Ulcerative Colitis in Children Clinical Presentation

Updated: Jul 26, 2019
  • Author: Judith R Kelsen, MD; Chief Editor: Carmen Cuffari, MD  more...
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Presentation

History

Ulcerative colitis (UC) is a diffuse mucosal inflammation limited to the colon that affects the rectum; it may extend proximally in a symmetric uninterrupted pattern involving only some of or the entire large intestine. The most common presenting symptoms are rectal bleeding, diarrhea, and abdominal pain. Many patterns of presentation occur in the pediatric age group.

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]  Meta-analyses have shown that at diagnosis, 28% of children have proctitis (E1), 35% had left-sided colitis (E2), and 37% had extensive colitis (E3). At follow up (median, 6.7 y), extension of disease was seen in 49% of cases, with 60% of patients demonstrating extensive colitis. 

Mild disease is observed in 50-60% of patients. This presentation involves insidious onset of diarrhea, later associated with hematochezia. No systemic findings of fever, weight loss, or hypoalbuminemia are observed. Mild UC is typically confined to the distal colon and responds well to therapy.

Moderate disease is observed in 30% of patients and is characterized by bloody diarrhea, cramps, urgency to defecate, and abdominal tenderness. Associated systemic findings, such as anorexia, weight loss, low-grade fever, and mild anemia, are present.

Severe disease occurs in approximately 10% of patients. This presentation involves more than 6 bloody stools per day, abdominal tenderness, fever, anemia, leukocytosis, and hypoalbuminemia. Patients with severe colitis may experience life-threatening complications, including severe hemorrhage, toxic megacolon, or intestinal perforation.

Less than 5% of children with UC present with predominantly extraintestinal manifestations, such as growth failure, arthropathy, skin manifestations, or liver disease.

A clinical scoring system known as the Pediatric Ulcerative Colitis Activity Index (PUCAI) has been developed and validated. [5] The PUCAI may be used to determine when it is necessary to escalate therapy in fulminant colitis. It is also used to assess disease activity in clinical trials involving pediatric patients with UC.

For more information about the general clinical approach to UC, see Ulcerative Colitis.

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Physical Examination

The findings on physical examination in ulcerative colitis (UC) vary with extent, duration, and severity of the disease. In addition to abdominal signs, many extraintestinal manifestations of UC may become evident during physical examination.

Vital signs may indicate fever. Tachycardia may represent anemia or hypovolemia. Tachypnea may be present because of abdominal splinting or as a compensatory mechanism for acidosis in cases of severe dehydration.

Comparison with growth charts may reveal delayed growth. Cushingoid appearance is indicative of steroid use, usually over a long period. The patient may appear toxic in cases of fulminant disease. Long-standing and severe disease may cause signs of malnutrition, such as muscle wasting.

Abdominal examination findings may be normal, but abdominal tenderness is likely. Voluntary or involuntary guarding may be present. Bowel sounds may be normal, hyperactive, or hypoactive; rushes or high-pitched tinkling may be found in cases of obstruction.

Rebound tenderness indicates severe disease and possible perforation. A palpable mass may indicate obstruction or megacolon. An enlarged spleen may be indicative of portal hypertension from associated autoimmune hepatitis or primary sclerosing cholangitis. Long-standing disease can be associated with colonic stricture and evidence for bowel obstruction.

Skin examination may reveal pallor in cases of anemia, decreased skin turgor in cases of dehydration, and jaundice, caput medusae, or spider angiomata when associated liver involvement is present. Erythema nodosum may be evident on extensor surfaces (more common in Crohn disease [CD] than UC); pyoderma gangrenosum affects approximately 1% of UC patients.

Patients with episcleritis can present with a painful erythematous eye. Cataracts may occur in patients with significant steroid history. Scleral icterus may be indicative of liver disease.

Joint pain (arthralgia) is a common finding in inflammatory bowel disease, although swollen or red joints (arthritis) occur less frequently. The large peripheral joints, such as the knees, ankles, wrists, and elbows, are most commonly involved, but any joint can be involved. Approximately 1% of UC patients develop ankylosing spondylitis; most of these patients test positive for human leukocyte antigen (HLA) type B27.

Unlike what is seen in some CD patients, perianal examination in UC patients should not reveal any evidence of fistulae or abscesses; chronic diarrhea may lead to perianal erythema, fissuring, or hemorrhoids, however.

Sexual development may be delayed in patients with UC, but this finding is more common in patients with CD.

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