Ulcerative Colitis

Ulcerative colitis was first described almost 150 years ago. Despite significant advancements in the knowledge of inflammatory bowel disease, this disorder's etiology and definitive treatment remain incompletely resolved. Although classically thought of as a disorder of adults, a significant number of children are afflicted. The pediatric surgeon is very often instrumental in caring for patients with this disabling disease.

Etiology

No etiology has been determined, although several theories have been suggested. A clear association with a number of factors suggest that all inflammatory bowel disorders (IBD) are due to a combination of genetic predisposition and environmental exposure to either enteric organisms or dietary factors. The identification of an IBD1 gene locus in Crohn's disease supports the genetic association with IBD. Although such a gene has not been identified in ulcerative colitis, linkage with genes on chromosomes, 3, 5, 7 and 12 have been found in some studies. Genetics clearly play a role, however, the fact that only 45% of identical twins develop IBD suggest that other processes are also important. Environmental factors including enteric organisms may influence whether IBD actually develops. An immunologic response to luminal bacteria is supported by clinical responsiveness to antimicrobials. This finding is also supported by the fact that murine models of IBD fail to develop colitis without the addition of enteric organisms. Others believe that bacteria or viruses are secondary invaders rather than primary agents, and that dietary factors have a more important role.

Approximately 15% of patients with ulcerative colitis are from families who suffer from inflammatory bowel disease. Studies of HLA antigens, particularly the W27 isoform, in patients with idiopathic ankylosing spondylitis, uveitis, and ulcerative colitis suggest a genetic predisposition, even though no definite predictable relationship has been determined. Although many workers suggest that psychologic factors may be partially etiologic, there seems to be no "premorbid personality." Psychologic factors as well as stress may provoke relapses, thus contributing to the chronicity of the disease, yet not necessarily causing the condition.

Pathology

Ulcerative colitis is primarily a disease of the rectal and colonic mucosa and submucosa. The rectum is involved in more than 95% of patients, and inflammation extends proximally in a contiguous manner. When the entire colon is involved (pan-colitis), the most severe pathologic changes are present in the rectum and sigmoid colon. Crypt abscesses are the most characteristic microscopic feature, leading to mucosal ulceration with undermining of the adjacent mucosa. Mucosal bridging and pseudopolyp formation often result. As the disease progresses, in the acute phase the colon distends, peristalsis decreases, and the muscularis becomes thin and diffusely hemorrhagic; this may progress to toxic megacolon (Fig. 65-1). Conversely, with chronic ulcerative colitis, the colon becomes stiff, thickened, and foreshortened with atrophic mucosa and loss of haustral folds. This often gives the appearance of a "lead pipe" in the descending and sigmoid colon (Fig. 65-2). In remission the mucosa may revert to a near-normal microscopic appearance. Mucosal biopsy is helpful to confirm the diagnosis and assess the activity of the disease.

From an immune perspective, the mucosa in ulcerative colitis consists of a dominate CD4+ populations of T-lymphocytes. These T-cells have an atypical phenotype with an increased production of transforming growth factor beta and interleukin-5. These cells appear to be activated by antigen presentation from adjacent epithelial cells. Additional mediators of inflammation are derived from activated macrophages which express a number of inflammatory cytokines including tumor necrosis factor, interleukin-1 and interleukin-6. These latter inflammatory factors form the basis for much of the newer medical management of this disease.

Clinical Features

Ulcerative colitis chiefly affects persons after the second decade of life, but at least 22% of all patients manifest their initial symptoms before the age of 18. Males and females are equally afflicted; however, the condition is four times more prevalent in whites than in blacks, Hispanics, or Asians. Ulcerative colitis is at least three times more common in Jews than in non-Jews. The disease appears to be increasing in both the United States and in Europe, although it remains uncommon in Asia.

In approximately 4% of patients with ulcerative colitis, the onset of their disease occurs before the age of 10 years. In 18% symptoms appear between 10 and 20 years, but the onset is most frequent during the middle of the third decade. Symptoms typically begin insidiously with persistent diarrhea followed by the appearance of blood, mucus, and pus in the stool. Cramping lower abdominal pain and tenesmus are common. Anorexia, weight loss, and growth retardation from chronic inflammation, poor appetite, and prolonged use of corticosteroids tend to occur when the disease is chronic. As a result many children suffer feelings of inferiority and lack a desire to participate in social and physical activities. Most develop remitting colitis with periodic relapses precipitated by emotional stress or intercurrent infection. After a few years occasional patients may achieve permanent remission, although most will experience chronic colitis with shorter and less frequent remissions. A single attack with complete remission occurs in less than 10% of children.

In approximately 15% of children, the onset of ulcerative colitis is acute and fulminating with profuse bloody diarrhea, severe abdominal cramps, fever, and occasionally sepsis, requiring prompt treatment. Although most will improve for varying periods with medical therapy, approximately 5% of patients will develop toxic megacolon, requiring urgent operation. At least 60% of children with colitis eventually require surgical resection.

Cancer of the colon or rectum has been reported in 3% of patients during the first 10 years of disease, increasing by as much as 20% in each subsequent decade. Cancer may develop even in those with apparent remission. Cancer is more common in patients who have pancolitis, in those whose symptoms began in childhood, and in those who have frequent flare-ups of symptoms. Evidence of dysplasia of the colonic mucosa on the biopsy specimen indicates a high risk of carcinoma development.

Extracolonic manifestations include growth retardation, arthralgias, skin lesions, failure of sexual maturation, anemia, liver disease, osteoporosis, nephrolithiasis, uveitis, and stomatitis (Figure 65-1). Growth retardation with delay in bone age frequently accompanies chronic ulcerative colitis in adolescents. Delayed sexual maturation may be in part because of abnormally low levels of 24-hour urinary gonadotropins. Growth hormone levels are usually normal for the patient's age.

Arthralgias occur in approximately 20% of patients with ulcerative colitis, usually involving the knees, ankles, and wrists. Joint symptoms occasionally precede the onset of intestinal symptoms, sometimes being confused with juvenile rheumatoid arthritis. Ankylosing spondylitis may be seen in 1 to 6%, and sacroiliitis in 4 to 18% of patients. Both of these conditions may be permanently disabling. Aphthous stomatitis, gingivitis and erythema nodusum are occasionally seen in patients with ulcerative colitis, but more frequently in Crohn's disease. A more common skin lesion in ulcerative colitis is pyoderma gangrenosum.

Abnormal liver function tests are found in approximately 15% of patients and may stem from a varitety of hepatic disorders. Up to 80% of patients with ulcerative colitis will have some abnormality of the liver on biopsy. The predominant lesions are a pericholangitis. Some 50 to 90% of patients will have a fatty infiltration of the liver. Of greater long-term concern is the development of sclerosing cholangitis which may be seen in 1 to 4% of patients with ulcerative colitis. Patients may have puritis and right upper quadrant pain with this condition. Although adults will manifest this with an elevation of alkaline phosphatase, caution must be taken in adolescents and children who normally have higher alkaline phosphatase levels. Diagnosis is made by endoscopic retrograde cholangiopancreatography. Sclerosisng cholangitis may persist after proctocolectomy, but will typically not occur if it has not developed before the patient's proctocolectomy. Anemia is common, usually the result of overt or occult blood loss in the stool. Osteoporosis and osteomalacia may occur from decreased calcium absorption associated with diminished uptake of fat-soluble vitamins and by increased urinary losses of calcium resulting from steroid therapy. Nephrolithiasis occurs in about 8% of patients, largely because of inadequate fluid intake to compensate for diarrheal losses, and increased oxylate absorption in the terminal ileum. Uveitis is an inflammation of the iris found in less than 2% of patients.

Children with mild ulcerative colitis or those who experience a period of remission may manifest few if any positive findings on examination, although sigmoidoscopy may demonstrate friable and edematous mucosa with a thin purulent exudate. These children often show evidence of delayed growth, lack of sexual maturation, anemia, pallor, and cushingoid features from chronic corticosteroid therapy. With more severe acute disease, children may develop fever, dehydration, and symptoms of systemic toxicity. Pain during palpation over the sigmoid colon is common. Although external hemorrhoids frequently develop from stool frequency, anal sinuses, fissures, and abscesses are much more indicative of Crohn's disease. On sigmoidoscopic examination, the mucosa is often edematous, hemorrhagic, and contains superficial ulcers. The mucosa is covered with a purulent, bloody exudate.

Anemia due to blood loss occurs in approximately two thirds of patients. The erythrocyte sedimentation rate is typically elevated, and the prothrombin time prolonged; the serum albumin level is usually low. Hyponatremia and hypokalemia may occur with protracted diarrhea. Stool cultures are consistently negative for pathogenic bacteria and parasites.

Although barium enema radiographs have been used for many years to establish the extent and severity of ulcerative colitis, most physicians recognize that better information can be obtained from flexible colonoscopy. Moreover, a contrast enema can precipitate the acute manifestations of colitis. When performed, the radiographic enema study may reveal a shortened, narrow, and rigid colon with loss of haustral folds and extensive pseudopolyp formation with chronic ulcerative colitis (Fig. 65-3). In acute colitis the bowel contour may have an irregular serrated border from mucosal ulcerations. The edematous mucosa between areas of ulceration appear as pseudopolyps. Swollen, inflamed mucosa can form symmetric defects along the borders, known as "thumb printing."

Differentiation of ulcerative colitis from other pathological is essential. An upper gastrointestinal series with small bowel follow through is critical, as Crohn's disease may often be confused with ulcerative colitis. Culturing of stool to rule out an infectious etiology is essential, as several organisms may mimic the symptoms of ulcerative colitis. Additionally, an accurate pathologic review of multiple biopsies from both colonoscopy, and if indicated upper endoscopy, is essential for the diagnosis. Nevertheless, up to 10% of patients may not be accurately assigned to either Crohn's or ulcerative colitis, and have a diagnosis of indeterminate colitis. To more accurately address the differentiation between these two disorders, a panel of serologic tests has been developed. These include anti-neutrophil cytoplasmic antibody (ANCA), with a perinuclear staining pattern being observed in ulcerative colitic patients (pANCA). Conversely, antibody to Saccharomyces cerevisiae (ASCA) had been identified in nearly 50% of patients with Crohn's disease. An additional antibody, anti-cathepsin G, has been identified in up to 63% of ulcerative colitis patients, and is particularly prevelant in those with active disease. Use of these antibodies has prompted a tremendous flurry of studies. Unfortunately, because of the relatively low predictive value, application of these studies is restricted to confirming a clinical and pathologic diagnosis at the present time. A potential use of these antibodies for the surgeon is in the 10% of patients who have the diagnosis of an indeterminate colitis, for whom surgery is contemplated. In these cases, although a fair amount of overlap exists, the use of these antibodies may help direct the surgical approach. Table 1 shows the relative frequency of these serologic markers in indeterminate colitis.

Nonoperative Treatment

Medical therapy for ulcerative colitis is nonspecific and is based on measures to provide symptomatic relief. At present, it is unlikely, however, that the ultimate course of the disease can be altered or a cure achieved by non-operative treatment. Medical management may be stratified based on the clinical severity of the disease process (Table 2). Patients with stable disease or who are in a remission may benefit from the use of 5-aminosalicylate-based compounds, such as sulfasalazine (Azulfidine, Proctor and Gamble, Cincinnati, OH). This is a mainstay for the treatment of patients with mild or moderate forms of the disease. The function of these drugs appears to be in the 5-aminosalicylate portion of the compound, which blocks the production of prostaglandins and leukotrienes. Additional actions include the blocking of bacterial peptide-induced neutrophil chemotaxis, adenosine-induced secretion and scavenging of reactive oxygen metabolites. Use of different derivatives of these compounds may direct therapy to a targeted site. This includes the use of a suppository or enema for proctitis, and oral compounds which are not broken down in the small bowel for targeting colonic tissue (Mesalamine enema, Rowasa enema or oral Pentasa).

For patients who have an acute exacerbation of their symptoms corticosteroid therapy is much more effective. Topical corticosteroids (Cort enemas) can be used for a severe, distal inflammatory process. Oral prednisone is used for severe ulcerative colitis, and intravenous dosing is given for those patients who are hospitalized. In general, patients will respond to steroids within 7 to 10 days. Steroid therapy should be given only as long as there is an acute inflammatory process. Tapering should be instituted, with a transition to other medications. While on corticosteroids, patients should receive an H-2 blocking agent. Additionally, side-effects of corticosteroids should be anticipated. These include growth failure, osteoporosis, hypertension, hyperglycemia and cushingoid features. Patients should also be placed on calcium, vitamin D and bisphosphonates.

Immunosuppressive therapy (azathioprine, 6-mercaptopurine, cyclosporine) has been advocated for patients with chronic or refractory ulcerative colitis. Azathioprine and mercaptopurine work on long-lived subgroups of T-cells which require a prolonged length of administration prior to their action taking effect. As such, these drugs should be started while the child is tapering off of corticosteroids. These drugs are toxic and monitoring of the white cell count is essential. Additionally, both drugs may cause pancreatitis and drug-induced hepatitis. The drugs are not only helpful in allowing steroid therapy to be tapered, but are also useful in maintaining remission. Cyclosporine works by preventing T-cell activation and by inhibition of IL-2 and IL-2 receptor expression. Its onset of action is much faster than azathioprine. It has been shown to have equal efficacy to corticosteroids in treating patients with severe ulcerative colitis. It is useful in severe steroid refractory disease. However, cyclosporine does have shortcomings in that it is highly immunosuppressive. Additionally, the success of the drug may not preclude future surgery. Approximately 30% to 70% of patients who have treated with cyclosporine for an acute exacerbation of ulcerative colitis will undergo a proctocolectomy within 6 months to a year.

Still somewhat controversial is the use of anti-tumor necrosis factor therapy (Infliximab, Centorcor, Malvern, PA). Although conventionally used in patients with Crohn's disease, a limited study of 17 adults has shown that 16 responded within 6 days, with a sustained response in 2 to 10 months. Colectomy was required in only one patient.

Psychotherapy may help the patient with chronic ulcerative colitis adjust to the disease, its complications, and its side effects. Even more important than psychotherapy is the ready availability of a sympathetic and interested physician as well as an understanding family on whom the patient can rely.

During acute flare-ups of ulcerative colitis, most patients require hospitalization with intravenous fluid administration, bowel rest, increased doses of steroids, and parenteral nutrition. These correct the patient's metabolic deficit and often reduce the clinical symptoms, yet often do not alter the course of the colitis. The primary benefit of intravenous nutrition is to reduce surgical risk by improving the patient's nitrogen balance. Progression of the colitis or failure to respond to therapy is an indication for urgent operation. When the acute attack subsides, the patient may begin consuming a bland high-calorie diet.

Antidiarrheal medications such as diphenoxylate hydrochloride with atropine sulfate (Lomotil) or loperamide hydrochloride (Imodium) may reduce the number of bowel movements and decrease rectal spasm, but they should be used with care since these drugs as well as opiates may occasionally induce toxic megacolon. Dietary modification may be useful to minimize intestinal stimulants; for example, elimination of chocolate, vinegar, spicy foods, fresh vegetables, and nuts. Marked anemia and hypoalbuminemia may necessitate blood and/or albumin infusion.

Operative Therapy

Ulcerative colitis can be cured by surgically removing the diseased colon and rectum. When this implied a permanent ileostomy, operation was often delayed until the patient was severely ill, the operative risk high, and the complication rate excessive. Since the development of the mucosal proctectomy and endorectal ileal pull-through procedure, serious consideration should be given to operation for any patient with chronic ulcerative colitis before severe disability and major complications develop.

Surgery in children who suffer with ulcerative colitis can be elective or emergent. Elective operation is performed on patients with chronic disease who experience continued symptoms despite medical therapy, growth retardation, severe limitation of activities, and an unacceptable quality of life. Emergency indications for operation include fulminant disease refractory to medical therapy, extensive rectal bleeding, and toxic megacolon. Careful monitoring and the use of steroids have reduced the number of operations in an emergency setting. If the indication for surgery is growth failure, the diseased colon should be removed while the epiphyses are still open to allow for growth and development. Evaluation of the child's condition should be made periodically during the course of therapy by the surgeon as well as the gastroenterologist to consider alternatives to long-term medical therapy.

Surgical options are discussed beforehand in detail with the patient and the parents. It is helpful if the patient speaks to another child of the same sex and similar age who has undergone surgery to alleviate fears and concerns about an ileostomy, and to support the decision for surgery. Preoperative discussion with an enterostomal therapist also helps to prepare the child and parents for an ileostomy. A short course of parenteral hyperalimentation is used if the patient is severely malnourished. Anemia, hypoalbuminemia, and electrolyte abnormalities are corrected preoperatively. Corticosteroid therapy is maintained to avoid an acute flare-up preoperatively. Oral intake is restricted to clear liquids for 48 hours before surgery. Cleansing enemas are avoided because they may precipitate an acute flare-up of colitis. Oral antibiotics are given on the day before the operation, and intravenous antibiotics are given preoperatively.

Complete proctocolectomy with permanent ileostomy is curative for ulcerative colitis and is done as a one-stage operation. It has been used with low morbidity and mortality for more than 50 years. However, a major concern of patients, particularly children, is the required lifetime ileostomy appliance. Although the care of an ileostomy is usually easily mastered by a child, the presence of a stoma often creates embarrassment during physical and social activities. Although post-operative impotence and/or bladder dysfunction after proctocolectomy in children are uncommon, these major concerns have caused many children, parents, and physicians to defer operation until severe debilitation by the colitis and/or steroid therapy causes irreversible systemic complications. Other operations less commonly employed include a subtotal colectomy with preservation of the rectum and an ileorectal anastomosis. Active disease in the rectum in most of these patients, require continued medical therapy and eventual surgical removal due to the risk of cancer. The Kock continent ileal reservoir with nipple valve construction obviates the need to wear an ileostomy drainage bag for most patients. Drainage is provided by inserting a silastic catheter into the reservoir several times daily. Complications related to stasis and distention from incomplete emptying, with chronic reservoir inflammation as well as incompetence of the nipple valve, leads to multiple reoperations and eventual removal in many patients. The procedure is currently used primarily for patients who already have a permanent ileostomy and who are severely handicapped by it.

Inasmuch as ulcerative colitis is primarily a disease of the mucosa, a modification of the rectal mucosal stripping procedure described by Soave in 1963 for treatment of Hirschsprung's disease has become the treatment of choice for both children and adults. Removal of the entire rectal mucosa down to the dentate line does not interfere appreciably with anorectal sphincter function or the ability to discriminate between gas and liquid or solid contents. Clinical use of the endorectal ileal pull-through procedure was not adopted until a successful outcome was reported in 15 of 17 children with a straight ileal pull-through operation.

Although numerous modifications of the endorectal pullthrough have been made during the ensuing years, it is now generally accepted as a highly desirable option for surgical treatment of ulcerative colitis and familial polyposis. Appealing features of this operation include absence of a permanent stoma, lack of repeated catheterization (as needed with a Kock pouch), and development of a near-normal pattern of defecation. Despite these advantages, patient expectations need to be addressed preoperatively. Stooling frequency is typically quite high initially regardless of whether a pouch is or is not utilized. Another problem is nocturnal incontinence. This latter problem is particularly prevalent in younger children, with the process resolving as the child matures. Finally, it is not uncommon for children to be on multiple medications to control stooling frequency; however, the majority of these patients eventually wean off most of these medications.

The approach to the operation involves either a straight pull-through or the creation of a pouch. Choice of either depends on the weighing of risks and benefits of each procedure. The pouch is associated with fewer bowel movements, particularly in the first year following the procedure. However, a pouch has the attendant risk of pouchitis which may occur in up to 50% of patients after 10 years following pullthrough. The straight pull-through avoids the risk of pouchiits, however, is associated with more frequent bowel movements in the first postoperative year. Regardless of the type of procedure performed, as long as the lower 4 cm of the rectal muscle is not damaged, the anal sphincter resting pressure and the anal sphincter squeeze pressure will approach normal values within 6 weeks.

Early surgical experience with pull-through operations indicated that a completely diverting, protecting ileostomy for approximately 2 months is advisable to minimize the risk of pelvic infection. Children with chronic ulcerative colitis who receive long-term steroids are often malnourished and frequently have a suppressed immune response, which increases the risk of anastomotic leak. In fact, the risk of such a leak has been found in approximately 10 to 15% of patients, and has the attendant risk of leading to anal stricturing.

Four basic reservoir types have been used clinically; the S-shaped reservoir, the J reservoir, the lateral isoperistaltic reservoir, and the W reservoir (Fig. 65-5). In general the W pouch and lateral isoperistaltic reservoirs have been abandoned. Both the S and the W reservoirs must be hand-sutured, requiring longer operating time than that needed for other pouches. The blood supply to the lower ileum of these two pouches may be partially obstructed by bending the mesentery, particularly if it is thick. Pouch stasis is common with these two reservoirs, and an irrigating catheter is often required for adequate emptying. After several months the reservoir tends to enlarge and the spout elongates. Thus when using the S-pouch it is critical to make the distal spout extremely short (1 to 1.5 cm in length).

The J-shaped reservoir, the most common pouch currently used, is usually constructed with a stapling instrument. A major advantage is the placement of the lower end of the reservoir close to the anus without a spout. The drawback is that it is sometimes difficult to bring the side of the ileum down to the anus without tension, particularly in heavy or tall patients. The S-pouch has the same overall mesenteric length as a straight pull-through. It is thus a very useful technique in those patients who desire a reservoir, but in whom adequate length cannot be achieved with a J-pouch.

Intravenous steroids are tapered rapidly after surgery, and oral prednisone can usually be discontinued within 3 weeks. Most children are discharged from the hospital by the seventh postoperative day. A water-soluble contrast enema is performed within the first 2 months to assure that the ileal reservoir has healed securely, and there are no leaks or sinus tracts. Most children resume full physical activities within 3 weeks.

Approximately 2 months subsequent to the first operation, the child is rehospitalized for ileostomy closure and sigmoidoscopy; the ileoanal anastomosis is dilated at this time.

Management of patients once the stoma has closed may be quite challenging. Once oral feedings are begun, it is important to initially withhold some foods which may cause excess stooling including chocolate, vinegar salad dressings, and spicy foods. Medical management will start with a combination of pectins in the form of Kaopectate and low doses of Imodium. Imodium dosing will increase as needed. Small amounts of fiber (Metamucil or Fibercon) may be given to increase fecal bulk for the first few weeks, if necessary. Occasional rectal examinations are performed to maintain the patency of the rectal anastomosis. Use of oral metronidazole may be quite helpful in controlling episodes of frequent stooling, because it leads to considerable bulking of the stool.

Results

Regardless of the type of pull-through, stool frequency and continence are quite similar in large series of patients followed for a number of years. Complications are multiple, and children need long-term follow up and care to attend to these problems.

Leakage from the anastomosis may occur in up to 20% of patients if there is no protective stoma. Although many of these will resolve with subsequent formation of a protective stoma, patients are left at increased risk for anastomotic stricture and potential problems with future continence. Thus, although inconvenient to the patient, it has been our practice to perform a diverting stoma in the majority of patients.

Despite the advantages of a stoma, several problems are associated with its use including retraction, parastomal hernias, dehydration and most commonly obstruction due to fecal material or excessive fascial tightness. Patients who present with signs of a bowel obstruction may often be relieved of the blockage with the passage of a large red rubber catheter through the proximal limb of the stoma.

Small bowel obstruction is a rather common problem with reported frequencies between 15% and 25%. Adhesions are the major cause of the obstruction, however, internal hernias, and outlet obstruction from the ileostomy are other common causes.

Pouchitis was first termed by Kock as an inflammatory state due to stasis within the reservoir. Incidence may be as high as 50% in patients followed for more than 10 years. Interestingly, those patients with a positive pANCA serologic marker are most at risk for the development of pouchitis. The process may relate to the original disease as pouchitis is infrequently seen in patients with familial polyposis. Symptoms include low-grade fever, pelvic pain, bloody stools, diarrhea and malaise. Treatment with antibiotics (metronidazole or ciprofloxacin) is usually successful. Occasionally, patients may benefit from steroid enemas. The use of probiotics may be beneficial in preventing reoccurrence of pouchitis once the patient is in remission. Recurrent pouchitis may actually be a manifestation of Crohn's disease and biopsies should be performed. It has become apparent that pouchitis is more common in larger reservoirs which empty only partially with each defecation. Although stenosis at the ileoanal anastomosis or lower end of the reservoir may appear to be a mild, annoying problem, it can cause reservoir distention, stasis, and pouchitis if not corrected early.

Fistulas from the pouch to the perianal skin or vagina appear related to technical problems at the time of the pullthrough. Fistulas formation may be seen in up to 4% to 7% of patients. Pouch loss due to multiple, often failed procedures, may be as high as 30%. Although technical problems may be a major causative factor, the possibility that the patient has Crohn's disease must be excluded by extensive biopsies of the pouch and more proximal bowel.

Other complications include the rare occurance of pouch perforation, erectile dysfunction, reduced fertility, and incorrect diagnosis. Temporary ileostomy may be needed in certain patients, particularly if growth and development during adolescent years are not progressing at optimum rates. After the reservoir reconstruction has been completed, and the child has resumed normal growth, the ileostomy may be safely closed.

Long-term follow-up must include proctosocpies every 2 to 3 years with biopsies of the retained 1 to 2 cm of rectal cuff to rule out malignancy.

Figure Legend

Figure 1. Complications of ulcerative colitis may be confined to the colon, or they may be systemic.

Figure 2. Barium enema radiograph showing shortened and narrow left-sided colon with absence of haustral folds, giving a "lead pipe" appearance. Also note the presence of pseudopolyps in the transverse colon.

Figure 3. A. Barium enema radiograph showing mucosal details of the transverse colon. Note the irregular mucosa with thumb printing.

B. Gross specimen of colon showing mucosal details. Note the extensive pseudopolyps over the entire surface.

C. Histologic appearance of the mucosa. Note the crypts are filled with neutrophils (crypt abscesses).

Figure 4. Ileal reservoir configurations in current clinical use for patients undergoing an endorectal ileal pull-through procedure. The length of the reservoir for the J-pouch is often much shorter in children (10-12cm).

This information is provided by the University of Michigan Department of Surgery, Section of Pediatric Surgery and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. For additional health information, please contact your health care provider or our offices.