PMC3901942_01
Radiographs of patient 4, female, MPS IVA. a AP pelvis and hips, age 9 years: dysplastic CFE; mildly tapered lower iliac wings merging into the acetabular roofs. b AP spine and ribs, age 9 years: severe scoliosis with hardware; normal ribs. c Lateral spine, age 21/2 years: gibbus; hypoplastic T12; middle beaked L1. d AP hands and wrists, age 6 years: relatively normal, but carpal ossification delay
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Fig4
Review of the radiographs of this patient (Fig. 4) shows mild but significant changes of dysostosis multiplex with dysplastic CFE.
Radiographs of patient 5, male, MPS IVA. a AP pelvis and hips, age 7 years: dysplastic superiorly eroded CFE. b Lateral spine, age 9 years: kyphosis; mildly hypoplastic T11 and T12 and posterior scalloping
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Fig5
Retrospective evaluation of the radiographs of this patient (Fig. 5) reveals no specific evidence of dysostosis multiplex.
Radiographs of patient 6, female, Maroteaux-Lamy syndrome (MPS VI). a AP pelvis and hips, age 8 years: relatively normal pelvis; CFE are a little dysplastic with asymmetrical involvement; superior flattening, but erosive notching on the right. b AP spine and ribs, age 8 years: mild scoliosis (nonspecific finding); spine otherwise normal; ribs are normal without thickening/paddle shaping; clavicles are normal. c Lateral spine, age 8 years: mildly superiorly notched T9
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Fig6
Radiographs of this patient taken at age 8 were reviewed (Fig. 6).
Radiographs of patient 7, female, MPS VI. a AP pelvis and hips, age 11 years: slightly abnormal pelvis with mild residua of inferior ileal tapering; bilateral CFE dysplasia with flattened epiphysis on the right and flat erosive changes on the left. b AP chest, age 11 years: the ribs are thick, but not paddle-shaped; clavicles are also thick. c AP hand and wrist, age 11 years: normal; no proximal pointing or brachydactyly; normal size/shape of carpal bones
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Fig7
Figure 7 shows the chest and pelvis/hip radiographs of this patient obtained at age 11 years (most recent modified skeletal survey).
Radiographs and MR image of patient 8, male, MPS VI. a AP pelvis and hips, age 12 years: hypoplastic CFE. b AP chest, age 12 years: normal. c Lateral spine, age 12 years: superior notched T12 vertebral body. d MRI lateral spine, age 12 years: anterior/superior vertebral body irregularity/notching with intact region of cartilaginous anlage. e AP hands and wrists, age 10 years: normal
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Fig8
On review, radiographs of this patient (Fig. 8) reveal hypoplastic CFE and a single superiorly notched T12 vertebral body (nonspecific finding).