Surgery for Cronkhite-Canada syndrome complicated with intussusception: A case report and review of literature

Abstract

Background: Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disease with a syndrome of multiple gastrointestinal polyps, skin pigmentation, hair loss, and fingernail/toenail dystrophy. Intussusception is a serious condition with an occurrence rate of 5% in adults, which is mainly caused by intestinal tumors or other intestinal occupations.

Case summary: A 57-year-old woman was admitted to our hospital due to abdominal distension and pain for the past year. Her nausea and vomiting symptoms had been aggravated for the past month. Previous transoral enteroscopy results one year prior showed chronic erosive gastritis protuberans, duodenitis, and jejunitis. She had sparse body hair and brown pigmentation on the skin of her hands and bilateral anterior tibias. The nails of both hands were pale and lacked luster, and the fingernail of her ring finger was longitudinally cracked. Gastroscopy showed extensive diffuse polypoid lump changes in the gastric body and antrum, of 0.5-3 cm in size. Colonoscopy showed multiple polypoid mucosal bulges in the terminal ileum and multiple polyps (0.3-5 cm) throughout the colon. The patient was diagnosed with CCS and underwent partial excision of the polyps, but she refused hormone therapy. One month later, the patient complained of nausea and vomiting, accompanied by abdominal pain and inability to pass gas or stool. Contrast-enhanced computed tomography of the abdomen showed gastrointestinal polyposis and ileocecal intussusception. She underwent stomach and bowel surgery.

Conclusion: CCS, as a rare disease with poor prognosis, should be treated aggressively. Systematic steroids, immunosuppressive agents, and biological agents were not applied; thus, the patient's symptoms quickly progressed, and intussusception occurred. She had to undergo surgery. Improved compliance may lead to a better prognosis.

Keywords: Case report; Cronkhite-Canada syndrome; Intussusception; Prognosis; Surgery; Treatment.

Figure 1

Physical examination. A: Sparse hair; B: Nail dystrophy; C: Skin pigmentation of the hands; D: Skin pigmentation of the legs.

Figure 2

Abdominal enhanced computed tomography. Thickening of the gastrointestinal tract with multiple cauliflower-like and nodular protrusions. A: The stomach wall; B: Part of the small intestinal wall; C and D: Part of the colon wall.

Figure 3

Positron-emission tomography/computed tomography showing multiple nodules with increased fluorodeoxyglucose uptake in the stomach wall, descending duodenum, and bulb, in the small intestine (obvious increase in the ileum), and the colon (obvious increase in the ascending colon). Multiple nodular thickening with increased fluorodeoxyglucose (FDG) uptake was observed in the proximal rectum. A: Whole-body maximum intensity projection 18F-FDG and positron-emission tomography (PET) image; B: PET; C: Computed tomography (CT); D: PET/CT.

Figure 4

Endoscopic findings in the stomach. Extensive and diffuse polypoid eminences in the stomach. A: Antrum; B: Lower part of the gastric body; C: Middle part of the gastric body; D: Upper part of the gastric body; E: Gastric fundus; F: Duodenal bulb.

Figure 5

Endoscopic findings in the colon. Multiple polypoid mucosal bulges in the distal small intestine and multiple polyps throughout the colon. Some were villus-like changes, and severe hyperemia was observed on the surface. A: Terminal ileum; B: Cecum; C and D: Ascending colon; E and F: Transverse colon; G: Descending colon; H: Sigmoid colon; I: Rectum.

Figure 6

Histopathology and hematoxylin and eosin staining of gastroscopic pathology samples suggested a diagnosis of juvenile polyps. A: × 11; B: × 12.5.

Figure 7

Histopathology and hematoxylin and eosin staining of colonoscopic pathology samples suggested a diagnosis of juvenile polyps. A: × 12.5; B: × 25.

Figure 8

Abdominal enhanced computed tomography. Multiple concentric ring signs in the ileocecal area indicating ileocecal intussusception. A: Plain computed tomography scan; B: Arterial phase; C and D: Venous phase.