“Shaggy” or actinia-(anemone)-like villi in Cronkhite Canada syndrome

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We describe a patient with Cronkhite Canada syndrome in whom we performed a deep enteroscopy because of ongoing diarrhea and anemia. On deep enteroscopy the upper and middle jejunum where characterized by a partial...

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We describe a patient with Cronkhite Canada syndrome in whom we performed a deep enteroscopy because of ongoing diarrhea and anemia. On deep enteroscopy the upper and middle jejunum where characterized by a partial villous atrophy and multiple patchy areas of villous loss. Using the water immersion technique with the DBE, the mucosa appeared like an anemone. Note that the villi had a long, thin, finger-like appearance. The feeding arteriole of the villi can be seen as a red string in the center of some villi.

“Shaggy” villi have been described in Peutz-Jeghers syndrome (PJS) (1). Cao et al. described similar findings in a patient with Cronkhite Canada syndrome (CCS) (2). The authors stated that the jejunal mucosa was “actinia-like” (actinia is the Latin name for anemones), very similar to the shaggy appearance in PJS. Interestingly there exists a phenotypic overlap between CCS and the other hamartomatous syndromes (2,3). This novel new mucosal finding may be common to all hamartomatous polyposis syndromes, which include juvenile polyposis, PJS, hereditary mixed polyposis syndrome, the phosphatase- and tensin-homolog-gene hamartoma tumor syndromes (Cowden and Bannayan-Riley-Ruvalcaba syndromes), which are autosomal-dominantly inherited, and the Cronkhite-Canada syndrome (CCS), which is acquired (1-3).

References:

1. Noel RJ, Werlin SL. Peutz-Jeghers syndrome: are "shaggy" villi part of the pathology? Gastrointest Endosc. 2008;68:1004-1005.
2. Cao XC, Wang BM, Han ZC. Wireless capsule endoscopic finding in Cronkhite-Canada syndrome. Gut. 2006;55:899-900.
3. Calva D, Howe JR. Hamartomatous polyposis syndromes. Surg Clin North Am. 2008;88:779-817.

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