Fig. 8

Appearances of multiplex dysostosis. A 6-year-old boy with MPS II. Radiographs show several morphological appearances of multiplex dysostosis in long bones, with proximal humeral notching (white arrow, a), long and narrow femoral neck (curved white arrow, b), and frayed and flared tibial metaphyses (c). All segments, particularly those of the upper limb, are short and squat; they also have hypoplastic epiphyses, cortical thinning and flaring of the diaphyseal canal