Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Surg. Feb 27, 2022; 14(2): 200-210
Published online Feb 27, 2022. doi: 10.4240/wjgs.v14.i2.200
Surgery for Cronkhite-Canada syndrome complicated with intussusception: A case report and review of literature
Jie Dong, Tian-Shi Ma, Jiang-Feng Tu, You-Wei Chen
Jie Dong, Jiang-Feng Tu, You-Wei Chen, Cancer Center, Department of Gastroenterology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), Hangzhou 310014, Zhejiang Province, China
Tian-Shi Ma, Cancer Center, Department of Pathology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), Hangzhou 310014, Zhejiang Province, China
Author contributions: Dong J was the patient’s doctor in charge, who was responsible for collecting medical history, reviewed the literature and drafting the paper; Tu JF did the literature review; Ma TS was a pathologist who gave the pathological results; Chen YW designed the study with Dong J and made contribution to revise the manuscript; All authors have read and approved the final manuscript.
Supported by The Medical Health Science and Technology Project of Zhejiang Provincial Health Commission, No. 2021436506; and General Scientific Research Project of Zhejiang Science and Technology Department, No. Y202044280.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: You-Wei Chen, MSc, Associate Chief Physician, Cancer Center, Department of Gastroenterology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), No. 158 Shangtang Road, Hangzhou 310014, Zhejiang Province, China. cyw@zju.edu.cn
Received: August 18, 2021
Peer-review started: August 18, 2021
First decision: October 2, 2021
Revised: October 15, 2021
Accepted: January 20, 2022
Article in press: January 20, 2022
Published online: February 27, 2022
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: Cronkhite-Canada syndrome, Intussusception, Treatment, Prognosis, Surgery, Case report

Core Tip: Cronkhite-Canada syndrome (CCS), a syndrome of multiple gastrointestinal polyps, skin pigmentation, hair loss, and fingernail/toenail dystrophy, is a rare nonhereditary disease. We report a case of CCS that quickly progressed, and intussusception occurred, which eventually led to surgery because systematic steroids, immunosuppressive agents, and biological agents were not applied. As a rare disease with poor prognosis, CCS should be treated aggressively. Meanwhile, improved compliance may lead to a better prognosis.