Ostomy Certification Test Bank Exam 2026 -2027 \COMPLETE
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FAP (Familial adenomatous polyposis)
-FAP is inherited in an autosomal dominant pattern with a 100% risk of
developing colon cancer by age 40 years.
-mutation of APC gene (adenomatous polyposis coli)
-FAP develop hundreds to thousands of adenomatous polyps in the colon and
in the duodenum and stomach. Each of these is at risk for malignant
transformation.
-Dx requires at least 100 colorectal adenomatous polyps.
-should undergo total colectomy or proctocolectomy with IPAA for treatment
and risk reduction rather than segmental resection alone. There is a high risk of
recurrence in the rectum if proctectomy is not performed.
FAP screening
-colorectal polyp develop around 16 y.o
-CRC develops 5-30 years later
-known carriers, colonoscopy at 12-14 y/o.
-once polyps identified annual screening
-upper endo every 1-3 years
-annual colonoscopy still needed after IPAA
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Gardner syndrome
-considered linked to FAP
-inherited polyposis syndrome with APC gene mutation
-causes benign tumors to form in many different organs and cause higher
risk CRC & FAP-related CAs
-associated with osteomas, epidermoid cysts, soft tissue tumors, fibromas,
and/or desmoid tumors
Gardner syndrome skin manifestations
-epidermoid cysts, trichilemmal hybrid, or pilomatricomas developing on the
face, scalp, or limbs of patients; something called a nuchal fibroma (diffuse
induration and swelling of the back of the neck)
-"Gardner fibroma" area of thick collagen bundles and interspersed fibroblasts
located mostly on the trunk
-Osteomas can arise on the mandible and skull.
-Dental lesions occur in almost one fifth of patients including odontomas,
absent, excess, or rudimentary teeth, or multiple caries.
Gardner syndrome sx management
-total colectomy is not curative due to potential for extra intestinal tissue
growth
-sx may be required for desmoid tumors obstructing organs
-ab sx can accelerate/precipitate desmoid tumor formation/growth
Gardner syndrome management
-endo surveillance
-panoramic dental radiographs
-celecoxib (poss. cardiac issues)
-sunlindac (stomach issues) mus be on protective GI coating drugs & H2
blockers, PPIs
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Peutz-Jeghers syndrome (PJS)
-Polyposis syndrome with formation of hamartomatous polyps in GI tract
-Rare disease, autosomal dominant inheritance (LKBI/STKII gene)
-Polyps common in small bowel, colon is most malignant area
PJS clinical presentation
-Presents around 11 y/o
-Altered pigmentation in dark blue/brown macules around mouth, eyes,
nostrils, buccal mucosa, palmar surface of the hands, and genitalia and
perianally
-complications leading to dx: abdominal pain due to bowel obstruction,
intussusception, volvulus, and rectal bleeding
-Check family members as well
PJS diagnosis
A definite diagnosis of PJS includes at least two of the following
characteristics: (1) hyperpigmentation of the lips or buccal mucosa, (2) two
or more hamartomatous polyps in the GI tract, or (3) a PJS family history
PJS medical management
-ongoing intestinal endoscopic surveillance plus continuous screening (PAPs,
mammo, testicular UA, etc.)
-SB series, capsule endo around 8 y/o
-Polyps removed endoscopically
-Surveillance endo every 2-3 years
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PJS sx management
-Acute surgical intervention is sometimes required for intussusception,
volvulus, and small bowel obstruction.
-CRCs associated with PJS should be managed like other CRCs with
segmental resection.
-Prophylactic colectomy is not recommended for PJS given its location in
multiple body sites
-polyps are above 1.5 cm in size or are suspicious for malignancy, they
should be removed via endo or sx
diverticular disease
-disorder that represents a spectrum of clinical presentation varying from
totally asymptomatic and uncomplicated to acute situations requiring
emergency surgery with a diversion of fecal stream
-Diverticular disease includes both diverticulosis and diverticulitis
-disease of aging, acquired over time and possible link to diet
-gut microbiome can be a factor
-other factors: NSAIDs, steroids, opiates
diverticulosis
-the presence of colonic diverticula, that is, small sac-like outpouchings of
the intestinal wall
-most people it is discovered only incidentally at colonoscopy or barium enema
testing
-mostly asymptomatic
-2 complications: acute episodes bleeding & diverticulitis
-chronic vague GI symptoms.. mild ab pain, bloating, constipation, diarrhea,
fluctuating bowel habits
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