Ulcerative colitis (UC) is an inflammatory bowel disease (IBD). IBD is a general term for noninfectious conditions that involve chronic inflammation of the intestines, causing symptoms such as diarrhea, abdominal pain, fevers, bleeding and growth problems.

Ulcerative colitis is limited to the colon and the rectum. In 80 percent of children, it affects the entire colon, but in some it is limited to the lower part and called left-sided colitis or proctitis. The inflammation does not affect the full thickness of intestine and only involves the inner lining of the colon and rectum. 

What causes ulcerative colitis?

The first symptom of ulcerative colitis is often blood in the stool or persistent and sometimes progressively worsening diarrhea. The bloody stool may be accompanied by abdominal pain, cramps and a severe urgency to have a bowel movement.

Other symptoms may include:

  • Anemia
  • Fatigue
  • Fever
  • Joint pain
  • Rectal bleeding
  • Skin or mouth lesions
  • Weight loss

The blood loss, chronic diarrhea, and inflammation can reduce the amount of nutrients absorbed from food and lead to stunted growth and delayed puberty in children with ulcerative colitis. The inflammation can be found in other parts of the body. Extra-intestinal manifestations can involve nearly any organ system—including the musculoskeletal, skin, liver and bile ducts, eyes, kidneys, and lungs.

Cause for the inflammation is unknown but can be hereditary. Children with UC can have a family history of inflammatory bowel disease; about 10-25 percent of people have a parent or sibling who have IBD.  

How is ulcerative colitis diagnosed?

Your child's doctor will first do a physical examination and take a medical history. There's no single test for diagnosing ulcerative colitis, so if the doctor suspects ulcerative colitis, a series of tests is required to make a definitive diagnosis. Tests may include:

  • Blood Tests — Blood tests check for anemia, which may be a sign of bleeding in the intestines. They can also detect a high white blood cell count or elevated levels of C-reactive protein, both markers of inflammation.
  • Stool Sample — Shows whether there is bleeding, inflammation, or infection in the intestines.
  • Magnetic Resonance Enterography — Provides detailed images of the small bowel that can pinpoint areas of inflammation, bleeding and other problems. Before the test, your child drinks a contrast material (sometimes just milk is enough) to highlight the small bowel in the images. MR enterography is noninvasive and does not involve radiation or expose your child to any radiation.
  • Colonoscopy — Allows the doctor to examine the entire length of the colon, using a long, flexible tube about as thick as your index finger with a tiny video camera and light on the end. Video images from the camera appear on a monitor. A colonoscopy is done under anesthesia in most children.
    As the colonoscope is pulled back out, the doctor gets even better views and can do a more careful examination. The colonoscope may also contain built-in instruments for taking tissue samples. Later, the doctor can further evaluate what's going on by examining these tiny biopsies of the lining (tissue) of the colon under a microscope.
  • Upper Endoscopy (aka esophagogastroduodenoscopy or EGD) — Enables the doctor to examine the upper GI tract, from the mouth through the esophagus, the stomach, and the first part of the intestine (as far as the duodenum and sometimes the first part of the jejunum). Like a colonoscopy, this procedure is usually done under anesthesia using a flexible tube with a tiny video camera and a light. Video images are shown on a monitor, and tissue samples can be taken for the doctor to examine later under a microscope.
  • Capsule Endoscopy (or PillCam) — No anesthesia or sedative is needed for this test. The child swallows a capsule containing a light and a camera, which takes pictures of the entire intestinal tract as it travels from the stomach to the colon and eventually out of the body in the stool – typically a four- to five-hour process. (Sometimes a test capsule is given first, to make sure there's no intestinal stricture that might block its passage.) The disadvantage of capsule endoscopy is that no tissue is obtained for later study under a microscope.
    Note: The capsules are quite large, and smaller children can't always swallow them. In that case, the capsule is put into the intestine during an upper endoscopy.

How is ulcerative colitis treated?

Treatment for ulcerative colitis varies depending on the seriousness of the disease. Most people need long-term medication to relieve symptoms and control the problems. In severe cases, surgery may be required to remove the diseased colon, which cures the disease.

Medications and Nutritional Therapies

Medications for ulcerative colitis may improve your child's quality of life by inducing and maintaining remission, or at least provide symptom-free periods. The four most commonly prescribed types of medications — aminosalicylates, corticosteroids, immunomodulators and biologic medicines — all work by reducing inflammation. Newer medications, such as biologics, are under investigation to see if they help children with ulcerative colitis.

Nutritional therapies are mainly used to supplement the diet and medications and are not effective alone.

Surgery

Most children who don't respond to medication will get relief from a surgical procedure known as restorative proctocolectomy and ileal pouch-anal anastomosis (IPAA). During the procedure, while your child is under general anesthesia, the surgeon removes the colon and the rectum, leaving a short residual rectal cuff near the external anal sphincter muscle complex and the anal canal. The end of the small intestine, called the ileum, is then pulled down to the pelvis and attached to the anal canal. The colon’s main function is to absorb water so after colectomy, your child will still be able to absorb all the nutrients need to grow and develop.  The main function of the rectum is to store stool so that a person does not have frequent bowel movements. After this operation, children can pass stool normally, although bowel movements may be more frequent and watery.  

This procedure can be done in one stage, two stages, or three stages.  Majority of children require three stages (three operations) because severe inflammation in the colon and rectum can cause malnutrition and patients are on strong medications that negatively affect the body’s ability to heal after operation (making one-stage or two-stage operation unsafe).  The first stage involves removal of the colon, leaving the rectum in pelvis, and pulling the end of the small intestine up through the abdominal wall and creating an ileostomy.  Stool comes out of the ileostomy (link to ileostomy webpage) and collects in the bag.  After your child recovers from this operation and regains their strength and weight and is overall in a healthy state, the second stage can occur at any time.  The second operation involves removal of the rectum from the pelvis, creating ileal pouch (to increase the capacity of the small intestine to hold stool), connecting the ileal pouch to the anal canal in the pelvis, and creating a diverting loop ileostomy to allow the new connection in the pelvis to heal.  About two months later, once the connection in the pelvis has healed, the final operation involves closing the ileostomy and allowing stool to pass through the anus again.  

Depending on the individual case, the UCSF pediatric surgeon usually perform the procedure laparoscopically, using tiny instruments and small, Band-Aid-size incisions. Patients who undergo laparoscopic procedures have less pain, a quicker recovery and a shorter hospital stay.

How long will my child be in the hospital?

The length of time your child stays in the hospital depends on which stage of the procedure is done. On average, your child will be in the hospital for about a week.  It is very important for the recovery for your child to get out of bed and walk the days after the operation.  Pain medications will be given to help minimize the discomfort; usually only acetaminophen and ibuprofen are needed when your child goes home.   After each operation, a dietitian will be available to provide education on the type of foods your child can eat.  Usually, your child will be started on low residue diet, but can reintroduce foods with fiber and return to normal diet within 2-4 weeks after the operation.  

What is expected after surgery?

Restorative proctocolectomy and ileal pouch-anal anastomosis can cure ulcerative colitis and change your child’s life for the better. However, there are some complications that may occur and will require your child to continue to see gastroenterologist for the remainder of his/her life.  After the operation, there is still a residual rim of distal rectal cuff near the external anal sphincter muscle complex.  This area may become inflamed and cause tenesmus (painful urgency to defecate), increased number of bowel movements, and blood in the stool.  In addition, the ileal pouch may also become inflamed and irritated, a condition called pouchitis, which has the same symptoms. Both these conditions can be managed with local medications.  Rarely, older patients can develop severe pouchitis or cuffitis or are discovered to have Crohn’s disease (a different form of IBD that affects the entire GI tract) and may need to have ileostomy recreated to treat the problem.  Our multidisciplinary IBD team at the UCSF Benioff Children’s Hospitals consists of pediatric surgeons, pediatric gastroenterologists, advanced practice providers (nurse practitioners and physician assistants), wound care specialists, and dietitians who are here to support and care for your child.