Cronkhite-Canada Syndrome Workup
- Author: Kruti Parikh; Chief Editor: Dirk M Elston, MD more...
Various laboratory studies may include the following:
Electrolyte and micronutrient determination - Hypokalemia; hypocalcemia; depressed serum levels of zinc, iron, copper, and magnesium; and vitamin B-12
Hematology - Depressed white blood cell count, hemoglobin, red blood cell count, and hematocrit
Serum proteins - Hypoproteinemia with hypoalbuminemia; increased in alpha1 globulin level
Raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
Elevated serum levels of gastrin, histamine-fast achlorhydria, hypochlorhydria.
Biochemical and hematologic tests - Total protein level; albumin level; glucose and lipids concentration; iron, magnesium, zinc, calcium, sodium, potassium, and copper concentrations; ESR; CRP
Decreased cholinesterase activity
Stool examination - Occult blood and Sudan III staining
H pylori infection test
Endoscopic (also wireless capsule endoscopy ) procedures (ie, gastroscopy, colonoscopy, sigmoidoscopy) reveal polyp lesions of the sessile or semipedunculated type throughout the stomach, duodenum, ileum, and colon, sparing the esophagus. Ikeda et al found pedunculated polyps in patients with Cronkhite-Canada syndrome. A few reports have described selective sparing of the stomach, small intestine, and/or the colorectum.[32, 33] Their size varied from a few millimeters to 2 cm in diameter. Endoscopic findings in the stomach also include reddish and edematous granular lesions with mucoid exudate and giant folds. Abdominal CT scanning may reveal thickened gastric folds.
Regarding radiography, a barium enema and small intestine double-contrast radiology examination show polypoid lesions.
A CT endoscopy using a multidetector-row CT scan with 3-dimensional reconstruction has been shown to be useful for the detection of Cronkhite-Canada syndrome polyps and for the monitoring of effects of therapy.
Magnified chromoendoscopy with crystal violet reveals sparsely distributed crypt openings with widening of the pericryptal space on the surface of the polyps and the intervening colonic mucosa.
Fluoroscopic examination of the stomach may show rough granular changes of the mucosa with edematous giant rugae and polypoid lesions.
Scintigraphy using technetium Tc 99m–labeled human albumin may result in leakage to the gastrointestinal tract.
Consider intraoperative or postoperative examination of p55 immunoreactivity accumulation in the tissue suspected of having a carcinomatous component.
See the list below:
Presence of antinuclear antibodies, usually with a nucleolar pattern
Malignancy markers (carcinoembryonic antigen, alpha-fetoprotein)
D-xylose absorption test (impaired)
Glucose tolerance test (may be impaired)
Fecal fat excretion (increased)
Gastrointestinal clearance of alpha-1 antitrypsin (usually elevated)
Culture of nail scrapings for fungi (for differential diagnosis)
HIV testing (possibly)
Stool culture for Salmonella, Shigella, Yersinia, and Campylobacter species and for parasites.
Procedures may include the following:
Endoscopic procedures (gastroscopic, duodenoscopic, and colonoscopic examinations)
X-ray procedures (eg, barium enema examination, small intestine double-contrast radiology after Sellink)
Fluoroscopic examination of the gastrointestinal tract
Abdominal computed tomographic scan
Endoscopic biopsy of the polypoid lesion
Histologic examination of polypoid lesions and, if necessary, the scalp lesion
Hyperpigmentation is related to an increase in melanin within the basal layer with or without the melanocyte proliferation, pigment incontinence, hyperkeratosis, and nonspecific perivascular inflammation.[37, 38]
Scalp biopsy shows a marked noninflammatory loss of follicular units, miniaturization of the hair shafts, markedly dilated follicles, a heavy deposition of glycosaminoglycans in the reticular dermis, and anagen-telogen conversion.
Histologically, polyps in patients with Cronkhite-Canada syndrome are pseudopolypoid-inflammatory changes with cystic dilatation. Ikeda et al found that some of the colon polyps presented a histologic pattern of a tubulovillous adenoma and others exhibited that of a juvenile-type polyp. The latter polyps were characterized by the presence of elongated and/or tortuous crypts with microcystic dilatation and inflamed edematous wide stroma. The areas of focal intestinal metaplasia were present. Even though cellular atypia was present, the adenomatous polyps showed histologic similarity to juvenile polyps with inflamed edematous stroma and occasional cystic glands. Extensive diffuse mucosal thickening has been reported instead of individual polyp growth. Often, this diffuse thickening, possibly an early active stage of disease, is seen in the upper gastrointestinal tract, leading to giant gastric and/or duodenal rugal folds. Although rare, flat or atrophic mucosa has also been described in patients with Cronkhite-Canada syndrome.
According to Burke and Sobin, Cronkhite-Canada syndrome polyps are characterized by their broad sessile base, expanded edematous lamina propria, and cystic glands. The only reliable distinction between Cronkhite-Canada syndrome and colonic juvenile polyposis is the pedunculated growth of the latter with the exception of the gastric polyps. Gastric polyps in Cronkhite-Canada syndrome are sessile and composed of focally dilated irregular foveolar glands within a lamina propria expanded by edema and often an inflammatory infiltrate. Most polyps contain smooth muscle fibers in the lamina propria, and a minority has surface erosions. Gastric Cronkhite-Canada syndrome polyps are quite similar to juvenile or hyperplastic polyps. Prominent mast cells, eosinophils, and IgG4 plasma cell infiltration have all been reported as well.[5, 41]
The most constant features of Cronkhite-Canada syndrome polyps are a sessile base, an expanded edematous lamina propria, and dilated glands. Other features, including inflammation, a small number of smooth muscle fibers, and a complex contour, are variable.
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