Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Apr 26, 2020; 8(8): 1502-1506
Published online Apr 26, 2020. doi: 10.12998/wjcc.v8.i8.1502
Unusual association of Axenfeld-Rieger syndrome and wandering spleen: A case report
Yi-Lin Chang, Joseph Lin, Yu-Hsien Li, Lien-Cheng Tsao
Yi-Lin Chang, Joseph Lin, Yu-Hsien Li, Lien-Cheng Tsao, Department of General Surgery, Changhua Christian Hospital, Changhua 50006, Taiwan
Author contributions: Chang YL and Lin J contributed equally to this work; Tsao LC drafted the study; Chang YL analyzed the data; Lin J wrote the manuscript; Li YH and Tsao LC operated on the patient.
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: Lien-Cheng, Tsao, MD, Surgeon, Department of General Surgery, Changhua Christian Hospital, No. 135, Nanxiao Street, Changhua 50006, Taiwan. yannick881009@gmail.com
Received: January 9, 2020
Peer-review started: January 9, 2020
First decision: March 18, 2020
Revised: March 27, 2020
Accepted: April 15, 2020
Article in press: April 15, 2020
Published online: April 26, 2020
Processing time: 105 Days and 20.2 Hours
Core Tip

Core tip: We demonstrated a rare case of Axenfeld-Rieger syndrome (ARS) and its association with torsion of wandering spleen. ARS is an autosomal dominant genetic disease characterized by ocular developmental disorders. This 25-year-old female presented with a lower abdominal pain of increasing severity, and she was diagnosed splenic torsion by diagnostic computed tomography. An emergent laparoscopic exploration was performed, and an ischemic spleen without its normal ligamentous attachments was noted. The spleen did not regain its normal vascularity after detorsion; thus, we performed the laparoscopic total splenectomy. This case demonstrates a rare association of wandering spleen and ARS.