Fibrous Dysplasia of the Proximal Part of the Femur. Long-Term Results of Curettage and Bone-Grafting and Mechanical Realignment*

Jt, Guille; Sj, Kumar; Gd, MacEwen

doi:10.2106/00004623-199805000-00005

Cited by 129 publications

(146 citation statements)

References 12 publications

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“…Later, cortical struts and subtotal resection of the lesions were proposed [53], and others have proposed the use of cryosurgery in association with grafting [54]. However, others have not been able to reproduce successful longterm results with these techniques, and have noted disappearance of bone graft observed over time and no decrease in the size of the lesion [49,51,55]. Since most published cohorts of surgically treated FD are small, it remains controversial as to whether bone grafting should be used and if so what kind of grafting materials should be employed.…”

Section: The Role Of Bone Graftingmentioning

confidence: 99%

Current approach to fibrous dysplasia of bone and McCune–Albright syndrome

Leet

Collins

2007

Journal of Children's Orthopaedics

106

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Fibrous dysplasia (FD) of bone is an uncommon disease caused by sporadic, congenital mutations in the cAMP regulating protein, Gsα. It is an example of somatic mosaicism in which a wide spectrum of disease is possible. Widespread skeletal involvement is often associated with varying combinations of café-au-lait skin spots, and/or endocrine dysfunction (precocious puberty, renal phosphate wasting, hyperthyroidism, and/or growth hormone excess). Unrecognized and untreated endocrine dysfunction can exacerbate the skeletal disease. The diagnosis is usually established on clinical grounds on the basis of physical examination and typical radiographic appearance. Occasionally, gene testing of affected tissue may be helpful. The skeletal sites involved with disease are established at an early age, and the complications of fracture deformity are most pronounced in childhood. Bone pain in the absence of a fracture is more common in adults, but can also be present in children. Treatment with bisphosphonates is usually effective at relieving pain, but probably has no effect on the natural history of the disease. Scoliosis, which was previously thought to be an uncommon occurrence, has been shown to be common and progressive, and as such, warrants investigation and, when necessary, surgical treatment. The surgical management of FD remains challenging. Timing and technique remain controversial, but some consensus exists in that grafting materials (of any type) usually fail and should not be a central aspect of the surgical approach. Intramedullary devices are in general superior to side plates and screws. In extremely widespread disease with very early fracture and deformity, no surgical approach will affect final functional outcome. Efforts should be made for the initiation of international collaborative studies to better define optimal surgical approaches to the treatment of this challenging disease.

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Section: The Role Of Bone Graftingmentioning

confidence: 99%

Current approach to fibrous dysplasia of bone and McCune–Albright syndrome

Leet

Collins

2007

Journal of Children's Orthopaedics

106

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“…One patient took longer to return to full activity due to pathological fracture of another limb. The clinical score was in accordance with the modified criteria described by Guille et al (8). The clinical score improved from an average of 4.4 (range, 3-6) points prior to surgery to an average of 8 (range, 6-10) points at the last follow-up.…”

Section: Patient Recovery and Symptomsmentioning

confidence: 85%

“…However, it is difficult to provide sufficient stability with plates and screws in the vicinity of the weakened bones, and fracture or refracture may occur due to the stress-shielding effect of the distal part of the plate. Guille et al (8) suggested that a refracture may easily occur after the plate and screws are removed in patients with a lesion involving a large area. O'Sullivan and Zacharin (16) reported that intramedullary nailing and bisphosphonate treatment of 10 femurs with McCune-Albright syndrome prevented fractures and resulted in improved walking.…”

Section: Discussionmentioning

confidence: 99%

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Intramedullary nailing for fibrous dysplasia of lower limbs

et al. 2012

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Abstract. Fibrous dysplasia (FD) of the bone is rare and self-limiting. However, lesion expansion may occur, causing pain, deformity and pathological fracture, in which case surgery is occasionally required. Indicators of FD have not been previously described, although there are several surgical procedures reported (curettage, curettage and graft, and internal fixation). In this study we discuss whether intramedullary nailing of these lesions could result in more favorable outcomes in correcting deformities, including the prevention of secondary fractures and maintenance of the bone, compared to other internal fixation methods. A total of 39 patients with FD of the bone treated with intramedullary nailing were retrospectively analyzed. The surgical procedures involved curettage, grafting and intramedullary nailing. No infection, thromboembolism or other notable complications occurred. The patients resumed full activities of daily living. At the last follow-up, 33 patients presented no pain and seven patients had occasional mild pain. The clinical score according to the modified criteria of Guille improved from an average of 4.4 points prior to surgery to an average of 8 points following surgery. The neck shaft angle of the femur improved from an average of 90˚ prior to surgery to 125˚ following surgery. Intramedullary nailing may be used to correct deformity and prevent pain and refracture in FD of the bone of the lower limbs with large lesions, pathological fracture or deformities. All patients were allowed full athletic recovery following surgery.

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“…Stephenson et al [8] described satisfactory outcomes in 18 of 21 patients under the age of 18 who had lower extremity lesions treated by internal fixation. Guille et al [13] proposed internal fixation (with no distinct advantage of one type of internal fixation over another) with valgus osteotomy early in the course of the disease as the best treatment approach to patients with fibrous dysplasia of the proximal femur with varus deformity.…”

Section: Discussionmentioning

confidence: 99%

Intramedullary rod fixation of fibrous dysplasia without use of bisphosphonates

Gaski

Hansen

Willis

et al. 2013

Journal of Children's Orthopaedics

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Purpose Controversy exists regarding approach to treatment of pediatric patients with fibrous dysplasia. Methods We retrospectively reviewed medical records of seven patients who were treated at our institution for fibrous dysplasia by intramedullary rod fixation without bisphosphonate supplementation. Results Seven patients with a total of ten fibrous dysplasia lesion sites surgically treated by intramedullary rod fixation were included. Of these ten lesion sites, eight demonstrated pathologic fracture at the time of fixation. Complete fracture healing was observed in all eight sites, with no incidence of recurrent pathologic fractures examined radiographically. There were no major infections or neurologic deficits, and lesions appeared to stabilize. Conclusions In this series, intramedullary rod fixation proved to be successful in treatment of acute pathologic fracture and incompletely healed fibrous dysplasia lesions. We observed partial resolution of fibrous dysplasia lesions at all ten sites without significant long-term complications. Following treatment, there were no refractures. Level of evidence Level IV, case series.

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Fibrous Dysplasia of the Proximal Part of the Femur. Long-Term Results of Curettage and Bone-Grafting and Mechanical Realignment*

Cited by 129 publications

References 12 publications

Current approach to fibrous dysplasia of bone and McCune–Albright syndrome

Current approach to fibrous dysplasia of bone and McCune–Albright syndrome

Intramedullary nailing for fibrous dysplasia of lower limbs

Intramedullary rod fixation of fibrous dysplasia without use of bisphosphonates

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