Metatropic dysplasia in children.

ABSTRACT

Metatropic dysplasia is a rare genetic condition characterized by progressive dwarfism. Metatropic dysplasia is defined as a short limb skeletal dysplasia characterized by dumbell like configuration of long bones, a narrow but normal length of thorax and occasionally a coccygeal appendage similar to a tail. Children born with this condition show different signs and symptoms throughout the childhood. An eight years old boy was admitted in the Paediatric ward of Bangbandhu Sheikh Mujib Medical University (BSMMU), Dhaka with the complain of swelling in the back for 7 1/2 years. On examination the boy had features suggestive of Metatropic dysplasia. Radiological findings included exaggerated curvature of the spine which was different at the different levels giving rise to "S" shaped deformity, pectus carinatum and flattening of the vertebral bodies which were variable at different levels. From the history, clinical examination and radiological findings the boy was diagnosed as a case of metatropic dysplasia.