CROHN DISEASE — 8 would knowingly perform in a patient with Crohn disease, every year there are many such patients who undergo this procedure as treatment of colonic inﬂammatory bowel disease that initially is incorrectly presumed to be ulcerative colitis but later is diag- nosed as Crohn disease (on the basis of either ﬁnal pathologic analysis of the resected specimen or the disease’s clinical behav- ior). Generally speaking, in the absence of ﬁstulizing disease, most of these patients are able to maintain their pouch, but they require medical therapy for disease control.30,37-40 ANAL DISEASE Types of Disease With stenosis For patients with anal strictures that are not reg- ularly dilated, the outlook is poor. Such strictures pose functional ob- structions and typically lead to continuing problems with ﬁstulas and suppurative disease.They frequently become more and more ﬁbrot- ic over time and often extend proximally. Most of these patients eventually require fecal diversion. Management generally involves Figure 10 Shown is so-called watering-can perineum secondary self-dilation, which can often be done with Hegar dilators. If the to severe perianal Crohn disease. stenosis is not dealt with, all other treatment of the Crohn disease is doomed to failure; obstruction at the level of the anal canal inevitably results in the persistence of anorectal disease. there have already been some reports documenting the safety of segmental resection in cases of limited disease.36 In patients with Without stenosis Anal Crohn disease without stenosis is colonic strictures resulting in obstruction, segmental resection into much easier to treat medically. Long-term oral metronidazole ther- palpably normal areas of the bowel yields prompt resolution of apy is often helpful; other medications (e.g., anti–tumor necrosis symptoms. Because the colon performs an important water-absorb- factor antibody) may be useful as well. Broad ﬁssures are usually ing function, many patients with a limited amount of small bowel asymptomatic. Surgical treatment should be avoided unless the can still live without intravenous supplementation if a signiﬁcant lesions are causing symptoms. Because they tend to have more liq- segment of the colon is left in situ. However, patients with seg- uid bowel movements, patients with Crohn disease need an opti- mental Crohn disease appear to have a higher recurrence rate than mally functioning anal sphincter; hence, ﬁstulotomies, which those with pancolitis, as do patients with granulomas.30 Surgical divide portions of the sphincter, should be avoided if at all possi- treatment of Crohn disease continues to undergo reevaluation and ble. Placement of setons through ﬁstula tracts can often prevent reassessment of results on the basis of the availability of newer abscess formation, provide drainage, and thereby prevent perianal medical therapies. pain while minimizing sphincter trauma. Silk sutures, vessel loops, or Penrose drains also can be used as setons [see Figure 11]. Recto- Colectomy with IPAA Although colectomy with IPAA [see vaginal ﬁstulas pose a particular challenge. In the presence of 5:33 Procedures for Ulcerative Colitis] is not an operation that one active Crohn disease, advancement ﬂap repair of such ﬁstulas has a low success rate.41 Laparoscopic-assisted loop ileostomy improves the success rate, but unfortunately, the ﬁstulas may recur when intestinal continuity is reestablished. Postoperative Management CHEMOPROPHYLAXIS In 1995, a prospective, randomized study showed that patients who underwent ileocolic resection and were given mesalamine postoperatively had a signiﬁcant reduction in both the symptom- atic and the endoscopic rate of recurrence.29 Not all of the work done since then has conﬁrmed these results, but several studies and a meta-analysis have indicated that mesalamine does reduce the postoperative recurrence rate of Crohn disease.42 Many pa- tients undergoing surgical treatment of Crohn disease are ad- vised to take some type of postoperative preventive medical ther- apy—either a 5-ASA derivative (e.g., mesalamine) or a stronger immunosuppressive agent (e.g., 6-mercaptopurine or azathio- prine). Better studies are required to document the efﬁcacy of Figure 11 Vessel loops can be used as setons for drainage of the latter agents in preventing recurrence. It is hoped that abscesses caused by perianal Crohn disease. They can be left in as chemoprophylaxis will reduce the anticipated recurrence rates long as necessary and help prevent recurrent abscess formation. by 30% to 40%.
© 2004 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 5 GASTROINTESTINAL TRACT AND ABDOMEN 11 CROHN DISEASE — 9 SURVEILLANCE BEHAVIORAL MODIFICATION At present, there are no clear guidelines for surveillance after Exposure to cigarette smoke is known to exacerbate the symp- operative treatment of Crohn disease. In my opinion, however, toms of Crohn disease. Smoking has been reported to affect the given the increased risk of colorectal cancer in this setting, overall severity of the disease, with smokers having a 34% higher patients with Crohn disease who retain some colon should recurrence rate and a higher rate of reoperation than nonsmok- undergo colonoscopy every 2 years, not only to detect any ers.43-45 A 1999 study of 141 Crohn disease patients who had development of colonic neoplasia but also to identify any recur- undergone ileocolic resection, of whom 79 were nonsmokers and rence of disease in a timely manner. If recurrent Crohn disease the remainder were smokers, found that the respective 5- and 10- is detected, appropriate medical therapy should be promptly year recurrence-free rates were 65% and 45% in smokers and 81% instituted, with the aim of avoiding subsequent operation if and 64% in nonsmokers. The recurrence rates were higher in possible. heavy smokers (≥ 15 cigarettes/day) than in moderate smokers.46 References 1. Farmer RG, Hawk WA, Turnbull RB Jr: Clinical 187:47, 2004 tions for primary and recurrent Crohn’s disease patterns in Crohn’s disease: a statistical study of 17. Murray JJ, Schoetz DJ Jr, Nugent FW, et al: of the large intestine. Surg Gynecol Obstet 148:1, 615 cases. Gastroenterology 68:627, 1975 Surgical management of Crohn’s disease involv- 1979 2. Gasche C, Scholmerich J, Brynskov J, et al: A ing the duodenum. Am J Surg 147:58, 1984 33. Ritchie JK, Lockhart-Mummery HE: Non- simple classiﬁcation of Crohn’s disease: report of 18. 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By Burril B. 39:1315, 1996 cessful management of Crohn’s disease of the Crohn, Leon Ginzburg, and Gordon D. Oppen- ileoanal pouch with inﬂiximab. Gastroenterology heimer. JAMA 251:73, 1984 25. Dietz DW, Laureti S, Strong SA, et al: Safety and 117:429, 1999 9. Fazio VW, Marchetti F, Church M, et al: Effect long-term efficacy of strictureplasty in 314 patients with obstructing small bowel Crohn’s 41. Sonoda T, Hull T, Piedmonte MR, et al: Out- of resection margins on the recurrence of disease. J Am Coll Surg 192:330, 2001 comes of primary repair of anorectal and recto- Crohn’s disease in the small bowel: a randomized vaginal ﬁstulas using the endorectal advance- controlled trial. Ann Surg 224:563, 1996 26. Hamilton SR, Reese J, Pennington L, et al: The ment ﬂap. Dis Colon Rectum 45:1622, 2002 10. Galandiuk S, O’Neill M, McDonald P, et al: A role of resection margin frozen section in the sur- gical management of Crohn’s disease. Surg 42. 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Gillen CD, Walmsley RS, Prior P, et al: Ulcera- Effect of cigarette smoking on recurrence of and ileocolitis: crude and cumulative rates. N tive colitis and Crohn’s disease: a comparison of Crohn’s disease. Gastroenterology 98:1123, 1990 Engl J Med 293:685, 1975 the colorectal cancer risk in extensive colitis. Gut 29. McLeod RS, Wolff BG, Steinhart AH, et al: Pro- 45. Cottone M, Rosselli M, Orlando A, et al: Smok- 35:1590, 1994 phylactic mesalamine treatment decreases post- ing habits and recurrence in Crohn’s disease. 14. Milsom JW, Hammerhofer KA, Bohm B, et al: Gastroenterology 106:643, 1994 operative recurrence of Crohn’s disease. Gastro- Prospective, randomized trial comparing laparo- enterology 109:404, 1995 46. Yamamoto T, Keighley MR: The association of scopic vs. conventional surgery for refractory 30. Morpurgo E, Petras R, Kimberling J, et al: Char- cigarette smoking with a high risk of recurrence ileocolic Crohn’s disease. Dis Colon Rectum 44:1, 2001 acterization and clinical behavior of Crohn’s dis- after ileocolonic resection for ileocecal Crohn’s ease initially presenting predominantly as colitis. disease. Surg Today 29:579, 1999 15. Young-Fadok TM, HallLong K, McConnell EJ, et al: Advantages of laparoscopic resection for Dis Colon Rectum 46:918, 2003 ileocolic Crohn’s disease: improved outcomes 31. Hull T, Fazio VW: Surgery for toxic megacolon. and reduced costs. Surg Endosc 15:450, 2001 Mastery of Surgery, 3rd ed. Nyhus LM, Baker 16. Moorthy K, Shaul T, Foley RJ: Factors that pre- RJ, Frischer JE, Eds. Little Brown & Co, Boston, Acknowledgment dict conversion in patients undergoing laparo- 1996, p 1437 scopic surgery for Crohn’s disease. Am J Surg 32. Goligher JC: The outcome of excisional opera- Figures 3 and 4 Tom Moore.
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