Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Mar 18;13(3):329-338.
doi: 10.5312/wjo.v13.i3.329.

Surgical treatment of femoral deformities in polyostotic fibrous dysplasia and McCune-Albright syndrome: A literature review

Affiliations

Surgical treatment of femoral deformities in polyostotic fibrous dysplasia and McCune-Albright syndrome: A literature review

Giulio Gorgolini et al. World J Orthop. .

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

PubMed Disclaimer

Conflict of interest statement

Conflict-of-interest statement: The authors did not receive any funding or financial support or potential sources of conflict of interest.

Figures

Figure 1
Figure 1
Flow diagram of the search process.

References

    1. Bianco P, Kuznetsov SA, Riminucci M, Fisher LW, Spiegel AM, Robey PG. Reproduction of human fibrous dysplasia of bone in immunocompromised mice by transplanted mosaics of normal and Gsalpha-mutated skeletal progenitor cells. J Clin Invest. 1998;101:1737–1744. - PMC - PubMed
    1. Hart ES, Kelly MH, Brillante B, Chen CC, Ziran N, Lee JS, Feuillan P, Leet AI, Kushner H, Robey PG, Collins MT. Onset, progression, and plateau of skeletal lesions in fibrous dysplasia and the relationship to functional outcome. J Bone Miner Res. 2007;22:1468–1474. - PubMed
    1. Javaid MK, Boyce A, Appelman-Dijkstra N, Ong J, Defabianis P, Offiah A, Arundel P, Shaw N, Pos VD, Underhil A, Portero D, Heral L, Heegaard AM, Masi L, Monsell F, Stanton R, Dijkstra PDS, Brandi ML, Chapurlat R, Hamdy NAT, Collins MT. Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortium. Orphanet J Rare Dis. 2019;14:139. - PMC - PubMed
    1. Leet AI, Collins MT. Current approach to fibrous dysplasia of bone and McCune-Albright syndrome. J Child Orthop. 2007;1:3–17. - PMC - PubMed
    1. Bunnell WP. The natural history of idiopathic scoliosis before skeletal maturity. Spine (Phila Pa 1976) 1986;11:773–776. - PubMed