Case Report
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jun 14, 2016; 22(22): 5293-5296
Published online Jun 14, 2016. doi: 10.3748/wjg.v22.i22.5293
Laparoscopic restorative proctocolectomy with ileal pouch-anal anastomosis for Peutz-Jeghers syndrome with synchronous rectal cancer
Min-Er Zhong, Bei-Zhan Niu, Wu-Yang Ji, Bin Wu
Min-Er Zhong, Bei-Zhan Niu, Wu-Yang Ji, Bin Wu, Department of General Surgery, Peking Union Medical College Hospital, Beijing 100730, China
Author contributions: Wu B and Zhong ME designed the report; Zhong ME, Niu BZ and Ji WY collected the patient’s clinical data and wrote the paper.
Institutional review board statement: This article was reviewed and approved by Peking Union Medical College Hospital Review Board.
Informed consent statement: The patient involved in this study gave her written informed consent prior to study enrollment.
Conflict-of-interest statement: The authors have no conflicts of interests to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Bin Wu, MD, Professor of Surgery, Department of General Surgery, Peking Union Medical College Hospital, Shuaifuyuan Rd. 1, Beijing 100730, China. wubin0279@hotmail.com
Telephone: +86-10-69152210 Fax: +86-10-69152211
Received: March 15, 2016
Peer-review started: March 16, 2016
First decision: March 31, 2016
Revised: April 12, 2016
Accepted: May 4, 2016
Article in press: May 4, 2016
Published online: June 14, 2016
Processing time: 78 Days and 15.5 Hours
Abstract

We report on a patient diagnosed with Peutz-Jeghers syndrome (PJS) with synchronous rectal cancer who was treated with laparoscopic restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA). PJS is an autosomal dominant syndrome characterized by multiple hamartomatous polyps in the gastrointestinal tract, mucocutaneous pigmentation, and increased risks of gastrointestinal and nongastrointestinal cancer. This report presents a patient with a 20-year history of intermittent bloody stool, mucocutaneous pigmentation and a family history of PJS, which together led to a diagnosis of PJS. Moreover, colonoscopy and biopsy revealed the presence of multiple serried giant pedunculated polyps and rectal adenocarcinoma. Currently, few options exist for the therapeutic management of PJS with synchronous rectal cancer. For this case, we adopted an unconventional surgical strategy and ultimately performed laparoscopic restorative proctocolectomy with IPAA. This procedure is widely considered to be the first-line treatment option for patients with ulcerative colitis or familial adenomatous polyposis. However, there are no previous reports of treating PJS patients with laparoscopic IPAA. Since the operation, the patient has experienced no further episodes of gastrointestinal bleeding and has demonstrated satisfactory bowel control. Laparoscopic restorative proctocolectomy with IPAA may be a safe and effective treatment for patients with PJS with synchronous rectal cancer.

Keywords: Peutz-Jeghers syndrome; Laparoscopy; Ileal pouch-anal anastomosis; Restorative proctocolectomy; Multiple polyps in gastrointestinal tract

Core tip: Few options currently exist for the therapeutic management of Peutz-Jeghers syndrome with synchronous rectal cancer. Here, we present a patient diagnosed with Peutz-Jeghers syndrome with synchronous rectal cancer treated with laparoscopic restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA). The patient has experienced no further episodes of gastrointestinal bleeding and has demonstrated satisfactory bowel control. To our knowledge, this is the first report on laparoscopic restorative proctocolectomy with IPAA performed for the treatment of Peutz-Jeghers syndrome with synchronous rectal cancer.