Table 2.
Affected bones, fractures, and surgical operations in pediatric patients with polyostotic fibrous dysplasia (n = 16).
| Patient number | Age category (years)a | Height SDSa | Affected bones | Craniofacial involvement | Fractures | Orthopedic (or other) surgery | Orthopedic disabilities/problems | Chronic pain/head aches |
|---|---|---|---|---|---|---|---|---|
| 1 | 10–15 | −0.5 | Ribs, ilium, and femur | − | + | Fixation of femoral fracture | − | |
| 2 | 15–20 | +0.2 | Orbital and parietal bones | + | − | − | − | |
| 3 | 15–20 | −0.5 | Pelvis and femur | − | + | Epiphysiodesis and spondylodesis | Disabling deformity of pelvis and hip | |
| 4 | 10–15 | −0.2 | Orbital, maxillar, frontal, sphenoidal, temporal, mandibular bones, humerus, fibula, tibia, and femur | + Stenosis of nervus opticus | + | Fixation of femoral fracture; osteotomy | − | + Headaches |
| 5 | 15–20 | +0.4 | Sphenoidal, frontal and orbital bones unilaterally | + Stenosis of nervus opticus | − | (Decompression of optic nerve) | − | + Headaches |
| 6 | 15–20 | −1.7 | Skull base, parietal, occipital, orbital, temporal, humerus, fibula, tibia, and femur; all unilaterally | + | + | Fixation of femoral fracture times 3; correction of coxa valgus deformity | − | + Headaches |
| 7 | 15–20 | +0.5 | Occipital, parietal, maxillar, mandibular bones, humerus, radius, femur, and tibia | + | + | − | − | |
| 8 | 5–10 | N/Ab | Ceiling of orbita, maxillar, mandibular, frontal, ethmoidal, sphenoidal, temporal, occipital, and parietal bones, femur, tibia, and fibula | + | + | Fixation of femoral neck bilaterally; osteotomy | Severe scoliosis | |
| 9 | 15–20 | +0.1 | Sphenoidal, maxillar, and ceiling of orbita unilaterally; frontal bone, foramina optica bilaterally | + Stenosis of nervus opticus | − | − | − | + Headaches |
| 10 | 15–20 | −1.3 | Skull base (mostly unilaterally): frontal, sphenoidal, orbital, maxillar, and temporal bones; pelvis, femur bilaterally | + Stenosis of nervus opticus | + | Femoral osteosynthesis bilaterally | − | Chronic pain |
| 11 | 10–15 | +1.6 | Femur and fibula bilaterally; tibia, skull base (widely), mandibular, zygomatic, maxillar bones, humerus, and orbital bones | + Stenosis of nervus opticus | + | − | Varus malalignment of hip | Chronic pain |
| 12 | 10–15 | −0.3 | Frontal, parietal, and ethmoidal bones, concha superior, sinus maxillaris unilaterally, and sinus ethmoidalis bilaterally | + | + | (Skull operation) | − | |
| 13 | 10–15 | +0.5 | Maxillar and sphenoidal bones, fossa cranii media | + | − | − | − | |
| 14 | 10–15 | −1.3 | Tibia (widely), fibula unilaterally | − | + | − | − | |
| 15 | 5–10 | +1.2 | Pelvis, femur, tibia, talus, humerus, and radius | − | + | + Fixation of femur fracture with osteosynthesis; osteotomy | − | |
| 16 | 10–15 | −0.1 | Maxillar bone bilaterally | + | − | − | − | + Headaches |
SDS, standard deviation score.
aAge and height SDS at last follow-up visit.
bHeight measure not possible; arm span used as a proxy for height.