Major duodenal papilla prolapse in Cronkhite–Canada syndrome

We describe the case of a 59-year-old Japanese woman presenting with recurrent proctorrhagia. Her clinical history was unremarkable except for recently presenting alopecia, dysgeusia, and onychodystrophy.

Ileocolonoscopy showed multiple strawberry-like sessile polyps ranging from 5 to 20 mm in size in the rectosigmoid ([Fig. 1]).

Fig. 1 Cronkhite–Canada colonic polyposis in a 59-year-old Japanese woman with recurrent proctorrhagia and recently presenting alopecia, dysgeusia, and onychodystrophy.

Upper endoscopy demonstrated hypertrophic gastric plicae and many sessile polyps of 5 – 15 mm in size spreading from the stomach to the distal duodenum ([Fig. 2 a, b]). i-SCAN digital contrast (I-SCAN) and optical enhancement virtual chromoendoscopy (Optivista EPK-i7010 video processor; Pentax, Tokyo, Japan) were activated to increase the detection of subtle mucosal changes, revealing several erosion-like mucosal defects within the surface of the polyps ([Video 1]). During withdrawal, the dynamics of a major duodenal papillary prolapse were clearly observed within the context of a large, laterally spreading, superficial, and elevated polypoid projection ([Video 1]).

Fig. 2 Cronkhite–Canada endoscopic appearances: a Hyperplastic plicae and polyposis of the stomach; b duodenal polyposis.

Video 1 Cronkhite–Canada syndrome. Enteroscopy conducted to the proximal jejunum, using high definition and optical enhancement virtual chromoendoscopy. A prolapsing major papilla is seen in duodenal polyposis.

Targeted biopsy samples showed histological features consistent with a diagnosis of Cronkhite–Canada syndrome ([Fig. 3 a, b]). A capsule endoscopy excluded additional polyp locations and systemic steroid treatment was introduced to reduce both polyp formation and bleeding.

Fig. 3 Histological appearances in Cronkhite–Canada syndrome: a gastric mucosa with hyperplastic glands without atypia (hematoxylin and eosin [H&E], × 100); b myxoid-edematous stroma with very scanty inflammatory infiltrates (H&E, × 400).

Cronkhite–Canada syndrome is an extremely rare nonhereditary gastrointestinal polyposis, with 450 cases reported in the literature, mainly in the Japanese population [1]. The syndrome usually presents with a triad of dermatologic disorders, including alopecia, onycodystrophy, and hyperpigmentation, associated with a variable degree of gastrointestinal symptoms including diarrhea, malabsorption, and hemorrhage [2]. Cronkhite–Canada polyps are usually sessile, with abundant stromal edema, hyperplastic glands, and cystic mucous retention, plus mild inflammation with predominant eosinophilic infiltrate within the surrounding mucosa [3]. The prognosis for Cronkhite–Canada syndrome is often unfavorable, because of complications (malabsorption, gastrointestinal hemorrhage, or intussusception) and the lack of standardized treatments [4]. The malignant transformation of polyps is still a matter of debate and no validated protocols for endoscopic surveillance are available [5].

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• References

• 1 Cronkhite Jr LW, Canada WJ. Generalized gastrointestinal polyposis; an unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia. N Engl J Med 1955; 252: 1011-1015
• 2 Watanabe C, Komoto S, Tomita K. et al. Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome: a Japanese nationwide survey. J Gastroenterol 2016; 51: 327-336
• 3 Zhao R, Huang M, Banafea O. et al. Cronkhite-Canada syndrome: a rare case report and literature review. BMC Gastroenterology 2016; 16: 23
• 4 Yun SH, Cho JW, Kim JW. et al. Cronkhite-Canada syndrome associated with serrated adenoma and malignant polyp: a case report and a literature review of 13 Cronkhite-Canada syndrome cases in Korea. Clin Endosc 2013; 46: 301-305
• 5 Tontini GE, Rimondi A, Neumann H. et al. Usefulness of virtual chromoendoscopy with optical enhancement in everyday clinical practice. Adv Res Gastroenterol Hepatol 2017; DOI: 10.19080/ARGH.2017.07.555719.