Conditions / Pediatric Ulcerative Colitis
Pediatric Ulcerative Colitis
Ulcerative colitis (UC) is a disease characterized by remitting and relapsing inflammation of the large intestine. UC, Crohn disease (CD) and indeterminate colitis account for the disorders that represent the inflammatory bowel diseases (IBDs).
Many patterns of presentation are possible within the pediatric age group. The hallmark symptoms of UC include abdominal cramping, diarrhea, and bloody stools, but physical symptoms vary with extent, duration, and severity of the disease. UC affects the rectum, with contiguous involvement that can include the entire large intestine. The disease phenotype can be characterized according to the Paris Classification, which divides the disease into isolated proctitis, left-sided colitis, extended colitis, and pancolitis.  Extraintestinal manifestations of UC, such as joint pain, ophthalmic conditions, and hepatobiliary disease may occur in some patients. See Presentation.
In the United States, 2 of every 100,000 children (age’s 10-19 y) are affected, and 20-25% of all cases UC occur in persons aged 20 years or younger and the disease is increasing among young children (< 5 y).
Colonoscopy with biopsy is the most valuable procedure in evaluating patients with inflammatory bowel disease. Typical findings in someone with UC are inflammation that is first evident in the rectum and that proximally extends in a contiguous fashion.
The therapeutic goal is to gain clinical and laboratory control of the disease with minimal adverse effects while permitting the patient to function as normally as possible. Approximately 5-10% of patients with UC require acute surgical intervention because of fulminant colitis refractory to medical therapy. Total proctocolectomy is often curative in patients with UC and removes the risk of colonic adenocarcinoma.
The mainstay of outpatient management is anti-inflammatory therapy with 5-aminosalicylic acid (5-ASA) preparations, such as sulfasalazine and mesalamine. Acute flares of UC in the pediatric population tend to respond well to corticosteroids, but numerous adverse effects prevent long-term use. Immunomodulatory agents and tumor necrosis factor inhibitors are also used to alleviate exacerbations of disease and increase patient comfort, as are newer therapies (eg, vedolizumab), and they can be a viable treatment option when the patient is steroid-dependent or steroid-refractory.
In the United States, 2 of every 100,000 children (age’s 10-19 y) develop ulcerative colitis (UC), and 20-30% of all cases occur in persons aged 20 years or younger. In most studies, UC incidence peaks between adolescence and early adulthood (ie, in people aged 15-30 y). A smaller peak occurs in patients aged 60-80 years. The incidence of IBD among young children (< 5 y) has increased. These children often have disease that is limited to the colon; however, the disease can later progress to involve other parts of the intestine.
Although many children with UC have a mild course and respond well to therapy, severe complications can arise in patients with ulcerative colitis during acute exacerbations of disease or as the disease progresses.
Toxic megacolon is the most serious acute complication of ulcerative colitis and is reported to occur in up to 5% of patients; it is rare in young patients. Toxic megacolon is considered a medical and surgical emergency.
The pathogenesis of toxic megacolon is related to severe inflammation resulting in disordered intestinal motility. Compromised mucosal integrity then may allow bacteria to enter the submucosal tissues, leading to necrosis and peritonitis. Absorptive function is also impaired, resulting in increased luminal fluid volume and electrolyte losses.
Toxic megacolon usually occurs in the presence of severe pancolitis. Concurrent infection, such as Clostridium difficile colitis can lead to toxic megacolon; use of antidiarrheal agents or recent barium enema study or colonoscopy have also been implicated as causes. In addition, metabolic abnormalities (eg, hypokalemia, hypomagnesemia, hypoproteinemia), impaired epithelial integrity of the colon, and altered motor function and are frequently found in patients with toxic megacolon.
Toxic megacolon is associated with fever, abdominal distention, and tenderness. Abdominal obstruction series reveals dilatation of the colon with loss of normal haustral markings and signs of edema. Toxic megacolon places the patient at risk for colonic perforation, gram-negative sepsis, and massive hemorrhage.
Colonic malignancy is a clinically significant complication in patients with ulcerative colitis. Disease duration and pancolitis are well-recognized risk factors for malignancy, with the cancer risk surpassing that of the general population after 10 years. Other less-characterized risk factors include sclerosing cholangitis, a bypassed and defunctionalized segment of bowel, and a low folate level.
Children who develop UC before age 14 years have a cumulative colorectal-cancer incidence of 5% at age 20 years and 40% at age 35 years. Patients aged 15-39 years who develop ulcerative colitis have a cumulative incidence of 5% at age 20 years and 30% at age 35 years. The risk for children with disease onset in the first decade of life is unknown, but these children should undergo colonoscopic screening for dysplasia beginning in adolescence.
Epithelial dysplasia generally precedes carcinoma; therefore, yearly screening with surveillance colonoscopy and biopsy should be performed. Dysplasia can be missed on surveillance biopsy; prophylactic colectomy should be considered for adults who developed UC during childhood. With this in mind, psychologically prepare adolescents and young adults by discussing surgical options before the need for surgery arises.
Extraintestinal manifestations are common in ulcerative colitis (UC); approximately 25-35% of patients with inflammatory bowel disease (IBD) have at least one extraintestinal manifestation. Extraintestinal disease may be prognostically important because the rate of pouchitis increases after colectomy in patients with UC and extraintestinal manifestations.
Pyoderma gangrenosum occurs in 1% of UC patients. An indolent chronic ulcer may occur even when disease is in remission. Intralesional therapy with steroids is useful, and colectomy results in healing in approximately 50% of patients.
Ophthalmologic manifestations most frequently occur when the disease is active. The incidence in adults is 4% but is less in children. The most common findings are episcleritis and anterior uveitis. Uveitis is usually symptomatic, causing pain or decreased vision. Patients with IBD should likely undergo routine ophthalmologic examination.
Arthritis is the most common extraintestinal manifestation of IBD, occurring in 10-25% of adolescents. The arthritis is usually a transient, nondeforming synovitis that involves the large joints in an asymmetric distribution. In children, arthritis may precede gastrointestinal (GI) symptoms by years.
Hepatobiliary disease is another common extraintestinal manifestation of UC in children. Hepatobiliary complications may precede the onset of GI symptoms, they may accompany active disease, or they may develop after surgical resection. Chronic active hepatitis, granulomatous hepatitis, amyloidosis, fatty liver, and pericholangitis are some of the intrahepatic manifestations of IBD. Extrahepatic manifestations include cholelithiasis and primary sclerosing cholangitis (PSC).
Thromboembolic disease is considered to be the result of a hypercoagulable state that parallels disease activity and is manifested by thrombocytosis; elevated plasma fibrinogen, factor V, and factor VIII; and decreased plasma antithrombin III. The hypercoagulable state may lead to deep venous thrombosis, pulmonary emboli, and neurovascular disease.
Thorough education about the pathophysiology, medications, and short-term and long-term risks of ulcerative colitis is an essential part of any treatment program.
The Crohn’s and Colitis Foundation of America and the Crohn’s and Colitis Foundation of Canada are nonprofit organizations dedicated to the education and treatment of patients affected by Crohn disease and UC.
The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition, in concert with the Children’s Digestive Health and Nutrition Foundation, have developed educational resources for children and families affected by IBD. These resources are available at Kids IBD.
For patient education information, see eMedicineHealth’s Digestive Disorders Center, as well as Crohn Disease in Children and Teens, Crohn Disease, and Inflammatory Bowel Disease.
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Pediatric Ulcerative Colitis