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Joint Diseases and
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Original Article · Open access

Fibrous dysplasia: Clinical features, deformities, management, and outcomes

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Mustafa Onur Karaca

Department of Orthopedics and Traumatology, Ankara University Faculty of Medicine, Ankara, Türkiye

ORCID: 0000-0003-0783-510X

Mustafa Özyıldıran

Department of Orthopedics and Traumatology, Sandıklı State Hospital, Afyonkarahisar, Türkiye

ORCID: 0000-0002-2187-9267

Mahmut Kalem

Department of Orthopedics and Traumatology, Ankara University Faculty of Medicine, Ankara, Türkiye

ORCID: 0000-0002-8598-0938

Yunus Şamil Gönüldenk

Department of Orthopedics and Traumatology, Ankara University Faculty of Medicine, Ankara, Türkiye

ORCID: 0009-0007-5350-288X

Kerem Başarır

Private Orthopedic Surgeon, Ankara, Türkiye

ORCID: 0000-0001-6247-8737

Hüseyin Hakan Kınık

Department of Orthopedics and Traumatology, Ankara University Faculty of Medicine, Ankara, Türkiye

ORCID: 0000-0003-4768-8415

Hüseyin Yusuf Yıldız

Department of Orthopedics and Traumatology, Ankara University Faculty of Medicine, Ankara, Türkiye

ORCID: 0000-0002-7669-6206

Abstract

Objectives: The aim of this study was to evaluate the rate of proximal femoral involvement and the incidence of shepherd’s crook deformity in patients with fibrous dysplasia (FD) and to identify possible factors associated with the development of shepherd’s crook deformity.

Patients and methods: Between January 1990 and December 2022, a total of 158 consecutive patients (78 males, 80 females; mean age: 23.8 ± 15.0 years; range, 3 to 71 years) diagnosed with FD were retrospectively analyzed. Presenting symptoms, lesion location, radiological findings, treatment modalities, development of deformity, postoperative complications, and the need for secondary surgery were recorded.

Results: The mean follow-up was 64.2 ± 38.4 (range, 24 to 210) months. Among the patients, 125 had monostotic and 33 had polyostotic lesions. Of the 215 lesions identified, 83 were treated surgically, while 132 were managed with clinical observation. Shepherd’s crook deformity was observed in nine of 75 proximal femoral lesions. The deformity was significantly more common in polyostotic than in monostotic lesions (25.0% vs. 5.9% respectively; p = 0.026). McCune-Albright syndrome (MAS) was present in two patients, both exhibiting shepherd’s crook deformity, whereas the rate was 9.6% among patients without MAS. Valgus osteotomy was performed in all nine patients with shepherd’s crook deformity, improving the mean neck-shaft angle from 90.5° (range, 85 to 105°) preoperatively to 125.6° (range, 120 to 130°) postoperatively. In six of these patients, no marked loss in the postoperative neck-shaft angle was observed, while three patients required secondary surgery due to recurrence of the deformity.

Conclusion: Most lesions can be managed successfully with observation, while surgery is indicated for the eradication of symptomatic lesions, prevention of pathological fractures, and correction of deformities. Shepherd’s crook deformity seems to be more common in patients with polyostotic lesions and MAS. Although valgus osteotomy can achieve radiographic improvement, recurrence of deformity and the need for multiple surgeries are not uncommon.

Declarations

Author Contributions

M.O.K., K.B., H.Y.Y.: Idea/concept; M.O.K., M.Ö., Y.Ş.G.; Design, analysis/interpretation; M.O.K., H.Y.Y., M.K., H.H.K.; Control/supervision; M.Ö., Y.Ş.G.: Data collection/processing, literature review; M.O.K., M.Ö.: Writing; M.O.K., H.Y.Y.: Critical review.

Conflict of Interest

The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.

Financial Disclosure

The authors received no financial support for the research and/or authorship of this article.

Data Sharing Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.

AI Disclosure

The authors declare that artificial intelligence (AI) tools were not used, or were used solely for language editing, and had no role in data analysis, interpretation, or the formulation of conclusions. All scientific content, data interpretation, and conclusions are the sole responsibility of the authors. The authors further confirm that AI tools were not used to generate, fabricate, or ‘hallucinate’ references, and that all references have been carefully verified for accuracy.

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