Pediatric Total Proctocolectomy with J-Pouch Reconstruction

Ulcerative Colitis (UC)

Patients with ulcerative colitis that do not get better with medicine therapy may benefit from total proctocolectomy. For patients with UC, surgery is usually done in three stages.

Familial Adenomatous Polyposis (FAP)

In patients with FAP, a total proctocolectomy can prevent colon cancer. In patients with FAP, surgery is usually done in two stages, combining the first and second procedures.

Stage 1: Colectomy with End Ileostomy

In the first stage, the colon is removed, and the rectum is left in place. This procedure is a laparoscopic-assisted procedure, where your doctor will use small incisions to decrease pain and help your child heal faster. A temporary ileostomy is created using the ileum (the last part of the small intestine) which will give the intestines and body time to heal. An ileostomy creates a small opening in the abdominal (belly) wall where part of the small intestine is brought to the skin's surface to allow drainage of stool.

Stage 2: Proctectomy and J-pouch Creation

In the second stage of surgery, the rectum is removed, and the ileum (small intestine) is connected to the anus, which is called the J-pouch. It looks like a J. The J-pouch holds stool and allows the child to pass stool through the anus in a normal manner. Bowel movements may be more frequent and more watery than usual. The connection (anastomosis) between the J-pouch and anus is important and needs time to heal. To help the connection heal, a new temporary ostomy, called a loop ileostomy, is created. The loop ileostomy will be in the same place as the previous ileostomy.

Stage 3: Takedown “Removal” of the Loop Ileostomy

In the third stage of surgery; when the J-pouch has healed, the loop ileostomy is reversed, and the small intestine is reconnected to the pouch. After this surgery, your child will begin stooling from their bottom.

• Before surgery, we will follow ERAS (enhanced recovery after surgery) protocols to improve your child’s recovery and reduce their length of stay in the hospital. Please see the ERAS booklet for more information.   
• Diet: Your child will be on a clear liquid diet the day before surgery, we also ask that your child drink a carbohydrate-rich drink, such as Gatorade, until two hours prior to surgery.  
• You may meet with the wound/ostomy team before surgery for stoma marking. During this appointment, the team will talk with you about what to expect with an ostomy. They will also find the best location for the ostomy on your child’s skin. This helps your surgeon place the ostomy during the surgery. 

Stage 1: Colectomy with End Ileostomy

• Operation: Stage 1 takes about three to five hours in the operating room.   
• Hospital stay: After surgery, expect to stay in the hospital for three to five days. This helps your child heal and learn how to care for their new ostomy. During the admission, you will meet with the wound/ostomy team, who will teach you how to care for the ostomy and make sure you are comfortable.   
• Discharging home: When your child is tolerating a regular diet, pain is controlled with over-the-counter medications, they are walking, ostomy is working well, and you are comfortable managing the ostomy, your child will be discharged from the hospital.   
• Follow-up: Follow-up in clinic about two weeks after surgery to check-in and discuss next steps.

Stage 2: Proctectomy and J-pouch Creation

• Timing: Stage 2 typically happens two to three months after stage 1, after your child has had time to heal from the first surgery.   
• Operation: Stage 2 is the longest surgery and takes about six to eight hours.   
• Hospital stay: After surgery, expect to stay in the hospital for three to five days. During this time, your child will be healing and learning how to care for their new ostomy. You may notice more ostomy output after this surgery, because the stoma is closer to the start of the digestive process so the output is typically more liquid and contains more digestive juices. 
• Discharging home: When your child is tolerating a regular diet, pain is controlled with over-the-counter medications, they are walking, ostomy is working well, and you are comfortable managing the ostomy your child will be discharged from the hospital.   
• Follow-up: Follow-up in clinic about two weeks after surgery to check-in and discuss next steps.

Stage 3: Takedown of the Loop Ileostomy

• Timing: Stage 3 happens two to three months after stage 2. Before stage 3, your child will get a special enema and x-ray. The special enema will let your doctor see on x-ray what the connection between the J-pouch and the anus looks like.  
• Operation: Stage 3 typically takes about two hours.  
• Hospital stay: After surgery, expect to stay in the hospital for about three to five days.   
• Discharging home: When pain is well-controlled, your child is eating, and they are up and walking, they will be able to go home.   
• Follow-up: Follow-up in clinic about two to four weeks after surgery.

Activity

• No strenuous activity, contact sports, gym class and no heavy lifting for two to four weeks after surgery. The doctor will let you know when it’s safe to go back to normal activities at the follow-up appointment.  
• No submerging in a bathtub for one week and no swimming in pools, hot tubs or beach for two weeks.

Diet Restrictions

• After surgery, diet is slowly advanced as tolerated. Once at home, you will find some foods will increase ostomy output and gas. Please see diet handout for more information about foods that cause more liquid stool and foods that will help thicken stool.   
• Some patients require extra fiber and medicines to thicken up the stool and decrease stool output. Please see fiber handout for information about how to take supplemental fiber and anti-diarrhea medicines.

Ostomy

• While in the hospital, you will meet with our wound/ostomy team to learn all about caring for the new ostomy. They will provide you with education materials and supplies to care for your child’s ostomy.   
• At home, record ostomy output to make sure it is not too much or too little. 

Ostomy Concerns

• If you have concerns about your ostomy such as surrounding skin, rash or product issues, notify the wound team.  
• If you have concerns about your ostomy such as change in color of stoma, decrease or increase in output, retraction (pulling inward) or protrusion (pushing outward) of stoma, contact the general surgery team. If urgent, call the on-call team at 202-476-5000 and ask for the surgical team on call.

Other Concerns

• Fever greater than 101.4° F  
• The skin/incisions are draining, red, warm or tender to touch  
• Persistent or worsening nausea, vomiting or diarrhea  
• No urine in eight hours  
• Pain is not better with acetaminophen (Tylenol) and ibuprofen (Advil, Motrin)  
• Any other questions or concerns 

Please call the surgery outpatient team at 202-476-5221 or email the general surgery team. The outpatient team is available Monday-Friday from 8 a.m. - 4 p.m. (no holidays). If you have urgent concerns overnight or over the weekend, please call 202-476-5000 and ask to speak to the general surgery resident on call. 

• Risks of infection, bleeding and damage to surrounding structures. Your surgeon uses many special ways to decrease these risks.
  • Early complications: Bowel obstruction (blockage), ileostomy prolapse, and anastomotic leak or stricture.  
  • Late complications: Adhesions, pouchitis (inflammation of the J-pouch), cuffitis (inflammation of the small cuff of remaining rectum) and small bowel obstruction (blockage).  
  • Long-term monitoring: Regular follow-ups are necessary to look for complications and ensure proper growth and development.
• There is a risk of pouch or anastomosis (surgical connection between the intestines) leaking, called pouchitis and cuffitis.
  • Pouchitis is inflammation (swelling and redness) of the pouch that is treated with antibiotics. Symptoms are increased stool frequency, diarrhea, abdominal pain and urgency. In some cases, a prolonged course of antibiotics is needed.   
  • Cuffitis is inflammation (swelling and redness) of the remaining rectum (rectal cuff). This is treated with rectal steroids. Symptoms of cuffitis include pain, bleeding and increased frequency of bowel movements. In rare cases, some patients require restarting medications to treat the inflammation.