Cronkhite-Canada Syndrome: Comprehensive Imaging Guide and Clinical Overview
Overview
Cronkhite-Canada Syndrome derives its name from Leonard W. Cronkhite Jr. and Wilma J. Canada, who first described it in 1955 as a rare non-inherited disorder.
The naming rationale reflects their seminal case series linking diffuse gastrointestinal polyposis with ectodermal changes, distinguishing it from hereditary polyposis syndromes.
Clinically, patients present with alopecia, nail dystrophy, hyperpigmentation, diarrhea, weight loss, and protein-losing enteropathy due to extensive polyps throughout the gastrointestinal tract except the esophagus.
Cronkhite-Canada Syndrome follows a sporadic inheritance pattern with no genetic predisposition or familial occurrence reported.
Key Imaging Features
- Diffuse gastric involvement shows markedly enlarged rugal folds with nodular excrescences, often measuring greater than 1 cm in thickness, creating a cerebriform appearance on CT and MR enterography.
- “Whiskering” appears as multiple tiny projections on barium studies, resulting from barium trapping between irregular rugal nodules, a hallmark of Cronkhite-Canada Syndrome in the stomach.
- Innumerable small polyps carpet the colon and small bowel, with sizes ranging from 2-10 mm, showing uniform distribution on CT enterography without focal masses.
- Colonic polyps exhibit cystically dilated irregular crypts with luminal mucin pools on advanced imaging, alongside submucosal edema causing wall thickening up to 5-8 mm.
- Small bowel demonstrates patterns of innumerable carpet-like polyps, scattered varying-size polyps, or sparse small polyps, most prominent in duodenum and terminal ileum on MR enterography.
- Gastric antrum and angle display congested, edematous mucosa with nodular hyperplasia and ulcerated polyps, evolving to more diffuse involvement over months if untreated.
- Imaging pearls include concordance of polyp patterns between stomach and colon in most cases, with pitfalls of mistaking whiskering for malignancy due to irregular contours.
Pathophysiology
In Cronkhite-Canada Syndrome, non-inflammatory hamartomatous polyps arise from hyperplasia of the lamina propria with submucosal edema and inflammatory infiltrates, primarily eosinophils, leading to distorted gastrointestinal anatomy.
This polypoid proliferation traps contrast in inter-nodular spaces on barium studies, manifesting as whiskering and rugal thickening due to foveolar gland hyperplasia and villiform surface changes.
Mucosal edema and dilated crypts in colonic polyps create cystic appearances on CT, contributing to malabsorption and protein loss, which exacerbate wall thickening visible as hypoattenuating layers on cross-sectional imaging.
Over time, persistent pathological processes cause progression from focal distal gastric involvement to diffuse carpeting of the entire gastrointestinal tract, with potential complications like ulceration from mechanical irritation.
Differential Diagnosis
| Condition | Distinguishing Imaging Features from Cronkhite-Canada Syndrome |
|---|---|
| Familial Adenomatous Polyposis | Hundreds to thousands of small <5 mm colonic polyps without gastric rugal hypertrophy or whiskering; often includes duodenal adenomas and extraintestinal stigmata like osteomas, absent in Cronkhite-Canada Syndrome. |
| Peutz-Jeghers Syndrome | Larger, pedunculated small bowel polyps >1 cm with arborizing growth patterns on CT; pigmentation is perioral, not diffuse, and no ectodermal changes like alopecia. |
| Juvenile Polyposis Syndrome | Sparse, large solitary polyps mainly in colon with cystic dilation but lacking diffuse gastric whiskering and uniform carpeting seen in Cronkhite-Canada Syndrome. |
| Gastric Malignancy | Focal mass-like thickening >2 cm with lymphadenopathy on CT; lacks innumerable small polyps and ectodermal clinical features differentiating it from Cronkhite-Canada Syndrome. |
Imaging Protocols and Techniques
For suspected Cronkhite-Canada Syndrome, initiate with CT enterography or MR enterography using oral neutral contrast (e.g., VoLumen) and IV contrast at 1.5-2 mL/kg, with arterial and portal venous phases to assess gastrointestinal anatomy and polyp distribution.
Barium upper gastrointestinal study with small bowel follow-through and barium enema highlight whiskering and rugal patterns, though largely replaced by cross-sectional modalities for superior sensitivity in detecting small bowel involvement.
Colonoscopy with biopsy confirms histology, while capsule endoscopy visualizes duodenal and ileal changes; multiphase CT protocols (3-5 mm slices) quantify wall thickness and exclude complications like obstruction.
Pearls include multiplanar reformats on MR enterography for optimal crypt visualization; pitfalls involve overcalling edema as infiltration without clinical ectodermal correlation.
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