The deformation of the carpal bones and the rod-shaped changes of the metacarpals are seen in mucopolysaccharide storage disease type VI, also known as Maroteaux-Lamy syndrome and polytrophic dwarfism. It is an autosomal recessive disorder. How is the diagnosis made when a patient presents with a deformation of the carpal bones and a rod-shaped change in the metacarpals? The following is a brief introduction: The diagnosis of this type is based on the appearance of typical clinical symptoms, excessive urinary excretion of dermatopoietin sulfate and the appearance of obvious heterochromatic granules (Reilly vesicles) in leukocytes or skin or fibroblast cultures showing N-acetylgalactosamine-4-sulfate esterase deficiency. 1. Extremities: The femoral and tibial tuberosities have diaphyseal abnormalities, and many cases have epiphyseal plate and kilophysis deformities. The long tubular bones of the upper extremities have more pronounced changes than those of the lower extremities. The short bone changes of the hands and feet vary from person to person, but all have deformities, showing stick-like metacarpals and underdeveloped or deformed wrist bones. 2. Spine: The changes at the thoracolumbar spine are obvious, with small vertebrae, bird’s beak shape or other deformities. The spine may also have kyphosis. The ribs are paddle-shaped, accompanied by the sternum protruding forward, which can make the chest funnel-shaped. Rib deformation can make the chest bell-like. 3.Pelvis: the iliac wing is underdeveloped and deformed, the acetabulum is small, and the femoral epiphysis is irregular and flattened. 4.Cranium: the butterfly saddle is enlarged and “B”-shaped, with sclerotic mastoid process, dentate hypoplasia and herringbone suture fusion.