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): 1538-1546
Published online Apr 26, 2020. doi: 10.12998/wjcc.v8.i8.1538
Unusual presentation of congenital radioulnar synostosis with osteoporosis, fragility fracture and nonunion: A case report and review of literature
Zhan-Yu Yang, Jiang-Dong Ni, Ze Long, Le-Tian Kuang, Shi-Bin Tao
Zhan-Yu Yang, Jiang-Dong Ni, Ze Long, Le-Tian Kuang, Shi-Bin Tao, Department of Orthopedics, The Second Xiangya Hospital, Central South University, Changsha 410000, Hunan Province, China
Author contributions: Ni JD contributed to manuscript drafting; Yang ZY reviewed the literature and contributed to manuscript drafting; Long Z and Kuang LT were responsible for the follow-up and data collection; Tao SB was responsible for the revision of the manuscript; all authors approved the final version to be submitted.
Informed consent statement: Informed written consent was obtained from the guardians of 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: Jiang-Dong Ni, MD, Professor, Department of Orthopedics, The Second Xiangya Hospital, Central South University, No. 139 Renmin Street, Changsha 410000, Hunan Province, China. nijiangdong001@csu.edu.cn
Received: January 8, 2020
Peer-review started: January 8, 2020
First decision: March 18, 2020
Revised: March 26, 2020
Accepted: April 10, 2020
Article in press: April 10, 2020
Published online: April 26, 2020
Processing time: 107 Days and 2.9 Hours
Abstract
BACKGROUND

Congenital radioulnar synostosis (CRUS) is a rare deformity of the upper extremity. It is characterized by loss of rotation of the involved forearm and functional limitations in daily activities. No studies on CRUS with osteoporosis have been reported to date, and osteoporosis is usually recognized as an important dimension of genetic disorder in children. We discuss the possible relationship among this disorder, osteoporosis and fracture nonunion, investigate the strict surgical indications and recommended treatments.

CASE SUMMARY

A 14-year-old male patient with bilateral CRUS with osteoporosis, fragility fracture and nonunion of fractures in ulna and radius presented our institution for further treatment, complaining of limitation in rotation. The bone mineral density of the hip and lumbar spine was 0.687 g/cm2 and 0.705 g/cm2, respectively, and the Z-score for both was -2.1, which revealed osteoporosis and a high risk of fracture. Tow serum bone turnover markers indicated an imbalance of bone metabolism. Reoperation for ulna fracture with autogenous bone grafting and a postoperative physiotherapy program were adopted rather than the separation of pathological synostosis. Radiological examination, observational posture assessment and limb function scale were evaluated before and 1 year after surgery. At 1 year, the fracture nonunion had almost recovered, forearm movement function on the fracture side was restored, and function on the healthy side was significantly improved compared with that before rehabilitation.

CONCLUSION

Surgical indications for CRUS vary from person to person. Surgery should not be the first choice of treatment, and physiotherapy is not inferior to surgical treatment.

Keywords: Congenital radioulnar synostosis; Surgical indication; Osteoporosis; Fragility fracture; Nonunion; Case report

Core tip: Congenital radioulnar synostosis is a rare deformity of the upper extremity, which is characterized by loss of rotation and a variety of functional limitations. We present a rare case of bilateral congenital proximal radioulnar synostosis with osteoporosis, fragility fracture and nonunion. This case suggests that internal fixation plus bone grafting or stimulus of osteogenesis may result in a much better outcome than expected. In addition, this case highlights that surgery should not be the first choice except for those with severe daily activity limitations, and physiotherapy is not inferior to surgical treatment.