Systematic Reviews
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World J Orthop. Mar 18, 2022; 13(3): 329-338
Published online Mar 18, 2022. doi: 10.5312/wjo.v13.i3.329
Surgical treatment of femoral deformities in polyostotic fibrous dysplasia and McCune-Albright syndrome: A literature review
Giulio Gorgolini, Alessandro Caterini, Lorenzo Nicotra, Fernando De Maio, Kristian Efremov, Pasquale Farsetti
Giulio Gorgolini, Alessandro Caterini, Lorenzo Nicotra, Fernando De Maio, Kristian Efremov, Pasquale Farsetti, Department of Clinical Science and Translational Medicine, University of Rome Tor Vergata, Section of Orthopaedics and Traumatology, Tor Vergata Hospital, Rome 00133, Italy
Author contributions: Gorgolini G and Farsetti P designed the study and contributed to manuscript preparation and editing; Caterini A, De Maio F, and Efremov K equally contributed to data analysis and manuscript preparation; Nicotra L contributed to data collection and manuscript preparation.
Conflict-of-interest statement: The authors did not receive any funding or financial support or potential sources of conflict of interest.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.
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: Pasquale Farsetti, MD, Professor, Department of Clinical Science and Translational Medicine, University of Rome Tor Vergata, Section of Orthopaedics and Traumatology, Tor Vergata Hospital, Viale Oxford n. 81, Rome 00133, Italy. farsetti@uniroma2.it
Received: March 15, 2021
Peer-review started: March 15, 2021
First decision: October 17, 2021
Revised: October 25, 2021
Accepted: February 15, 2022
Article in press: February 15, 2022
Published online: March 18, 2022
Processing time: 366 Days and 23.9 Hours
Abstract
BACKGROUND

Surgical correction of femoral deformities in polyostotic fibrous dysplasia (PFD) or McCune-Albright syndrome (MAS), such as coxa vara or shepherd’s crook deformity, is a challenge.

AIM

To evaluate the treatment of patients with femoral deformities caused by PDF or MAS treated by osteotomies and stabilized with different methods, by analyzing the most relevant studies on the topic.

METHODS

A literature search was performed in Medline database (PubMed). Articles were screened for patients affected by PFD or MAS surgically managed by osteotomies and stabilized with different methods.

RESULTS

The initial search produced 184 studies, with 15 fulfilling the eligibility criteria of our study. Selected articles (1987-2019) included 111 patients overall (136 femurs).

CONCLUSION

Based on our results, the preferred method to stabilize corrective osteotomies is intramedullary nailing with neck cross pinning. When the deformity is limited to the proximal part of the femur, a screw or blade plate may be used, although there is a high risk of fracture below the plate. When the femur is entirely involved, a two-stage procedure may be considered.

Keywords: Polyostotic fibrous dysplasia; McCune-Albright syndrome; Coxa vara; Shepherd’s crook deformity; Femoral osteotomy; Intramedullary nailing

Core Tip: Polyostotic fibrous dysplasia and McCune-Albright syndrome commonly affect the femur, causing deformities and fractures. The proximal third of the femur represents the site where the most difficult to treat deformities are located, such as coxa vara and shepherd’s crook deformity. Surgical correction is difficult, since the fibrodysplastic bone is much weaker and more vascularized compared to normal bone and, in the most severe forms, the medullary canal is absent. The best device to stabilize corrective osteotomies seems to be the cervico-diaphyseal intramedullary nail, but the surgical technique may be difficult, because of the absence of the medullary canal and the high risk of bleeding.