Background
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. [1] 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). See Epidemiology.
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. See Workup.
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. See Treatment.
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. See Medication.
For information on the general pathophysiology, etiology, and prognosis in patients with ulcerative colitis, see Ulcerative Colitis.
Epidemiology
The incidence of pediatric-onset ulcerative colitis (UC) appears to be on the rise, often with a more severe disease presentation. [2] The prevalence of inflammatory bowel disease (IBD) overall increased in this population by 133% over a 9-year period (from 33.0/100,000 in 2007 to 77.0/100,000 in 2016). Among children, Crohn disease (CD) was twice as prevalent as UC (45.9 vs 21.6). Boys are affected more than girls for all forms of IBD. [2]
In the United States, 2-4 of every 100,000 children (age's 10-19 y) develop 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 inflammatory bowel disease 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.
Prognosis
Although many children with ulcerative colitis (UC) have a mild course and respond well to therapy, severe complications can arise in patients with UC during acute exacerbations of disease or as the disease progresses. Male sex and higher Pediatric Ulcerative Colitis Activity Index (PUCAI) score at diagnosis appear to be independent risk factors for colectomy in children with UC. [3]
A 2021 systematic review showed that at diagnosis, disease extent, PUCAI score (>65 points), hemoglobin and hematocrit levels, and white blood cell measurements (WBCs) may predict colectomy; PUCAI score >65 during the subsequent 3 months, family history of UC, and extraintestinal manifestations may predict colectomy. [4]
Factors predictive for the development of chronic pouchitis in pediatric ulcerative colitis are use of immunomodulators and the early occurrence of the first episode of pouchitis within 15 months after ileal pouch-anal anastomosis (IPAA). [5]
Toxic megacolon
Toxic megacolon is the most serious acute complication of UC 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 series reveal 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
Colonic malignancy is a clinically significant complication in patients with UC. 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 UC 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
Extraintestinal manifestations are common in 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. In an 11-year (2009-2020) population-based nested case-control study of Canadian children with IBD, 15 venous thromboembolic events (VTEs) (1.69%) occurred among 12 patients (12 with UC; 3 with Crohn disease). [6] Of children who developed VTE, females and those with with UC were disproportionately affected. Overall, the prognosis was good in those whose VTE were treated with anticoagulation therapy (there were no deaths or increased bleeding events reported). [6]
Patient Education
Thorough education about the pathophysiology, medications, and short-term and long-term risks of ulcerative colitis (UC) is an essential part of any treatment program.
The Crohn's and Colitis Foundation of America and Crohn's and Colitis 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 inflammatory bowel disease (IBD). These resources are available at GI Kids.
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Ulcerative Colitis in Children. Ulcerative colitis. This specimen from a colectomy reveals diffusely hemorrhagic granular mucosa in a continuous distribution.
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Ulcerative Colitis in Children. Histologic section: Note the diffuse inflammatory process, limited to mucosa and superficial portion of the submucosa (full thickness biopsy, staining, magnification).
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Ulcerative Colitis in Children. Histologic section: Note the diffuse inflammatory process, limited to mucosa and superficial portion of the submucosa (full thickness biopsy, staining, magnification).
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- Overview
- Presentation
- DDx
- Workup
- Treatment
- Guidelines
- ASCRS Clinical Practice Guidelines on Ulcerative Colitis (2021)
- WSES-AAST Inflammatory Bowel Disease Emergency Management Clinical Practice Guidelines (2021)
- ECCO Clinical Practice Guidelines on Infections in Inflammatory Bowel Disease (2021)
- AGA Guidelines on the Management of Mild-to-Moderate UC (2019)
- ECCO/ESPGHN Guideline Management of Pediatric Ulcerative Colitis (2018)
- ACG Guidelines on Preventive Care in IBD (2017)
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- Medication
- Questions & Answers
- Media Gallery
- References
