J-pouch Surgery

J-pouch surgery is a common name for a multi-step operation in which a surgeon removes the colon and rectum (both part of the large intestine), and creates a J-shaped pouch to collect waste. The pouch is stitched into place just inside the anus.

The official name for this surgery is as total proctocolectomy with ileal pouch-anal anastomosis (IPAA). The name comes from the fact that the pouch is created by taking the small intestine (the ileum), folding it back on itself in the shape of a letter J, and then using surgical stapling to create a small reservoir that functions like a new rectum.

The procedure may be offered to patients with ulcerative colitis who fail to respond to medications, or patients with familiar adenomatous polyposis (FAP).

Most children with ulcerative colitis have an excellent quality of life and do well with modern medical therapy. However, some will continue to have symptoms or serious complications from their disease despite medication and changes in their diet. For these patients, surgery may be considered as a treatment option. The decision to proceed with a major operation such as J-pouch surgery requires careful consideration of all the intended benefits and potential risks, and is made in collaboration between the patient and their family, a gastrointestinal (GI) specialist, and a pediatric surgeon.

Patients with familial adenomatous polyposis have a hereditary condition that causes polyps to form in the colon. If the polyps are relatively few, the patient can be followed with routine studies such as a colonoscopy and biopsy to rule out any signs of colon cancer. However, some patients with FAP develop a large number of polyps that cannot be safely monitored – even with frequent imaging studies. For these patients, surgical treatment may be recommended. Clinicians will discuss the risks and benefits of surgical treatment with the patient and their family.

In summary, J-pouch surgery may be considered a treatment option for:

• Patients with ulcerative colitis for whom medical management is no longer effective in relieving symptoms, to treat a complication of the disease, or to promote growth and weight gain
• Patients with FAP who have developed too many polyps to safely monitor, and are at-risk of developing colorectal cancer.

Although surgery to remove part or most of the colon is sometimes necessary, J-pouch surgery is generally not recommended for patients with Crohn's disease, perianal disease, small bowel disease, or disease outside the colon or rectum.

Clinicians will work closely with your family to determine if surgery is necessary and which procedure is most appropriate. Our surgeons are experts who specialize in GI-related procedures and colorectal surgery, and will work with your child's specialists to optimize your child’s quality of life.

Before J-pouch surgery, your child's doctor's will give you details about:

• What to expect during surgery
• What special preparations are needed before surgery
• Which tests may be performed the day of surgery
• What happens during surgery, possible reactions and side effects
• How long the surgery is expected to last
• What to expect immediately after surgery and long term

In most cases, your child will report to the hospital on the day of surgery.

J-pouch surgery is complex, but it is done frequently at CHOP with consistent, positive results. Our gastroenterology and general surgery teams (and oncology, if the child has FAP) work together to determine when surgery is needed and which procedure will produce the best results for your child. Our GI-Surgery team is among the best in the world, and have repeatedly been ranked No. 1 by U.S. News & World Report.

Before surgery, your child will be given medication to help them remain calm and pain-free during the procedure. Parents may remain with their child before surgery, and will be able to see them immediately after surgery.

At CHOP, J-pouch surgery for ulcerative colitis is usually performed in two stages, which includes two distinct operations a few months apart. The first operation is to remove the colon (subtotal colectomy) and create a temporary ileostomy (similar to a colostomy). In most cases, the first operation is performed laparoscopically and involves: making a few small incisions on the child's abdomen, using a laparoscope to "see" inside the abdominal cavity, and using long, thin surgical tools to help remove the colon.

After removing the colon (the rectum is left in place), an ileostomy is created in the lower right portion of the abdomen. After a few months — during which most patients feel better, wean off their medication, and gain weight — the patient returns to CHOP for a second operation to create the J-pouch and, in most cases, close the ileostomy.

Patients with FAP can usually have J-pouch surgery safely performed in one operation because they are usually otherwise healthy, well-nourished and not on powerful anti-inflammatory medications. Only rarely do patients with FAP need to have a temporary ileostomy placed and a second surgery performed.

After the first stage of J-pouch surgery, your child's body will need time to recover and adapt to life with an ostomy bag. Ileostomies are not painful and most children can resume a normal lifestyle, including school, games and some sports. Your child's healthcare team will instruct you and your child about how to manage the temporary ostomy and keep it clean. In most cases, the ostomy will remain in place for two to three months as your child's internal organs heal.

In rare cases, before reconnecting the bowel to the J-pouch, a contrast radiographic study may be performed to ensure the J-pouch has healed properly and it is safe to reconnect the bowel. The radiographic study is performed in preparation for the second stage of J-pouch surgery.

The second surgery reverses the temporary ostomy and reconnects the child's intestine to the J-pouch. The J-pouch functions like the rectum, allowing stool to pass through the anus as a bowel movement. All patients will experience an increased number of bowel movements that will gradually decrease with time, typically 3-4 times daily and occasionally at night. The vast majority of patients will have good control of their stools and are able to hold it long enough to find a bathroom. We encourage patients to engage in school and normal activities with courage and confidence.

After J-pouch surgery, most children are hospitalized for approximately 5-7 days to allow the body to heal. They are given medication to ease pain, and the healthcare team will show you and your child how to care for the wounds and the ostomy. Your child will be able to drink liquids and eat foods shortly after surgery.

The decision to discharge the patient to home is based on standard protocols and reaching certain milestones including:

• Eating and tolerating a regular diet
• Walking without assistance
• Being relatively comfortable with oral pain medicine
• Have a normal temperature (no fever)
• Having consistent bowel movements

Once released, children recovering from J-pouch surgery should rest at home and gradually resume normal activities over 2-3 weeks. Even after normal activity resumes, do not allow your child to participate in sports or lift more than about 10 pounds until they are cleared by the doctor at the routine follow-up visit. In most cases, children will be able to resume normal activities within 3-4 weeks after surgery.

Although J-pouch surgery is very successful in treating ulcerative colitis and FAP, complications can occur that require follow-up treatment. The most common complications include:

• Rectal stricture, which is a slight narrowing where the pouch is attached to the anus. Rectal stricture is easily treated with an anal dilation under general anesthesia in the operating room.
• Pouchitis, an inflammation of the J-pouch, which usually occurs within the first two years of placement, but can occur at any time. Symptoms of pouchitis may include: diarrhea, bleeding, abdominal pain, increased bowel movements, dehydration or fever. In most cases, pouchitis can be successfully treated with antibiotics or a minor surgical procedure. In very rare cases, an Ileostomy may be needed.
• Small bowel obstruction, which occurs due to bands of scar tissue (adhesions) that can develop between tissues and organs in the abdomen because of any operation. Symptoms include nausea, vomiting (typically bright green), abdominal pain, or the inability to pass gas or stool. In most cases, these obstructions can be treated with bowel rest and intravenous fluids during a brief hospital stay, but in some cases, an operation is needed to remove the adhesions and relieve the blockage.

Families should seek immediate medical attention if they suspect their child has one of these complications after J-pouch surgery.

Most children who undergo J-pouch surgery have good functional results including 3-4 bowel movements daily, with good control and few problems. Most enjoy a long-term improvement in symptoms, nutrition and quality of life. Most patients can attend school, enjoy activities and live a normal life following J-pouch surgery.

Reviewed by Peter Mattei, MD, FACS, FAAP, Andrew B. Grossman, MD, and Danielle Karasko, MSN, BSN, CRNP-AC