Cronkhite–Canada syndrome

Case contributed by Dr Jeffrey Hocking

Presentation

Presented with protein losing enteropathy. Clinically his finger nails had fallen out, much of his hair had disappeared and he had become quite pigmented.

Patient Data

Age: 70
Gender: Male
Fluoroscopy

Stomach - Ill-defined whiskering throughout the fundus and cardia, with innumerably filling defects carpeting the antrum and body, particularly the greater curvature, in keeping with polyps and gastric wall thickening. 

Duodenum - Trace amount of contrast is seen passing through the duodenum, with evidence of wall thickening and numerous sessile and polypid lesions.

Jejunum, Ileum - Generally preserved appearance of jejunum, with occasional polypoid filling defects seen in the distal ileum. 

Colon - Innumerable small polyps coat the large bowel and haustral folds, with less defined areas of whiskering particularly at the proximal ascending colon and proximal sigmoid.

On the 5 hour delayed imaging there are several small polyps present in the rectosigmoid.

CT


TECHNIQUE:
Portal venous CT Abdomen/Pelvis with oral contrast. Images were first acquired of the pelvis, followed by the upper abdomen with additional oral contrast and air.

FINDINGS:
Stomach wall appears thickened and polypoid, with irregular contouring especially noted at the cardia and greater curvature. Extensive soft tissue thickening of the gastric antrum and pylorus, which extends to involve the duodenum from D1 to the distal D2. The duodenal thickening appears to reflect a combination of frank wall edema with superficially layered sessile and semi-sessile polyps. The distal jejunum and majority of the ileum appear unaffected.

Mural thickening and sessile polypoid changes are present within the terminal ileum, extending to involve the cecum, ascending colon and hepatic flexure. Peristalsis and contrast artefact obscures fine detail of the transverse colon, although there are multiple non-contiguous segments of wall thickening and clusters of sessile polyps. Descending colon is not well opacified, but appears thick walled. The rectosigmoid was not imaged.

No evidence of frank bowel obstruction or free gas.
No suspicious lymphadenopathy.

Diffuse fatty infiltration throughout the liver. Gallbladder is decompressed and thin walled.

Pancreas, spleen and adrenal glands are unremarkable.

Contrast present within the renal collecting system. No hydronephrosis or hydroureter. Large fluid density cortical cyst within the left kidney.

IMPRESSION:
Diffuse mural thickening and innumerable sessile and pedunculated polyps throughout the gastrointestinal tract, with particular involvement of the stomach, proximal duodeunm, and large bowel.

Case Discussion

Cronkhite-Canada Syndrome is a rare gastro-intestinal pathology with an uncertain etiology. Approximately 500 cases have been reported in the literature1. It has been described as a protein-losing enteropathy, and presents with sequalae of malnutrition, diarrhea, alopecia, nail dystrophy and skin hyperpigmentation.

Discussion in the literature has mostly focused on clinical and histolopathological sequalae, with radiological characterization lacking. Those studies that have described these focus primarily on fluoroscopic manifestations. Common findings include involvement of the stomach, duodenum and large bowel, with variable involvement of the jejunum and ileum1,2. The esophagus appears to be spared.

On follow-through studies, described findings include peripheral "whiskering" either due to polypoid carpeting of the mucosa, or due to area gastrica. Other findings include diffuse nodularity, rugal and haustral thickening and sessile and polypoid filling defects2.

Findings on CT mirror those on fluoroscopy, in this case demonstrating diffuse polyps throughout the stomach, duodenum and large bowel. The duodenum was prominently affected in this case, which may be a combination of polyps and edema.

 

This case was donated to radiopaedia by the Dr. G. M. Moro Film Library

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Case information

rID: 73534
Published: 14th Jan 2020
Last edited: 15th Jan 2020
Inclusion in quiz mode: Included

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