Surgical Treatment of Fibrous Dysplasia in the Appendicular Skeleton: A Systematic Review of the Last 10 Years

Abstract

Surgical Treatment of Fibrous Dysplasia in the Appendicular Skeleton: A Systematic Review of the Last 10 Years

Introduction:
Fibrous dysplasia (FD) is a benign bone disorder caused by a somatic mutation in the GNAS gene, leading to the replacement of normal bone with immature fibro-osseous tissue. It may present in monostotic or polyostotic forms and can significantly impair the structure and function of the appendicular skeleton. Surgical treatment aims to alleviate pain, prevent fractures, correct deformities, and restore function; however, indications and techniques vary widely depending on anatomical location, extent of disease, and patient symptoms.

Objective:
To describe and analyze the clinical, functional, and radiological outcomes of surgical treatment for fibrous dysplasia in the appendicular skeleton, based on a systematic review of the past 10 years.

Methodology:
A systematic search was conducted using the terms:
"Fibrous Dysplasia" OR "fibrous dysplasia” OR "monostotic fibrous dysplasia"OR "polyostotic fibrous dysplasia" AND "Orthopedic Procedures" OR orthopedic OR orthopaedic OR "fracture fixation" OR  "osteosynthesis", limited to the last 10 years. From 75 articles retrieved, 18 original studies published in English or Spanish were included, of appendicular skeleton, after excluding case reports and previous systematic reviews.

Results:
Out of 75 articles reviewed, 18 met the inclusion criteria.

Most studies focused on lesions of the proximal femur and long bones of the lower limbs. The most commonly used surgical approaches were:

• Intramedullary nailing, particularly effective for polyostotic disease or significant femoral deformities, offering good functional outcomes and low recurrence rates.
• Angulated plate fixation and corrective osteotomies, useful for managing “shepherd’s crook” deformities, with success rates up to 72%, though iliotibial band pain was reported in some cases.
• Allogeneic cortical strut grafts, especially for diaphyseal fractures, showed good consolidation, though some authors noted the potential for dysplastic transformation of autografts.
• Minimally invasive techniques with calcium phosphate or alpha-tricalcium phosphate grafting in monostotic lesions provided satisfactory pain control and limited radiological progression.
• Total hip arthroplasty, in advanced FD with secondary osteoarthritis, showed favorable short-term results using uncemented long-stem implants and 3D preoperative planning.

Reported complications included persistent pain, deformity progression, implant failure, and revision surgeries in complex cases.

Conclusions:
Surgical management of fibrous dysplasia in the appendicular skeleton must be tailored to disease extent, anatomical location, and clinical presentation. Intramedullary nailing is particularly effective in polyostotic disease, while angulated plating and osteotomies are preferred for proximal femoral deformities. Bone grafts and arthroplasty have specific roles depending on patient needs. Long-term follow-up is essential to monitor disease progression and manage complications.

Key words:
Fibrous dysplasia, surgical treatment, orthopedic treatment