Lui FCW, Lo OSH. Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report . World J Clin Cases 2021; 9(16): 4081-4089 [PMID: 34141769 DOI: 10.12998/wjcc.v9.i16.4081]
Corresponding Author of This Article
Oswens Siu-Hung Lo, FRCS (Ed), MBBS, Surgeon, Department of Surgery, LKS Faculty of Medicine, The University of Hong Kong, No. 102 Pokfulam Road, Hong Kong, China. oswens@hku.hk
Research Domain of This Article
Surgery
Article-Type of This Article
Case Report
Open-Access Policy of This Article
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/
World J Clin Cases. Jun 6, 2021; 9(16): 4081-4089 Published online Jun 6, 2021. doi: 10.12998/wjcc.v9.i16.4081
Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report
Frederick Chi-Wai Lui, Oswens Siu-Hung Lo
Frederick Chi-Wai Lui, Oswens Siu-Hung Lo, Department of Surgery, LKS Faculty of Medicine, The University of Hong Kong, Hong Kong, China
Author contributions: Lui FCW was responsible for drafting and revision of the manuscript, review of the literature, photos and tables editing and inpatient care of the patient; Lo OSH was the operating surgeon, responsible for the inpatient care and outpatient follow-up of the patient, and for the drafting and revision of the manuscript for important intellectual content; issued the final approval for the version to be submitted.
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: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Oswens Siu-Hung Lo, FRCS (Ed), MBBS, Surgeon, Department of Surgery, LKS Faculty of Medicine, The University of Hong Kong, No. 102 Pokfulam Road, Hong Kong, China. oswens@hku.hk
Received: January 31, 2021 Peer-review started: January 31, 2021 First decision: February 22, 2021 Revised: March 10, 2021 Accepted: March 24, 2021 Article in press: March 24, 2021 Published online: June 6, 2021
Abstract
BACKGROUND
Glycogen storage disease (GSD) is an autosomal recessive inborn metabolic disorder. Patients with GSD are prone to hypoglycaemia, hyperlactacidemia and bleeding. GSD type 1b (GSD-1b) patients specifically can develop neutropenia, recurrent bacterial infection and inflammatory bowel disease (IBD). Documentation of the long-term outcomes of surgical management of GSD-1b has been scarce, especially for Asian patients. We herein describe a case of GSD-1b complicated by IBD-like colitis and coloduodenal fistula. The patient was managed successfully with surgical intervention.
CASE SUMMARY
A 20-year-old Chinese lady confirmed by genetic testing to have GSD-1b was initially managed with uncooked cornstarch and granulocyte-colony stimulating factor. With recurrent abdominal symptoms, her condition was treated as clinical “Crohn’s disease” with mesalazine, prednisolone and azathioprine conservatively. Colonoscopy showed a tight stricture at the hepatic flexure. Subsequent computerized tomographic colonography revealed a phlegmon at the ileocaecal region with a suspected coloduodenal fistula. Eventually an exploratory laparotomy was performed and severe colitis at the ascending colon with coloduodenal fistula was confirmed. Right hemicolectomy with primary anastomosis and repair of the duodenum were performed. Surgical management of complications from GSD-1b associated IBD-like colitis has rarely been described. First-line treatment would usually be conservative. Surgical intervention like hemicolectomy is mainly reserved for refractory cases.
CONCLUSION
Surgical management of coloduodenal fistula in GSD-1b patients is a feasible and safe option when failed conservative management.
Core Tip: Inflammatory bowel disease-like colitis is associated with a few rare genetic disorders or certain drug use. First-line treatment is usually conservative with nutritional support or steroids. Granulocyte-colony stimulating factor or immunosuppressant may be beneficial. Surgical intervention like hemicolectomy is mainly reserved for refractory cases with intestinal obstruction resistant to medical treatment.
