Ulcerative Colitis

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CHAPTER 31

INTRODUCTION

Ulcerative Colitis

Approximately 10% of all cases of ulcerative colitis have their onset during childhood. In the Western World the incidence of pediatric ulcerative colitis increased until the 1970s and 1980s but has remained at the same level thereafter. A typical age at the onset of symptoms is prepuberty or puberty. A trend towards earlier appearance of symptoms has been noted re- cently; some patients develop symptoms as early as their primary school ages.

The aetiology of ulcerative colitis is still unclear;

therefore, there is no curative treatment. The medical therapy of ulcerative colitis is based on systemic or local suppression of the immune response of the large bowel. This is best achieved by using acetylsali- cylic acid derivatives, and systemic or locally acting corticosteroids.

In children, ulcerative colitis is more aggressive than in adults. Children present more often with widespread disease and develop pancolitis more often than adults. Therefore, children require more aggressive medical treatment than adults; corticosteroids are usually needed to control the initial disease.

Systemic corticosteroids are a major concern; the side-effects of high-dose corticosteroid treatment on a growing and developing body are significant and are often an indication for surgical treatment.

Between 40–70% of children with ulcerative colitis undergo surgical treatment. As most patients can be stabilized by medical treatment, emergency oper- ations for toxic megacolon, unremitting bleeding or refractory fulminant colitis are not common today.

The typical indications for surgery of ulcerative colitis are poor response to optimal medical treatment, dependence on high-dose corticosteroids with significant side-effects, delay in growth and maturation and severe extra-intestinal manifestations of the disease. Surgery should not be considered as a primary or early treatment of ulcerative colitis. A significant proportion of patients achieve long-term symptom relief with conservative treatment and may remain in remission with minimal or no medication. Moreover, the functional outcome following restorative proctocolectomy is not comparable with normal bowel function. When patients go through several exacer- bation phases of the disease they gradually learn to accept that their bowel will function between a few to several times a day. Before proctocolectomy is under-

taken Crohn’s disease should be ruled out by every possible measures. Crohn’s disease patients should not undergo restorative proctocolectomy

The gold standard of surgery for ulcerative colitis had been proctocolectomy and permanent ileostomy. Limited colonic resections, and colectomy and ileorectal anastomosis have been abandoned because these have been associated with a high incidence of complications and recurrence of the disease. Proc- tocolectomy and permanent ileostomy gives excel- lent control of ulcerative colitis and related symptoms, but is not very well tolerated by children and adolescents because of significant social restrictions and permanently altered body image that are related to this operation. Since the late 1970’s restorative proctocolectomy with ileoanal anastomosis has gained overall acceptance as the standard operative procedure for adult and also pediatric ulcerative colitis. Many pediatric surgeons advocate use of an ileal reservoir; the most popular and easiest to construct is the J-pouch. Some pediatric surgeons still use straight ileoanal anastomosis without a reservoir.

Restorative proctocolectomy is a major operation with significant incidence of post-operative complications. Septic complications are common as most patients with refractory ulcerative colitis are immu- nosuppressed because of high-dose corticosteroid treatment. The nutritional status of many patients is often not very good due to long-term diarrhoea and poor nutrient intake. To avoid septic complications it is imperative that systemic corticosteroids are ta- pered to as low a level as possible, or preferably changed to locally acting budesonide that has less systemic immunosuppressive effects. The nutritional status should also be improved. It is usually possible to do this by dietary measures. Parenteral nutrition is sometimes but rarely required to restore proper nutritional status.

If the patient has chronic diarrhoea, as many of them have, the bowel may be emptied by a simple colonic washout. If the patient does not have diarrhoea a whole-bowel washout with polyethyleneglycol (PEG) solution is advisable. The site of the covering loop ileostomy should be marked with a water-resist- ant marker pen before the operation. A stoma site in the right lower abdominal quadrant is best deter- mined when the patient is sitting.

Risto J. Rintala

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Figure 31.1

Prophylactic antibiotic treatment (cefotaxime and metronidazole) is started at the induction of the anaesthesia. The operation is undertaken under gener- al anaesthesia; the use of nitrous oxide as an an- aesthetic is best avoided as it may distend the bowel.

Insertion of an epidural catheter for local anaesthet- ic infusion to control post-operative pain is advisable. Additional pain control can be achieved by ad- ministrating opioids by a PCA (patient controlled analgesia) system. A bladder catheter is inserted and left in place until pain control with opioids and epidural catheter can be discontinued.

The patient is positioned in a lithotomy position with a 10–15º Trendelenburg tilt. The abdomen is prepped from lower chest to perineum. A midline incision starting from the midpoint between the xi- phoid process and umbilicus and extending through the umbilicus down to suprapubic region is used to get free access to the whole length of colon. Usually there is no need to use self-retaining wound retractors; they may cause wound edge ischaemia and in- crease postoperative wound pain. The whole length of the bowel is inspected to rule out Crohn’s disease.

Figure 31.2

It is important that the surgeon assesses that the terminal ileum reaches down to perineum before colectomy is started. If the rotation of the bowel is normal and the terminal ileum reaches the pubic bone, it is very likely that an ileoanal anastomosis can be performed without undue tension. After mobilization of ileocecal region, the ileum is transected by a GIA stapler, flush to the ileocecal junction.

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Figure 31.3

The peritoneal reflections of the ascending colon and hepatic flexure are mobilized. The splenocolic liga- ment is severed and splenic flexure mobilized. The greater omentum can be preserved provided it does not tear much during its dissection off from the transverse colon. The dissection of the omentum is best performed by bipolar cautery or scissors; the at- tachments between transverse colon and omentum are transected flush to the colonic wall. The lateral

peritoneal reflections of the descending and sigmoid are transected. The vessels in the colonic mesenteri- um are ligated or cauterized near the bowel wall; usually only the main arteries to the colon, right, middle and left colic arteries need ligatures. The colon is transected at the junction of the sigmoid and rectum with a GIA stapler. The whole colon can be now removed from the operative field.

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Chapter 31 Ulcerative Colitis 335

Figure 31.1 Figure 31.2

Figure 31.3

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Figure 31.4, 31.5

Stay sutures or a right-angled large clamp on the proximal rectal stump facilitate the dissection of the rectum. These allow the surgeon to pull and move the bowel on either side freely. The mesentery of the rectum in patients with severe ulcerative colitis is often inflamed and very thick. Dissection within the mesentery is time consuming and bloody. The easiest way to proceed is to keep the plane of dissection right on the rectal wall. The small vessels entering the bowel are cauterised flush to the bowel wall. Broad and

long-bladed retractors and cranial pulling from the rectal stump facilitate the dissection.

The dissection is continued down to the level of pelvic floor. Rectal finger-examination is useful to assess the adequacy of abdominal dissection. If the lowest level of abdominal mobilization is within 3–4 cm from the anal verge, no problems are expect- ed in the transanal mucosectomy and rectal pull- through.

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Figure 31.4 Figure 31.5

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Figure 31.6

The next stage in the operation is the mobilization of the ileum to reach to anal canal. The ileocolic artery is ligated and transected. The mesentery of the ileum is mobilized up to the level of proximal superior mesenteric artery. This may require mobilization of the root of the mesentery from duodenum and lower rim of pancreas. The mesenteric arteries to the distal two or three vascular arcades of the terminal ileum are ligated and transected proximally. In order to ensure tension-free ileoanal anastomosis, the distal end of the ileum or the tip of the J-pouch should reach in front of the pelvic rim to the base of penis in males or anterior vestibulum in females.

Figure 31.7

The length of the J-pouch is between 7–10 cm. The terminal ileum is folded and the antimesenteric tip of the future pouch longitudinally opened with a cautery needle. The opening should be kept short, from 1.5 to 2 cm, as it widens significantly when pulled through to the anus. The GIA-stapler blades are inserted into each arm of the pouch; the stapler is closed and fired. One firing of a 75-mm stapler, or two of a 50-mm stapler, is usually sufficient to construct a pouch. The stapled suture line can be rein- forced with 4/0 or 5/0 absorbable sutures. The pouch and terminal ileum are wrapped in warm and moist swabs and returned the abdomen. The abdominal incision is loosely packed with warm and moist swabs.

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Figure 31.6 Figure 31.7

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Figure 31.8

The perineal phase of the operation starts by insertion of holding stitches between the mucocutaneous junction of the anal canal and a colostomy ring.

These keep the anus open and dilated, and give excel-

lent access to the anal canal. Adrenaline in saline (1:100,000) is injected under the mucosa to lift it up and decrease bleeding during the initial phases of transanal mucosectomy.

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Figure 31.9, 31.10

The transanal mucosectomy is started at the dentate line. A small rim (5–6 mm) of anal transitional epithelium should be left in situ, otherwise the sensibil- ity of the anus is significantly decreased and the anal sampling reflex may be lost. Disease recurrence in the transitional anal epithelium is unlikely. The whole circumference of the anal canal mucosa is in- cised and the mucosectomy started. The red line in Fig. 9 depicts the line of dissection between the mucosa and rectal muscle. Some surgeons prefer to use multiple stay sutures in the mucosa just above the level of the mucosal incision to facilitate mucosecto-

my. The author uses small triangular clamps to grasp the edge of the mucosal cuff. The mucosectomy is performed by a combination of sharp and blunt dissection with scissors. In colitis ulcerosa, the mucosectomy is much more difficult than in non-inflam- matory conditions; blood loss is often also significant. Pre-operative treatment with locally acting cor- tisone foam or suppositories may decrease blood loss and make the dissection easier. The mucosectomy is continued for 5–8 cm until the level above pelvic floor is reached.

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Figure 31.8

Figure 31.9 Figure 31.10

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Figure 31.11, 31.12

The cuff consisting of muscular lining of the anal canal and distal rectum can be transected transanally when the pelvic cavity is entered at the upper end of the mucosectomy. Pulling from the mucosal tube tents the proximal end of muscular cuff inside the more distal cuff; thus, the cuff can be safely transected without damaging urethra and prostate. Another option is to evert the rectum through anus and sever the muscular cuff outside the anus at the upper end of mucosectomy. Bleeding from small vessels in the cuff can be controlled by cautery.

A long soft clamp is inserted through the anal muscular cuff to pelvis. The J-pouch (or distal ileum

in case of straight pull-through) is grasped with the clamp and pulled through to the anus. The assistant confirms through the laparotomy incision that the mesentery of the pulled-through pouch is not twist- ed. The mesentery of the pulled-through J-pouch (or distal ileum) is the tightest component of the pulled- through segment and requires the shortest route.

Therefore, it is natural that in the pelvis the mesentery is positioned anteriorly to the bowel; this does not mean that the pull-through segment would be kinked.

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Figure 31.11 Figure 31.12

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Figure 31.13

As there is often significant tension when the first anastomotic stitches are inserted, it is advisable to use four-quadrant holding stitches to initiate the su- turing of the anastomosis. The anastomosis is performed in one layer with interrupted sutures between full-thickness ileum and anal canal. A suit- able material is absorbable 4/0 sutures. The tension in the suture line is usually relieved when the sutures between the anus and the stoma ring are cut. This al- lows the suture line to retract within the anal canal.

Figure 31.14

The space between pulled-through bowel and poste- rior peritoneum is closed with running 4/0 absorbable sutures. The pelvic cavity is inspected for bleeding sites. A round skin disk is cut off the stoma site. A cruciate incision is made to the abdominal muscle fascia at the site of the stoma. The opening in the fascia and abdominal muscles are bluntly enlarged to accommodate two fingers. An opening is made in the anterior peritoneum and a loop of ileum as near as possible to the ileoanal anastomosis is pulled out for stoma formation. The abdominal incision is closed in layers and the stoma is matured over a stoma rod.

Generally there is no need to drain the pelvic cavity.

Post-operative gastric decompression by nasogas- tric tube is usually not required. The bladder catheter can be removed when epidural anaesthesia is discontinued. Antibiotic prophylaxis is continued for 72 h post-operatively. If the patient has been on high-dose corticosteroids pre-operatively, post-operative parenteral corticosteroid therapy is required until oral intake of medication is possible. Post-operative corticosteroids can be discontinued when sufficient function of patient’s own adrenal glands is confirmed by an ACTH-stimulation test.

Enteral feeding is encouraged and most patients tolerate full enteral feeding within the first 5 post-operative days. Stoma output is often excessive and should be replaced according to output and electro- lyte content of the stoma fluid. In most cases Ringer’s lactate is sufficient for replacement. Oral sodium supplementation to decrease stoma output is started as soon as the patient tolerates intake of salt tablets.

Post-operative dietary management consists of lactose-free low residue diet. The amount of sodium supplementation can be monitored by spot urinary sodium measurements. The urinary sodium concen- tration should be kept higher than 20 mmol/l. Insuf- ficient salt intake leads to increased and watery stoma output.

The covering stoma can be taken down when the healing of the pouch and the ileoanal anastomosis is confirmed. A distal loopogram 3–6 weeks after the operation is used to assess the integrity of the ileoanal anastomosis and J-pouch. The immediate post- operative phase is characterized by loose and fre- quent bowel movements up to 10–12 times per 24 h.

Antipropulsive medication (loperamide) is useful in slowing down gut motility. The bowel frequency gradually decreases to 2–7 bowel movements per 24 h within 3–6 months. Low residue diet and salt supplementation are helpful during the adaptation phase.

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Figure 31.13 Figure 31.14

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CONCLUSION

Ileoanal anastomosis has revolutionized the treatment of ulcerative colitis also in children. Long-term patient satisfaction following the operation is excel- lent despite a high incidence of post-operative complications. In children with a pouch ileoanal anastomosis, the functional outcome in terms of fecal continence is good. Usually all patients resume full daytime faecal continence within 6 months following closure of the stoma. A few patients suffer from slight night-time staining that may require protective pads.

In the absence of major post-operative complications, gross faecal soiling is practically nonexistent.

The frequency of bowel movements 6–12 months post-operatively is between two and seven times per 24 h. In the series of Children’s Hospital, University of Helsinki, the median bowel frequency 6 months post-operatively is four, ranging from two to seven per 24 h.

Early and late complications occur in 20–50% of patients; most common are wound infections in patients who have been using high-dose corticosteroids prior to the operation, and bowel obstructions. Pelvic septic complications or separation of the ileoanal an- atomosis occur in less than 10% of the cases. Acute or

chronic inflammation in the pouch (pouchitis) is an innate problem related to ileoanal pull-through for ulcerative colitis. Its incidence varies between 20%

and 50% of the patients. Most acute bouts of pouchitis respond rapidly to a short course of oral antibiotics, for example metronidazole. Chronic pouchitis is much less common and occurs in less than 10% of the patients. The treatment of chronic pouchitis consists of long courses of low-dose antibiotics and in re- calcitrant cases oral corticosteroids, preferably budesonide. Chronic pouchitis may be a presentation of Crohn’s disease; eventually 5–15% of patients who have undergone ileoanal anastomosis for ulcerative colitis are discovered to have Crohn’s disease. An- other symptom that should raise suspicion of Crohn’s disease is pouch fistulization, especially re- current fistulas.

Despite the multitude of potential post-operative problems, a great majority of patients who have had restorative proctocolectomy for ulcerative colitis resume highly satisfactory lifestyle with complete faecal continence and acceptable frequency of daily bowel movements.

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SELECTED BIBLIOGRAPHY

Coran AG (1990) A personal experience with 100 consecutive total colectomies and straight ileoanal endorectal pull- throughs for benign disease of the colon and rectum in children and adults. Ann Surg 212 : 242–248

Durno C, Sherman P, Harris K et al (1998) Outcome after ileoanal anastomosis in pediatric with ulcerative colitis. J Pedi- atr Gastroenterol Nutr 27 : 501–507

Rintala RJ, Lindahl H (1996) Restorative proctocolectomy for ulcerative colitis in children – is the J-pouch better than straight pull-through. J Pediatr Surg 31 : 530–533

Sawczenko A, Sandhu BK (2003) Presenting features of inflam- matory bowel disease in Great Britain and Ireland.Arch Dis Child 88 : 995–1000

Stavlo PL, Libsch KD, Rodeberg DA et al (2003) Pediatric ileal pouch – anal anastomosis: functional outcomes and qual- ity of life. J Pediatr Surg 38 : 935–939