Surgery in Crohn’s Disease: Two Cases from Jamaica

Surgery in Crohn’s Disease

Two operative cases of Crohn’s Disease

Introduction

Crohn’s Disease is uncommon in Jamaica although recently there has been an increase in the number of patients with Crohn’s Disease seen at the University Hospital. Surgery is usually required for the complications of Crohn’s Disease. Acute free perforation, massive hemorrhage and toxic dilatation are rare acute complications. More commonly, patients come to surgery with intestinal obstruction, medical intractability, chronic anemia, fistula and abscess (Wolff 1986; Greenstein 1987). Surgery is usually undertaken for the latter indications. Two cases of Crohn’s Disease who had surgery at the University Hospital, Jamaica, will be presented and the management of Crohn’s Disease outlined.

Case I

Four years before presentation to hospital, a twenty-year-old male presented to a gastroenterologist with abdominal pains. He was investigated and diagnosed as having Crohn’s Disease for which he was maintained on sulfasalazine and prednisone. He was admitted to the Kingston Public Hospital with subacute intestinal obstruction. Azathioprine was added to his drug regimen and he had blood transfusions for anemia. There was improvement on conservative management but later he began vomiting intermittently and his stools became diarrheal. Although he became edematous, he lost 30 pounds of body weight over the next three months.

Barium enema examination showed a stricture in the ascending colon (Fig. 1: Barium enema showing stricture in ascending colon). At colonoscopy, a cobbled stone mucosa with ulceration was seen in the ascending colon distal to the stricture. Biopsies of this mucosa detected stromal fibrosis only.

In this admission, the patient had right axillary vein thrombosis and right calf deep vein thrombosis with pulmonary embolism. Anticoagulation was begun. He was then transferred to the Tropical Metabolic Research Unit (TMRU) for nutritional support. Here he became febrile and tachypneic with diminished breath sounds over the left side of the chest. His PO2 was 50 mmHg, PCO2 28 mmHg and pH 7.49. Chest x-ray showed a left pleural effusion and lung scan showed segmental perfusion defects consistent with a diagnosis of pulmonary embolism. The patient was then transferred to the medical floor of the University Hospital. Here his colicky pains were noted to be predominant and he was vomiting.

On physical examination the patient was emaciated and distressed by pain. His mucous membranes were pale. He had clubbing of the fingers and dependent edema. He weighed 110 lbs. His skin was dry and shiny with variable pigmentation and flaky squames. His tongue had no filiform papillae and his hair was sparse. His radial pulse rate was 120/min, blood pressure was 120/80 mmHg, and heart sounds were normal. Over his posterior chest, the percussion note was diminished and there were crackles over both bases. His abdomen was distended, tympanitic, and tender over a right lower abdominal mass. Bowel sounds were hyperactive. His perianal skin was erythematous and he had an anterior anal skin tag.

His hemoglobin concentration was 8.2 gm/dL, and white blood cell count 4.3×109/L. Serum urea, electrolytes and creatinine were normal. Results of other blood investigations were:

PT	26/14	sec
PTT	53/31	sec
Ca++	1.9	mmol/L
Bili(T)	5	mol/L
Alkaline phos	243	IU/L
SGOT	14	IU/L
GGT	314	IU/L
Proteins	43	g/L
Albumin	15	g/L
Globulin	28	g/L

His intestinal obstruction resolved on medical management and elective surgery was planned to follow his course of anticoagulation. Two weeks later intestinal obstruction recurred and conservative management was again undertaken but after three days of unremitting obstruction, emergency surgery was undertaken. Heparin was discontinued six hours before surgery. Preoperative PT and PTT were normal. Prophylactic antibiotics and heparin were given along with hydrocortisone. At operation, the bowel was dilated proximal to a stricture in the ascending colon. The ileocolic segment was inflamed, and “creeping fat” was noted on the distal ileum. Mesenteric lymph nodes were enlarged. A right hemicolectomy and an ileotransverse anastomosis were performed.

Parenteral nutritional support was begun on the first day after surgery. On postoperative day two the patient passed flatus and oral fluid, prednisone and sulfasalazine were started. Warfarin anticoagulation was commenced on day seven. Parenteral nutritional supplementation was given for 10 days. On day twelve the patient was discharged home on a supplemented diet, prednisone and sulfasalazine.

The excised ileocolic segment showed a fibrous stricture in the ascending colon. The intestinal wall was thickened and inflamed and the mucosa had extensive ulceration and a cobbled stone appearance (Fig. 2). The resection margins were grossly involved. Histological examination of the specimen showed mucosal ulceration and fissures. Some of the fissures extended into the submucosa. Additionally, there was lymphoid hyperplasia, lymphangiectasia, and hyperplasia of the muscularis mucosa, fibrosis and formation of ill-defined granulomas composed of epithelioid and multinucleated giant cells. There was accompanying glandular distortion without atrophy. The resection margins were involved to a lesser extent. Lymph nodes showed sinus edema and congestion but no granulomas.

Case II

Three years before attending the University Hospital, twenty-nine-year-old L.T. was admitted to another institution with right iliac fossa pain and tenderness. After appendectomy, he was diagnosed as having Crohn’s Disease. He suffered recurrent abdominal pains and diarrhea. He presented to the University Hospital with an exacerbation of abdominal pains and diarrhea. On admission, he was found to be anemic and to have an abdominal mass. Abdominal ultrasonography demonstrated a small bowel mass in the right iliac fossa. Subacute intestinal obstruction resolved on conservative management. He was discharged home on acetylsalicylate and prednisolone.

Three weeks later L.T. was readmitted to hospital with recurrence of abdominal cramps and distension. He began vomiting and his pain became constant. He then became febrile. On physical examination, he was found to be dehydrated and to have generalized peritonitis.

Investigations were as follows: hemoglobin concentration 12.7 gm% and white blood cell count 39.2×109/L. Urea and electrolytes were normal. X-rays of the chest showed extensive pneumoperitoneum.

The patient was resuscitated and given antibiotics. The findings at emergency laparotomy included a perforation of the distal jejunum, multiple small bowel strictures proximal and distal to the perforation. The distal bowel was matted and inflamed. Subphrenic, interloop and pericolic abscesses were found. The abscesses were drained and the grossly affected bowel resected and primarily anastomosed. The skin incision was left open to heal by secondary intention.

Recovery was uneventful. The patient began oral intake on the fourth postoperative day. He was discharged home on the tenth postoperative day. Histopathological examination of the resected specimen confirmed Crohn’s Disease and showed microscopic involvement of the resection margins.

Discussion

Before outlining the surgery for Crohn’s Disease, comments on Cases I&II will be made. Difficulty with biopsy diagnosis of Crohn’s Disease, as with Case I, is well known. The clinical and radiographic diagnoses, however, are usually characteristic. Both Cases I&II were malnourished. They both had recurrent abdominal pains due to bowel strictures and progressed to recurrent intestinal obstruction. As discussed below, they both had indications for earlier surgical intervention. In hospital, both patients developed further complications before coming to surgery. Case I had multiple deep vein thromboses and recurrent pulmonary embolism. A hypercoagulable state is well documented in Crohn’s Disease. On the other hand, Case II went on to develop the rare complication of free perforation. In this instance, the signs of intra-abdominal sepsis were masked by steroid therapy and the patient was taken to theatre some days after perforation.

At operation, both cases had resection across grossly involved bowel. In Case I the external involvement at the level of transection appeared mild and after division the bowel mucosa also appeared minimally affected. In Case II gross involvement was only apparent on the mucosal aspect of the bowel. Whether or not gross involvement at the resection margin predisposes to recurrence or leakage is debatable. Moreover, both cases were receiving steroids and Case II had intra-abdominal abscesses. Nevertheless, Case I had ileocolic resection and primary anastomosis and Case II had ileal resection and primary anastomosis. Neither case developed evidence of anastomotic leakage or abscess formation. Both patients regained weight. Case I was maintained on anticoagulation for 6 months postoperatively and has not had recurrence of deep vein thrombosis or pulmonary embolism. Two years after surgery Case I has not had re-operation although he is maintained on sulfasalazine for occasional abdominal cramps. Seven months after surgery Case II has not shown any clinical evidence of recurrence.

When to operate

Patients with active Crohn’s disease are usually on medical treatment and the decision to undertake elective surgery requires the sound judgement of gastroenterologist and surgeon. Patients, who have recurrent intestinal obstruction, disabling symptoms, malnutrition, growth retardation or other reasons for failure of medical management, should have elective surgery. Some physicians and surgeons delay surgical treatment in the hope that time or medical treatment will bring sustained benefit. Others are anxious about the risk of postoperative complications and recurrent disease. Controlled studies have shown no benefit with prolonged medical treatment and delaying surgical treatment will not reduce the incidence of postoperative complications. Surgery performed for proper indications is almost invariably rehabilitating (Allan 1988).

Principles of Surgery for Crohn’s Disease

Surgery for Crohn’s Disease is guided by the principles:

• Crohn’s Disease is panintestinal.
• Surgery does not cure the disease.
• Surgery should be made as safe as possible.
• Surgery is required to treat the complications.
• There is a 50% chance of recrudescence so bowel conservation should be practiced.
• Stenotic complications may be treated by stricturoplasty.
• Patients who have a recurrence may not need a further procedure and until they have a recurrence may have a normal, medication-free life-style for several years.
• Mortality from the disease itself is now low.

Preparation for surgery

Some patients scheduled for elective surgery are severely nutritionally depleted and anergic and require total parenteral nutrition (TPN) to gain weight and strength and to reverse the anergic state. All patients for elective surgery have bowel preparation and perioperative antibiotics. Patients on steroids also have perioperative hydrocortisone.

Further surgical management for Crohn’s Disease is best discussed according to location.

Gastric and duodenal disease

Surgical treatment of gastroduodenal disease is reserved for persistent or recurrent obstructive symptoms. Conventional treatment for stricture of the distal stomach or the duodenum is gastrojejunostomy. Strictureplasty has been used for duodenal strictures (Shepherd et al 1985).

Ileal and ileocolic disease

Crohn’s disease most commonly affects the distal ileum or ileocecal region and most commonly requires surgery for intestinal obstruction produced by a stricture. Obstruction is initially treated conservatively. Recurrent attacks and complete irreversible obstruction, however, are indications for operative intervention. Ileal or Ileocecal resection is the procedure of choice.

Generally, for involvement of a single bowel segment all disease is excised. However, if clear margins are not easily obtainable or if skip areas are found, only the severely involved segments are resected or strictureplasty is performed (Wolff, 1986). Margins of resection with extensive disease or skip areas are usually limited to 5 to 10 cms above macroscopic disease in an effort to preserve bowel (Fazio 1990). Recent studies using actuarial data show no difference in the rate of recurrence between those with microscopically normal and abnormal suture lines (Pennington et al 1980; Lee and Papaioannou 1980; Pennington et al 1980; Homan et al 1983). Some surgeons therefore take resection margins of macroscopically normal bowel as little as 2 cm away from diseased bowel.

Strictureplasty (Lee 1982), has been recommended for multiple, short, fibrotic strictures in a patient with obstructive symptoms or malabsorption due to Crohn’s Disease of the small bowel, and single or multiple, fibrotic strictures in a similarly symptomatic patient where previous small bowel resection has been done, and a short bowel syndrome is present or likely to develop (Wolff 1986; Fazio 1990).

Healing does not seem to be affected by the presence of active disease at the site, so that fistulae, leakage and intra-abdominal sepsis secondary to strictureplasty are uncommon. With this procedure, maximal conservation of small bowel is achieved for patients with extensive disease (Wolff 1986). It is contraindicated when factors are present that are deleterious to anastomotic healing (Fazio 1990). These factors are the presence of an actively inflamed segment of small bowel, free perforation or a peri-intestinal abscess, and a fistula.

Poor general condition of a patient also contraindicates strictureplasty.

Colonic disease

Failure of medical management is most frequently the indication for surgical intervention in Crohn’s disease involving the colon. Surgery is required for persistent and progressive weight loss. Surgery is also required in patients who have drug complications.

The preferred surgical option for extensive colonic disease is colectomy and ileorectal anastomosis. With extensive colitis but minimal anorectal disease, ileorectal anastomosis produces good results in approximately 30% of patients (Farnell et al 1980) but 30% of these eventually require proctectomy. If there is concomitant, severe rectal involvement panproctocolectomy is done (Allan et al 1977).

Intraperitoneal abscesses

About 30% of patients with Crohn’s Disease have intra-abdominal abscesses. All abscesses should be drained. This is usually accompanied by resection of involved bowel and exteriorization of bowel ends followed by later re-establishment of bowel continuity. Abscesses drained without resection of diseased bowel are usually complicated by fistula formation. Some selected patients with small abscesses and localized disease, like Case I, have resection and primary anastomosis.

Enteroenteric fistulae

Internal fistulae occur in approximately one-third of patients with Crohn’s disease. The most common internal fistulae are enteroenteric or enterogenitourinary. Complications of internal fistulae such as bacterial overgrowth require therapy. This includes the use of parenteral or enteral nutrition, antibiotics and immunosuppressive drugs. Although bowel rest and TPN can close fistulae in over 60% of cases, most recur on resuming oral feeds. Resection of the diseased segment is therefore often required.

Ileocolic fistulae are the most frequent enteroenteric fistulae and the majority of these are ileosigmoid. Most of these require surgery. However, the presence of an asymptomatic or minimally symptomatic enteroenteric fistula is not an indication for surgery.

With common ileosigmoid fistulae, preoperative colonoscopy delineates the extent of colonic disease. If colonic disease is minimal ileocolic resection and simple closure of the sigmoid is enough. If there is a long segment of colonic disease with strictures or fistulae then ileocolic and sigmoid resections are done. A proximal diverting loop ileostomy is added if an anterior resection is necessary for a low ileosigmoid or ileorectal fistula. If colonic disease is universal or extensive, subtotal colectomy with ileorectal anastomosis or panproctocolectomy and end ileostomy is recommended.

Gastrocolic fistulae are rare and almost all originate in diseased colon. Therapy is therefore colonic resection and wedge excision of stomach. Similarly, colo-duodenal fistulae are usually colonic in origin and colectomy with closure of the duodenum is the treatment of choice.

Rectovaginal fistulae

The majority of rectovaginal fistulae are associated with peri-anal abscesses and fistulae. Poor results are obtained if extensive rectal involvement coexists. However, for patients with localized rectal or anal disease, proximal diversion, fistulectomy and repair is successful in some cases.

Enterovesical

For enterovesical fistulae, optimal surgical treatment consists of resection of the diseased bowel segment, bowel reconstruction, and closure of the bladder (Greenstein et al 1984). Some patients have an abscess between bowel and bladder for which resection of the diseased segment of bowel, closure of the bladder, and bowel diversion with later bowel reconstruction is the safest option.

Enterocutaneous fistulae

Enterocutaneous fistulae include ileocutaneous, colocutaneous, peri-ileostomy, and perianal fistulae. Spontaneous external fistulae are rare. External fistulae more commonly follow surgery and develop immediately after surgery due to anastomotic leak, or late due to recurrent disease. Early postoperative fistulae may heal with hyperalimentation but usually require re-exploration with diversion for septic complications. Re-anastomosis is carried out later. Late fistulae may develop spontaneously or after incision and drainage of an intra-abdominal abscess. These fistulae are operated on electively. The segment of recurrent ileitis or colitis is resected and immediate reanastomosis performed (Greenstein 1987). Fistulectomy is performed and a wick placed to prevent premature closure and allow drainage of the track for several days (Wolff 1986). Peri-ileostomy fistulae require resection of the diseased bowel, fistulous tract and reconstruction of the ileostomy (Greenstein 1987).

Anorectal and perianal disease

For anorectal disease proximal diversion alone is ineffective (McIlrath 1971). In some studies, metronidazole has been effective in the resolution of anorectal disease, but long-term maintenance is usually necessary (Brandt et al 1982). Removal of all proximal disease seems to promote healing of the anal manifestations. However, if proximal residual disease remains or recrudescence occurs proximally, the anal manifestations recur as well.

Perianal disease occurs in over 90% of patients. Perianal fistulae occur in about 25% of patients (Greenstein 1987) and are usually associated with abscesses and occasionally associated with rectovaginal fistulae. Local therapy for perianal disease consists of Sitz baths, hygiene and topical steroids. Surgical treatment includes dilation of anal strictures and drainage of abscesses. A lateral internal sphincterotomy may be done for fissures. Superficial anal fistulas are treated by fistulectomy or fistulotomy. Deep anal fistulas that are asymptomatic should not be disturbed for fear of precipitating incontinence (Wolff 1986). For severe perianal disease, a trial of TPN and bowel rest can be instituted. Immunosuppressive therapy or proctectomy has a role here.

Free perforation

Bowel perforation in Crohn’s disease is rare. With large bowel perforation, resection, colostomy, mucous fistula and later reconstruction is recommended. Selected cases with small bowel perforation may have resection and primary anastomosis (as in Case II). Otherwise, ileostomy is a safe option.

Massive hemorrhage

At surgery for hemorrhage from small bowel disease, if disease is segmental, resection and anastomosis is performed, while for hemorrhage from large bowel disease, resection, colostomy and mucous fistula with later reconstruction is recommended. With universal colitis and hemorrhage, panproctocolectomy is the procedure of choice. If there is relative rectal sparing, consideration is given to preservation of the rectum and ileorectal anastomosis.

Toxic megacolon and fulminating colitis

The primary aim of management is prevention of perforation with its increased mortality. Initially a trial of aggressive medical management is undertaken. This includes rehydration, steroids and antibiotics. Failure of this management leads to subtotal colectomy with ileostomy and mucous fistula.

Complications of surgery

A common complication of proctectomy is the unhealed perineal wound. Continuous irrigation/suction drainage reduces its incidence to a minimum. A difficult unhealing perineal wound can be covered with split skin (Anderson and Turnbull 1976), or a myocutaneous flap (Baek et al 1981). Some cases require resection of sacrococcygeal bone (Silen and Glotzer 1974). A perineal sinus may persist but does not usually cause severe disability and generally closes with local therapy or re-excision (Wolff 1986).

Other postoperative complications and sequelae include wound infection, intra-abdominal abscess, hernias, short bowel syndrome, recurrence of Crohn’s disease and rarely colon cancer. Recurrence after resection is about 50%. The risk of recurrence is less for patients with involvement mainly of the large bowel rather than of the small bowel. The recurrence rate is 60% after small bowel resection, 40% after small-bowel resection and right hemicolectomy, 39% after right hemicolectomy alone, 29% after left hemicolectomy, and 11% after proctocolectomy. Recurrence is most commonly found in the small bowel proximal to an anastomosis and manifests itself early (one to two years after surgery) or late (five to 15 years after surgery) (De Dombal 1971). Recurrence rates are lowest following resection as compared to exclusion bypass or bypass in continuity (Homan and Dinaen 1978). Colon cancer complicates longstanding Crohn’s colitis.

Conclusion

At the University Hospital, patients with Crohn’s Disease come to elective surgery after deliberation amongst gastroenterologists and surgeons. The proper timing of surgery requires experience and sound judgement. Patients who come to elective surgery will most often have bowel resection and reconstruction. Patients for emergency surgery will more often have resection and ileostomy or colostomy. Postoperatively most patients will be rehabilitated with a vastly improved quality of life.