Familial Adenomatous Polyposis:     Therapy    

Therapy
 


Surgical Therapy
If polyps are found at examination, the doctor will recommend colorectal surgery. Removing the colon after polyps begin to develop is the only way to prevent the development of colorectal cancer. The goal of surgery is to completely remove the cancer, alleviating symptoms and offering the best chance of cure. Preoperative evaluations to rule out metastatic disease may include colonoscopy, barium enema, endoscopic ultrasound (EUS), CAT scan, chest x-ray, liver, and CEA (for baseline levels). Proctocolectomy and colectomy, demonstrated below, show the difference between having both colon and rectum removed and having just colon removed, while the rectum remains intact. 
   

Figure 16. Comparison of proctocolectomy and colectomy surgical procedures
    
Several different operations are currently available for treatment. The four most commonly performed operations are:

1. Total proctocolectomy with Brooke ileostomy (with pouch)
2. Total proctocolectomy with Koch pouch
3. Colectomy with ileorectostomy
4. Restorative proctocolectomy (ileoanal pouch procedure)

Total Proctocolectomy with Brooke Ileostomy
This procedure involves complete removal of the entire colon and rectum. The end of the small intestine is brought out as an ileostomy. Although the restorative proctocolectomy is the preferred procedure in most cases, total proctocolectomy with Brooke ileostomy is generally performed in situations when invasive cancers are present in the rectum, when the anal sphincter is not functioning correctly, or in elderly patients in whom strength of the sphincter muscles is diminished.  
 

Figure 17. Technique for total proctocolectomy with Brooke ileostomy (front and side view).

An ileostomy is an opening on the abdomen through which stool leaves the body. An ileostomy can be temporary or permanent. In most cases it is necessary to wear an appliance called an ileostomy bag to collect body wastes. An ileostomy should not be considered a handicap, although it is an inconvenience. With proper care, there should be no odor or uncleanliness. Thousands of people of every age and of both sexes have had ileostomy surgery. After surgery, people can be just as busy, successful, and involved in daily routines as before surgery. In fact, they may be more active because of improved health.

Total Proctocolectomy with Koch Pouch
This operation involves complete removal of the colon and rectum with the creation of a continent ileostomy. It is similar to that of the Brooke ileostomy, but here a pouch is created under the abdominal wall with a continent. This operation is rarely done today, but can be considered in selected cases in which transanal reconstruction is not a good option. 
 
Figure 18. Technique for total proctocolectomy with Koch pouch(front and side view).

Colectomy with Ileorectostomy (Ileorectal Anastomosis)
In this procedure, the colon is removed but all or most of the rectum is preserved. The small intestine is attached to the upper portion of the rectum. The advantage of the ileorectostomy procedure is that it is a less complicated, one-stage operation, yet still preserves fecal continence and maintains tolerable bowel function. The main concern with this procedure is that the rectum is left in place, despite its potential propensity for the development of polyps and cancer. Colectomy with ileorectal attachment is generally performed in patients who have few or no polyps in the rectum. In this case, often the remaining rectum requires frequent surveillance and removal of premalignant polyps.  
 
Figure 19. Technique for colectomy with ileorectal anastomosis.

Restorative Proctocolectomy (Ileoanal Pouch Procedure)
The restorative proctocolectomy involves removal of the entire colon and most of or the entire rectum. The end of the small intestine (ileum) is attached to the very distal rectum with the creation of an ileal pouch. With this operation, either a small portion of rectal mucosa can be left intact or the remainder of the rectal lining can be stripped. In this way, continence can be maintained and yet all or nearly all of the at-risk large intestinal lining can be removed. The ileal pouch provides a reservoir for fecal storage. Typically, the operation is performed in two stages. In the first stage a temporary ileostomy is created. After a period of time the ileostomy is removed to direct the intestinal stream during the healing of the ileoanal pouch. Alternatively some medical centers favor a single-stage procedure where no temporary ileostomy is used.   
 
Figure 20. Technique for restorative proctocolectomy(ileal pouch procedure)

     
Figure 21. Technique for the ileal pouch anal anatomosis(mucosal lining left intact).

       
Figure 22. Technique for ileal pouch anastomosis with distal rectal mucosal stripping.

All operations involve removal of all or most of the colon. After a complete discussion of these operations, the patient and surgeon together can decide which one is best.

In some cases, after colon removal, a person may have an ileostomy. An ileostomy is an opening on the abdomen through which stool leaves the body. An ileostomy can be temporary or permanent. In most cases it is necessary to wear an appliance called an ileostomy bag to collect body wastes. An ileostomy should not be considered a handicap, although it is an inconvenience. With proper care, there should be no odor or uncleanliness. Thousands of people of every age and of both sexes have had ileostomy surgery. After surgery, people can be just as busy, successful, and involved in daily routines as before surgery. In fact, they may be more active because of improved health. 
 
 


Colorectal Cancer Staging
Colorectal cancers are staged (or classified) at the time of surgery. Staging describes the extent that the tumor has gone into or through the bowel wall and determines whether or not the cancer has spread to lymph nodes or other organs. This is done by microscopic exam of the tumor cells removed during surgery and by radiological exam of the area around the colon using CAT scan or endoscopic ultrasound (EUS).

There are two different methods of describing a colorectal cancer stage.

Both systems are described below, and are compared with each other in the table at the end of this section.

Dukes System
The modified Dukes system separates colorectal cancers into four groups—A, B, C, and D.

Dukes A–includes tumors that are found only in the inner wall of the colon or rectum.
Dukes B–includes tumors that have penetrated the muscle layer of the bowel wall or have gone through the bowel.
Dukes C–includes tumors that have spread to lymph nodes in the same region.
Dukes D–includes tumors that have spread to distant sites, such as the liver.

TNM System
The TNM system separates colorectal cancer into five stages—Stages 0–IV.

This system is recommended by the American Joint Commission on Cancer. In this classification system three different characteristics of the tumor are evaluated and assigned a value. 
  

Figure 23. Tissue layers of the colon with cut-away detailing layers.

  
Table 4.

T is used to describe the size and extent of invasion of the main tumor. Levels 1 through 4 describe the depth of tumor penetration through the bowel wall. Tis means that the tumor is “in situ” (has not gone into the bowel wall).

N is used to describe whether lymph nodes have any cancer cells and the number of lymph nodes involved. N0 means no nodes are involved, N1 when one to three nodes are involved and N2 when more than three lymph nodes are positive.

M refers to metastasis or cancer spread to other parts of the body. For example, M0 means that there is no evidence of distant metastatic disease, M1 means there is cancer spread to another site. 
 

Figure 24. Progression of high-grade dysplasia to cancer using the TNM staging system.

  
Table 5. Comparison of the Staging Systems

The prognosis for colorectal cancer patients depends on the extent of disease and the adequacy of the surgical procedure. Patients have a worse prognosis if the cancer has spread to lymph nodes or distant organs, has invaded blood and lymphatic vessels, or is poorly differentiated.  
 
 


Follow-up Care After Surgery
1. Complete physical exam every year
2. Stool blood testing every year
3. Upper endoscopy at least every 4 years
4. a. Flexible sigmoidoscopy every 6 months for patients who still have their rectum; b. Flexible sigmoidoscopy every 6 months to 1 year for patients with ileoanal pouches
5. Follow American Cancer Society guidelines for cancer surveillance.
  
 




Dietary Considerations after Surgery
The following are suggestions for people who have a total colectomy with an ileostomy: Chew foods well, and eat slowly,
Drink 6–8 glasses of liquids every day.

In the first 6 weeks after surgery avoid fibrous and stringy foods that could cause a food blockage:

  •  Popcorn 
  •  Nuts 
  •  Corn 
  •  Mushrooms 
  •  Celery 
  •  Skins of fruit and vegetables 
  •  Fresh pineapple
  •  Raw carrots 
  •  Chinese vegetables 
  •  Wild rice 
  •  Stalks of broccoli 
  •  Cabbage
  •  Coconut 
  •  Dried fruits (raisins, etc.) 
  •  Seeds

After 6 weeks these foods may be eaten in moderation as long as you chew well and drink fluid with this food.

When the ileostomy is closed, stool is passing via the ileal pouch and the person is ready for solid food. At this time patients should:

  •  Follow a low-residue diet. 
  •  Avoid fatty foods, spicy foods, and caffeinated beverages. 
  •  Chew food well. 
  •  Eat 6 small meals rather than 3 regular meals (moderate amount). 
  •  Do not skip meals, this will not stop stool output. In fact, you will probably have increased gas and more liquid, irritating the output. 
  •  Drink 6–8 glasses of fluids per day.

Foods that may increase pouch output:

  •  Raw fruits and vegetables 
  •  Leafy green vegetables 
  •  Spicy foods 
  •  Beer 
  •  Chocolate 
  •  Wine 
  •  Caffeinated beverages 
  •  High-fat foods 
  •  Foods high in simple sugars (honey, candy, jellies, sweetened beverages)

Foods that may decrease output:

  •  Bananas 
  •  Applesauce 
  •  Creamy peanut butter
  •  Rice
  •  Tapioca pudding

Foods that may contribute to anal irritation:

  •  Spicy foods 
  •  Foods with seeds 
  •  Nuts 
  •  Popcorn 
  •  Dried fruits
  •  Chinese vegetables 
  •  Raw fruits and vegetables 
  •  Corn, coconut

Foods that may increase gas:

  •  Milk and milk products 
  •  Carbonated drinks 
  •  Onions 
  •   Beer, beans, and the cabbage family (broccoli, Brussels sprouts, cauliflower) 
  •  Cucumbers 
  •  Melons 
  •  Nuts 
  •  Eggs

After 6 weeks, you may experiment with foods, one at a time. There are individual variations. If a particular food causes a problem, wait a few weeks and try it again.

The pouch or reservoir adapts over time. The pouch enlarges and irritability decreases. The number of stools per day decreases and they become pastier.

After a year most people find they can eat most foods. Some foods may continue to cause diarrhea, gas, and anal irritation. 
  
 


Lifestyle After Surgery
Sexual function is not impaired after surgery or ileostomy. It is important that both partners understand the surgery, by talking with the surgeon or the family physician. There is usually no need for change in established sex practices or in one's capacity to enjoy sexual intercourse. It is also possible to have successful pregnancies. However, a woman who plans to become pregnant should consult her physician before becoming pregnant. Physicians usually recommend that a woman wait about a year after a colorectal operation before becoming pregnant. This delay gives plenty of time for abdominal scars to heal soundly and for the woman's health to return to normal. An ileostomy should not harm the baby or endanger the mother during childbirth.

 
 
Make an appointment today - call (410) 955-4166. 

     
      
 
 
 
 
 
  

 
Complications
 
Overview
In addition to the risk for colorectal cancer, other complications of this hereditary condition may occur. For example, pre-cancerous polyps may develop in other parts of the gastrointestinal system, such as the stomach and small intestine. Although most polyps that develop in the stomach and small intestine are benign, cancer may develop in them. Young children affected with FAP have a small increased risk for hepatoblastoma, a liver tumor. Tumors may also occur in the thyroid gland, adrenal gland, bile ducts, and pancreas. A type of tumor known as a desmoid can also occur in the abdomen. Thus, physicians recommend that patients continue with life-long follow-up examination for cancer prevention.

 
 
Make an appointment today - call (410) 955-4166.

 
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