Disease severity and functional factors associated with walking performance in polyostotic fibrous dysplasia

Abstract

The purpose of this study was to determine the association between measures of disease severity, impairment, and ambulation ability in persons with polyostotic fibrous dysplasia (PFD). A cross-sectional sample of 81 patients (ages 5–57) with polyostotic fibrous dysplasia was evaluated as part of an ongoing study. Subjects were scored on the Skeletal Disease Burden Score (SDBS), completed a 9-minute walk test (9MW), manual muscle testing (MMT), and measurements of range of motion (ROM). Correlations between continuous variables were calculated using the Pearson correlation coefficient and ordinal variables by Spearman correlation coefficient. It was found that subjects with more severe disease walked slower than those with less skeletal disease, with the exception of the youngest subjects. Walking velocity was faster in subjects with better hip strength and range of motion and slower in those with bilateral coxa vara. Those subjects with more severe disease had less range of motion, were weaker at the hips, and more likely to have leg length discrepancy. Skeletal disease severity was associated with hip weakness, leg length discrepancy, and loss of range of motion. Inmost cases, findings did not differ in the presence or absence of associated endocrinopathies. Skeletal disease severity, MMT and ROM each has an impact on walking efficiency in persons with PFD. These findings suggest that treatment focused on strategies to improve or, at least, maintain hip strength and range of motion, correct leg length discrepancies and hip malalignment may help preserve ambulation ability in persons with PFD and that treatment should begin at a young age.

Scoliosis in Fibrous Dysplasia/McCune-Albright Syndrome: Factors Associated With Curve Progression and Effects of Bisphosphonates

“…However, skeletal disease burden was highly associated with scoliosis even in subjects without spinal FD, suggesting a potential causative role for functional deficits. Of note, leg length discrepancy has been independently associated with decreased hip strength, range‐of‐motion, and gait efficiency in patients with FD . These findings highlight the critical importance of monitoring for and treating leg length discrepancies as part of routine care.…”

“…Of note, leg length discrepancy has been independently associated with decreased hip strength, rangeof-motion, and gait efficiency in patients with FD. (14) These findings highlight the critical importance of monitoring for and treating leg length discrepancies as part of routine care. Our current practice includes functional evaluation at least yearly for all patients with FD involving the spine or lower extremities, and more frequently for patients experiencing complications that may alter gait dynamics, such as fractures and surgeries.…”

Fibrous Dysplasia of Bone and McCune–Albright Syndrome: A Bench to Bedside Review

“…Ideally, treatment should be orchestrated by a multidisciplinary team consisting of endocrinologists, orthopedists, dentists, physiatrists, radiologists, and other subspecialists, including ophthalmologists, otolaryngologists, and neurosurgeons, depending on disease involvement. Treatment often consists of surgery to preserve function and reduce pain, as well as conservative techniques that may include a combination of physical therapy, orthoses, avoidance of prolonged immobilization, and management of underlying endocrinopathies [66].…”

Fibrous dysplasia of bone: craniofacial and dental implications

“…Additionally, physical therapy is integral to maintain strength and range of motion. Orthopedic devices are used to correct limb length discrepancies while promoting function (Paul et al , ). Bone grafting in the axial/appendicular skeleton has been shown to have high rates of graft resorption, particularly in patients less than 18 years (Leet and Collins, ; Leet et al , ).…”