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Familial adenomatous polyposis

Cybil Corning, MD
FACS, FASCRS
Peter Kuan-Teh Lee
MD, FACS
Stefanie Schluender, MD
FACS, FASCRS
Jennifer Ford
FNP-BC, RNFA
Sarah Plummer
FNP-C, CRNFA

Practice Highlights


Robotic colorectal surgery since 2014

WELL-ESTABLISHED ENHANCED RECOVERY AFTER SURGERY (ERAS) PROGRAM

High-resolution anoscopy

Multidisciplinary GI tumor board

Sphincter-sparing surgery for rectal carcinoma

Sacral nerve stimulation for fecal incontinence

Anal manometry

What is familial adenomatous polyposis?

Familial Adenomatous polyposis (FAP) is a rare condition. It causes hundreds of polyps to form in the large intestine and colon – sometimes even thousands. They can also develop in the small intestine. The tumors begin forming in a person’s teens. The polyps can develop into cancer by the age of 40 if not treated.

There are three variations of FAP.

  • Attenuated familial adenomatous polyposis (AFAP). People with this variant have fewer colon polyps, and cancer develops in their 40s-50s.
  • Gardner syndrome. This type of FAP not only causes the polyps in the colon but also causes non-cancerous skin tumors and tumors in the soft tissues and bones.
  • Turcot syndrome is a rare, also inherited condition. People with this condition have an even higher risk of adenomatous polyps and cancer. They are also prone to medulloblastoma, a type of brain cancer.

Who gets familial adenomatous polyposis?

This genetic mutation is most often a hereditary disorder. However, in 25 to 30 percent of people this mutation can happen on its own. Genetic testing is done to determine someone’s risk factor.

Problems Associated with familial adenomatous polyposis.

  • Periampullary polyps can develop in the area where the pancreas and bile ducts go into the duodenum. If detected early, they can be removed but if left unchecked they can become cancerous.
  • Gastric fundic polyps can develop in the stomach’s lining.
  • are non-cancerous masses that can develop in the abdomen. They can be serious if they grow into nerves or blood vessels.
  • People with FAP can be at risk of developing cancer in other organs, the thyroid, central nervous system or adrenal glands.

People with FAP are also at risk for benign skin tumors, benign bone growths, pigmental changes in the retinas, anemia and dental abnormalities.

Treatment for familial adenomatous polyposis?

In order to prevent the polyps from turning cancerous, surgery is performed to remove the large intestine. Polyps often form in the duodenum (the first part of the small intestine), but those are usually monitored and polyps are frequently removed.

The surgery required could include:

  • Subtotal colectomy with ileorectal anastomosis. Your surgeon removes your large intestine, but leaves your rectum intact. The bottom of your small intestine (ileum) is attached to your rectum.
  • Total proctocolectomy with continent ileostomy. Your surgeon removes your colon and rectum and makes an opening (stoma) in your abdominal wall for an ileostomy. Solid waste passes through the stoma into a bag outside your body.
  • Total proctocolectomy with ileoanal anastomosis (also called J-pouch surgery). Your surgeon removes your colon and rectum, but creates a pouch with the bottom of your small intestine so you can have normal bowel movements.

 

Colorectal Disease Specialties We Treat

Colon Carcinoma
Rectal Carcinoma
Colorectral Polyps
Familial adenomatous polyposis
Anal Carcinoma
Small Bowel Cancers
Diverticulitis
Ulcerative colitis
| Crohn’s
Rectal prolapse
Pelvic exenteration
TAMIS for Rectal lesions
Hemorrhoids
Anal fissure
anal fistula
Perianal abscess

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