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Colorectal Surgery: Polyposis Syndromes and Inflammatory Bowel Disease


  • 1.

    Identify four different classes of intestinal polyps.

    • Neoplastic (adenomatous, tubular, villous, tubulovillous, serrated)

    • Hamartomatous

    • Inflammatory and lymphoid

    • Hyperplastic

  • 2.

    What is a hamartoma?

    A hamartoma is an exuberant growth of normal tissue in an abnormal amount or location. An isolated hamartomatous polyp has no malignant potential.

  • 3.

    Which intestinal polyposis syndromes are associated with hamartomatous polyps?

    • Peutz-Jeghers syndrome (PJS)

    • Juvenile polyposis (familial or generalized)

    • Cronkhite-Canada syndrome (hamartomatous polyps with alopecia, cutaneous pigmentation, and toenail and fingernail atrophy)

    • Intestinal ganglioneuromatosis (isolated or with von Recklinghausen disease or multiple endocrine neoplasia type 2)

    • Ruvalcaba-Myhre-Smith syndrome (polyps of colon and tongue, macrocephaly, retardation, unique facies, pigmented penile macules)

    • Cowden disease (gastrointestinal [GI] polyps with oral and cutaneous verrucous papules [tricholemmomas], associated with breast cancer, thyroid neoplasia, and ovarian cysts)

  • 4.

    How is PJS manifested?

    This autosomal dominant trait is often heralded by the presence of melanin spots on the lips and buccal mucosa. Hamartomas are almost always present on the small intestine and occasionally on the stomach and colon. Previously considered a benign process, patients with PJS are at increased risk for multiple cancers: breast (50%), GI (50%), pancreatic (35%), gynecologic (10%-20%), and testicular (< 10%) . Aggressive cancer screening programs are recommended for persons with PJS.

  • 5.

    Describe the manifestation of familial adenomatous polyposis (FAP).

    FAP is an autosomal-dominant, non–sex-linked disease in which more than 100 adenomatous polyps affect the colon and rectum. FAP is caused by mutation in the adenomatous polyposis coli (APC) gene on the long arm of chromosome 5 at the 5q21-q22 locus. The APC protein is a tumor suppressor that, when mutated, fails to bind beta-catenin and allows for unregulated cellular growth. One third of patients present as the propositus case (presumed mutation) with no prior family history. The disease invariably leads to invasive colon cancer if not treated. The average age at diagnosis of colon cancer is 39 years compared with 65 years for routine colon cancer.

  • 6.

    What is Gardner syndrome?

    Gardner syndrome is a phenotypic variant of FAP manifest by colonic polyposis plus fibromas of the skin, osteomas (typically of the mandible, maxilla, and skull), epidermoid cysts, desmoid tumors, and extra dentition.

  • 7.

    How does one screen for FAP?

    When family history is positive, children should undergo annual sigmoidoscopic surveillance beginning at age 10 to 12 years. When polyps are identified, a full colonoscopy is recommended. Once multiple adenomas are documented, colectomy is recommended. Mutational analysis of the APC gene is the most accurate diagnosis. Ophthalmoscopic examination for congenital hypertrophy of the retinal pigment epithelium (CHRPE) can detect involved patients as early as 3 months of age with a 97% positive predictive value for developing FAP. CHRPE is present in 55% to 100% of FAP patients.

  • 8.

    What are the surgical indications for ulcerative colitis?

    • Intractability or failure of medical management

    • Fulminant colitis (toxic megacolon, bleeding, diarrhea)

    • Prophylaxis of carcinoma (presence of high-grade dysplasia)

    • Treatment of carcinoma

  • 9.

    What are the elective surgical options for FAP and chronic ulcerative colitis?

    • Total proctocolectomy with end (Brooke) ileostomy

    • Total proctocolectomy with continent ileostomy reservoir (Kock pouch)

    • Abdominal colectomy with ileorectal anastomosis

    • Near-total proctocolectomy ± rectal mucosectomy and ileal pouch-anal anastomosis (IPAA)

  • 10.

    Can one always tell the difference between Crohn’s disease and ulcerative colitis?

    No. Colitis that cannot be categorized as definitely Crohn or ulcerative colitis is called indeterminate colitis and may account for 5% to 10% of cases referred for surgical consideration.

  • 11.

    What is pouchitis?

    Pouchitis, one of the most frequent long-term complications of IPAA, is a nonspecific acute or chronic inflammation of the reservoir. Pouchitis is found in 7% to 44% of patients with IPAA; it presents with watery, bloody stools, urgency, frequency, abdominal pain, fever, malaise, and possible exacerbation of extra-intestinal manifestations of inflammatory bowel disease. The cause is uncertain, but the risk is greater in chronic ulcerative colitis than in familial polyposis. Pouch stasis, bacterial overgrowth, dysbiosis, ischemia, pelvic sepsis, oxygen-derived free radicals, altered immune status, and lack of mucosal trophic factors have been proposed as etiologic factors.

  • 12.

    How is pouchitis treated?

    Successful treatment regimens include metronidazole and other antianaerobic antibiotics as well as steroid or 5-aminosalicylate enemas. Topical volatile fatty acids and glutamine have been used with variable success. Maintenance with the probiotic VSL#3 has been reported to help prevent recurrences. Although half of patients with pouchitis at some time suffer a recurrence, very few develop intractable involvement requiring pouch excision.

  • 13.

    Does a defunctionalized colon develop colitis?

    Although controversial, some patients with a portion or the entire colon out of the fecal stream develop an inflammation difficult to distinguish from ulcerative colitis on biopsy. The diagnosis of diversion colitis is suggested when bloody mucopus is passed from the separate colorectal segment . The colon may be isolated by diverting ileostomy, end or loop colostomy, mucous fistula, or Hartmann procedure. It is believed that short-chain fatty acids normally produced by anaerobic bacteria serve as a trophic factor for the colonocytes. The diversion colitis quickly resolves on restoration of intestinal continuity; when restoration is not possible, the administration of short-chain fatty acid enemas is beneficial.

  • 14.

    What type of ileal pouches are used?

    Although higher-volume pouches are advocated (W = quadruple, S = triple, and J = two-limbed), the long-term functional results may not be all that different. The author prefers a long (15 cm) J pouch (when feasible in one stage) preserving the anal transition zone. The procedure has classically been two-staged, with construction of a temporary ileostomy followed at an interval by ileostomy takedown. Recent experience has shown that the morbidity of a one-stage IPAA may be less if the patient is taking no or low-dose steroids and the operation is performed without complication. Because there is really only one shot at getting it right (pelvic sepsis significantly diminishes ultimate pouch function), intraoperative judgment is at a premium.

Anorectal Disease

  • 15.

    What are anal fissures?

    A generally painful rip or tear in the sensitive anoderm of the anal canal. Most anal fissures are located in the posterior (90%) or anterior (10%) midline of the anal canal.

  • 16.

    What disorders should be considered in patients with laterally situated anal fissures?

    Disorders to consider are Crohn’s disease, ulcerative colitis, syphilis, tuberculosis, leukemia, carcinoma, and acquired immunodeficiency syndrome.

  • 17.

    How are acute fissures managed?

    Conservative treatment consists of stool softeners and bulk agents to avoid hard bowel movements, sitz baths to help decrease sphincter spasm, topical anesthetics, and topical steroids. Topical nitroglycerin or nifedipine ointment reduces anal spasm. Injection of botulinum toxin also has been used to relax the anal sphincter.

  • 18.

    What are the signs of a chronic anal fissure? What do they imply?

    A chronic anal fissure can be identified by the presence of a sentinel pile (skin tag or hemorrhoid), anal ulcer (with fibropurulent material or visible internal sphincter muscle in the base), and a hypertrophied anal papilla arising from the dentate line. A chronic anal fissure usually does not respond to conservative treatment, and surgical intervention is in order.

  • 19.

    Which surgical procedures are available for treatment of a chronic anal fissure?

    Open or closed lateral internal sphincterotomy, excision (ulcerectomy), excision and Y-V or other anoplasty, or anal dilation.

  • 20.

    Differentiate external from internal hemorrhoids.

    • External hemorrhoids originate distal to the dentate line of the anus and are covered by squamous epithelium. External hemorrhoids may thrombose or become filled with clotted blood. Typically these are painful, involving the anoderm.

    • Internal hemorrhoids arise above (proximal to) the dentate line and are covered with transitional and columnar epithelium. First-degree hemorrhoids swell and bleed. Second-degree hemorrhoids prolapse and spontaneously reduce. Third-degree hemorrhoids prolapse and can be manually reduced, whereas fourth-degree hemorrhoids are irreducible. Typically these are not painful above the anoderm.

  • 21.

    How are acute hemorrhoids treated?

    • Topical medicines such as anesthetics, hydrocortisone preparations, and astringents (witch hazel, glycerin, magnesium sulfate) can be used.

    • Emergency hemorrhoidectomy is performed to excise the inflamed hemorrhoid. Circular stapling devices have also been used to treat larger hemorrhoids.

  • 22.

    List several minimally invasive outpatient treatments of internal hemorrhoids.

    Outpatient treatments are rubber band ligation, bipolar cautery, direct current electrical therapy, infrared coagulation, sclerotherapy, and cryotherapy.

  • 23.

    How is an acute thrombosed external hemorrhoid best treated?

    Excision of the clot and involved hemorrhoidal complex (as opposed to incision alone) best prevent future recurrence at the same site.

  • 24.

    Explain the cause of anorectal abscesses and fistulas.

    A cryptoglandular origin seems to provide the best explanation. Four to 10 anal glands enter the anal canal at the level of the crypts in the dentate line. The glands extend back into the internal sphincter two thirds of the time and into the intersphincteric space half the time. Blockage of the gland leads to an overgrowth of bacteria with resultant pressure necrosis and abscess formation. An abscess or infection that causes an abnormal communication between two surfaces (such as the anal canal and perianal skin) creates a fistula.

  • 25.

    List the various types and locations of anorectal abscesses.

    Types and locations are submucosal, intersphincteric, perianal (anal verge), ischiorectal (perirectal), and supralevator.

  • 26.

    What is the best treatment for an anorectal abscess?

    Prompt incision and drainage. There is little or no role for antibiotics (exceptions are immunocompromised patients and patients with prosthetic heart valves or severe cellulitis) and no reason to wait for the abscess to point or become fluctuant before surgical treatment.

  • 27.

    What is the Goodsall rule?

    See Figure 76-1 .

    Figure 76-1, The Goodsall rule helps predict the location of the internal opening of an anal fistula based on the site of its external opening. Accurately determining the criminal crypt of fistula origin on the dentate line is important at the time of surgical treatment, generally fistulotomy. If the anus is divided into imaginary anterior and posterior halves in the coronal plane, posterior fistulas tend to curve into the posterior midline. Anterior fistulas shorter than 3 cm tend to proceed radially to the dentate line, whereas anterior fistulas longer than 3 cm may track back to the posterior midline.

  • 28.

    What is a seton?

    A seton is a drainage device used to control and treat an anal fistulous abscess. It is inserted through and through a fistula tract and secured to itself, thus making a circle about some portion of the anal sphincter muscle. It serves as a cutting device to exteriorize the fistula slowly. Typical setons are Penrose drains, Silastic vessel loops, or silk sutures.

  • 29.

    What are the common indications for inserting a seton?

    • High fistulous abscesses involving greater than one-half the length of the anal canal muscle

    • Anterior fistulas in a woman

    • Inflammatory bowel disease

    • Older adult patients or patients with multiple previous anorectal surgeries

  • 30.

    List treatment options for anorectal fistulas.

    • Fibrin sealant glues

    • Collagen plug

    • Monoclonal anti–tumor necrosis factor antibodies for Crohn’s fistulas

    • Park fistulotomy procedure, with excision and debridement of the fistula tract, muscle repair, advancement flap coverage of the internal opening, and drainage of the external portion of the fistula tract

  • 31.

    When is anorectal suppurative disease especially dangerous?

    In the presence of neutropenia, as associated with chemotherapy. Unfortunately, surgery and even anorectal digital examination may be contraindicated. Often bacterial infection is widespread without formation of purulence or a classic abscess.

  • 32.

    What is Fournier gangrene?

    Fournier gangrene is a necrotizing soft tissue infection of the perineum. Although rare, it can present as a suspected perirectal abscess, so a high index of suspicion must be maintained. Treatment is prompt surgical debridement.

    • Local signs —crepitance, bullae, cellulitis

    • Systemic signs —altered mental status, hypotension, oliguria

  • 33.

    What is perianal Paget disease?

    Perianal (extramammary) Paget disease is characterized by a scaly, inflamed dermis resembling eczema. Biopsy reveals typical Paget cells with round, pale, vacuolated, mucin-positive cytoplasm with an eccentric reticular nucleus. It is often a chronic condition, but underlying carcinoma must be ruled out as invasive anorectal cancer may be associated with Paget disease.

  • 34.

    Which patient characteristics are associated with rectal prolapse?

    • Chronic constipation

    • Deep pouch of Douglas

    • Neurologic disease

    • Patulous anus

    • Female sex

    • Diastasis of the levator ani muscles

    • Nulliparity

    • Lack of fixation of the rectum to the sacrum

    • Redundant rectosigmoid colon

    • Previous anorectal surgery

  • 35.

    What surgical options are available for rectal prolapse?

    Resection (standard or laparoscopic approach) of redundant colon and rectum with a rectal fixation (rectopexy) is generally associated with the best long-term results in patients who are fit for a major operation. For patients at higher risk for major surgery, other procedures have been described, including narrowing the anal orifice (Thiersch operation) or a perineal rectosigmoidectomy with levator placation (Altemeier procedure).

Colorectal Malignancies

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