Division of Pediatric General, Thoracic and Fetal Surgery

What is Crohn's disease?
What are the symptoms of Crohn's disease?
How is Crohn's disease diagnosed?
How is Crohn's disease treated?
When is surgery used to treat Crohn's disease?
What operations for Crohn's disease are offered at CHOP?
Resources for families

Crohn's Disease

What is Crohn’s disease?

Crohn’s disease is a chronic inflammatory disease of the bowel. It can affect any part of the digestive tract from the mouth to the anus, but most commonly affects the terminal ileum (the lowest part of the small intestine), the colon, or the rectum. The disease results in inflammation of the intestine, including thickening of the wall of the intestine and deep ulcers in the lining of the bowel. Over time, this can lead to areas of narrowing, called strictures, and full-thickness perforations, called fistulas.

The cause of Crohn’s disease is unknown, but it is believed that genetic, immunologic and environmental factors are involved. While dietary factors and stress do not cause Crohn’s disease, some patients report that these factors seem to make the condition worse or cause it to flare.

Crohn’s disease can affect people of any age, but it most commonly occurs in adolescents and young adults between the ages of 15 and 35. Males and females are affected equally. It is estimated that up to 20% of people with Crohn’s disease have a family member who also has an inflammatory bowel condition. There appears to be an increased incidence of Crohn’s disease in those of eastern European descent, those who live in urban areas, and those in northern climates.

What are the symptoms of Crohn’s disease?

The clinical signs of Crohn’s disease can vary from patient to patient. The most common symptoms and signs include some combination of chronic and recurrent abdominal pain, diarrhea, blood in the stool, or weight loss. Some patients present with skin rashes, joint pain, fatigue, or delayed puberty. Patients with predominantly rectal or perianal Crohn’s disease may present with rectal bleeding, anal fissures and skin tags, or perirectal abscess. The symptoms of Crohn’s disease can be nonspecific and mild at first, which makes it difficult to diagnose early on. In fact, the typical patient with Crohn’s disease has symptoms for more than a year before the disease is confirmed.

How is Crohn’s disease diagnosed?

A gastroenterologist will diagnose Crohn’s disease using a variety of tests, including blood work, imaging studies and endoscopy with biopsies. The diagnosis can be difficult to confirm at first. It is also sometimes difficult to distinguish Crohn’s disease from ulcerative colitis, the other common type of inflammatory bowel disease.

How is Crohn’s disease treated?

Although Crohn’s disease is not curable, the disease can usually be controlled with medication. For patients with Crohn’s disease, the goals of treatment are to control inflammation, relieve symptoms, and improve nutrition. Primary treatment includes medications and supplemental nutrition. The medications most often used in the treatment of Crohn’s disease are anti-inflammatory drugs, corticosteroids, antibiotics and drugs that alter the immune system. Surgery is considered for patients who have developed a complication of the disease, or for whom medications are no longer effective.

When is surgery used to treat Crohn’s disease?

Surgery is used in conjunction with medical management to relieve symptoms and achieve conditions that are optimal for growth and weight gain. Surgery is generally considered an option of last resort. The decision to proceed with surgical intervention is made in collaboration with the patient and his family, gastroenterologist and surgeon.

Examples of patients for whom medical management is no longer effective include:

• The teenager with Crohn’s disease who has stopped growing or not started puberty when expected
• The patient who has pain or other symptoms when he is weaned from oral corticosteroids
• Any patient who has chronic or daily symptoms and difficulty gaining weight

Surgical intervention is also considered for patients who have developed complications due to their Crohn’s disease, such as:

• Bowel stricture (a narrowing) or actual obstruction
• Bowel perforation
• Fistula (an abnormal connection from the intestine to another part of the bowel or another organ)
• Intra-abdominal abscess (pockets of infected fluid)

Preoperative evaluation

Your child’s gastroenterologist will recommend consultation with a surgeon when they feel surgical intervention might be necessary. The surgical consultation consists of a thorough health history and physical exam. A detailed history of medications including steroid use will be reviewed. We will review your child’s growth chart, blood work, and other diagnostic tests, including radiologic examinations such as upper GI, MRI or CT scan.

What operations for Crohn’s disease are offered at the Children’s Hospital of Philadelphia?

1. Bowel resection: Crohn’s disease causes inflammation of the digestive tract, which can affect the overall thickness of the bowel wall. This inflammation often occurs in segments, with portions of normal bowel between diseased portions of bowel. By removing the diseased segment of the bowel, one can often achieve a period of remission, in which the symptoms are minimal and the need for medications is limited. This period of remission may also lead to the start of puberty and allow for a growth spurt in children suffering from Crohn’s disease.
   
   

   Bowel resection involves removing the diseased segment of bowel and reattaching the two ends of healthy bowel. Resections can be performed using a laparoscopic-assisted approach or a standard open technique. Most of the time, a primary anastomosis (a new connection in the bowel) can be performed. Ileostomy (similar to a colostomy bag) is only very rarely needed (and always only if there is no other safe option).

   After undergoing a bowel resection, your child will be admitted to the hospital. The length of hospital stay depends upon the return of your child’s bowel function. The CHOP surgical team is often able to avoid use of a nasogastric tube (placed through the nose into the stomach to allow for bowel rest) at the time of a bowel resection. Your child may be allowed to start drinking fluids right after the surgery, depending on the physical examination findings.

   Your child’s diet will be advanced as tolerated. Initially, intravenous pain medication will be given, but soon after your child begins to eat we will transition your child to an oral pain medication. Your child will be discharged when he is eating, drinking, comfortable on oral pain medications, and has had a bowel movement.

2. Examination under anesthesia: Patients with rectal or perianal Crohn’s disease can have fissures with or without skin tags, fistulas with or without perirectal abscess, or rectal strictures, all of which are difficult to assess comfortably in the exam room. To ensure your child’s comfort, it is often advisable to perform a thorough examination in the operating room with your child under general anesthesia. Sometimes an MRI is performed to provide supplementary information before the examination.
   
   

   During an exam under anesthesia (EUA), the surgeon can relieve symptoms, control infection, or stabilize the inflammatory process while waiting for medical management to be effective. Surgical maneuvers that can be conducted during an EUA include:

   • Incision and drainage of perirectal abscess
   • Placement of a drain or silk thread (known as a seton) to help control a fistula
   • Dilatation of a rectal stricture

   Due to the extremely poor wound healing patients with Crohn’s disease often have in the perianal region, it is usually advisable for the surgeon not to excise fissures, remove anal skin tags, or perform fistulectomy or fistulotomy.

   Anal and rectal procedures are typically done on an outpatient basis, meaning your child will not require an overnight hospital stay. In certain circumstances, however, an overnight stay may be recommended.

3. Placement of a seton: When Crohn’s disease results in a rectal fistula, a seton (pronounced see-tahn) may be used to promote healing by allowing the tract to drain both internally (in the rectum) and externally (at the skin level). A seton is a suture or drain that is placed within the fistula tract so that it cannot close at either end, thus preventing the accumulation of fluid and subsequent infection. The procedure to place the seton is done at the time of an examination under anesthesia. The seton will remain in place for anywhere from a few weeks to months, and it requires no special care – they are painless and usually fall out on their own within six months to a year of being placed.

Although there is no operation that will cure a patient of Crohn’s disease, surgery is often helpful in patients with complications of the disease and when medical management is no longer effective. It can result in a period of remission that can last years. Although it is true that patients with Crohn’s disease who require surgery are at slightly higher risk for requiring surgery again sometime in the future, this is due to the nature of their disease and not the operation itself.

While surgical treatment for Crohn’s disease should be considered an option of last resort, when performed before a surgical emergency develops it can often be performed using minimally invasive techniques and with minimal risk of complications or adverse consequences.

Resources for families

• The Center for Pediatric Inflammatory Bowel Disease at The Children's Hospital of Philadelphia
• The Crohn's & Colitis Foundation of America (CCFA)

To make an appointment to have your child evaluated, please contact the Division of Pediatric General Thoracic and Fetal Surgery at 215-590-2730.

Created by: Mary Kate Klarich, MSN, CRNP and Natalie Walker, MSN, CRNP
Reviewed by: Peter Mattei, MD, FACS, FAAP
September 2012