Eight-year-old Alex is in constant motion. The difference in the length of his legs and wearing a built-up sneaker doesn’t slow him down one bit. His ...
The major radiographic features of Morquio syndrome include marked platyspondyly in the thoracic and lumbar spine. The shape of the vertebrae change from ovoid, to ovoid with anterior projection, to flat. Odontoid hypoplasia with atlanto-axial instability is typically. With progression of the disease, acute thoracolumbar kyphosis is possible; the first indication of spinal cord compression is at the level of C1/C2.The skull is mildly dolichocephalic with underdevelopment of mastoid cells and flat or concave mandibular condyles. A flaring lower rib cage with pectus carinatum is typical of the thorax. A premature fusion of the ossification centers of the sternum usually occurs. The long bones are short and curved, with irregular tabulation. Metaphyses are irreguarly wide. Ossification centers tend to develop slowly. Coxa valga is characteristic, along with an abnormal femoral neck and flattening of the femoral head. Genu Valgus and a medial spur of tibial metaphysis are often times seen. The bases of the second through fifth metacarpals are conically shaped. The feet have irregular contour with delayed ossification of the tarsal bones. There is central constriction and general shortness of the metacarpals and phalanges.
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