DOI: 10.36347/sjmcr.2018.v06i03.017

Pages: 176-180

Rhizomelic chondrodysplasia punctata (RCDP) is very rare. It is an autosomal recessive metabolic disorder which is associated with multiple orthopaedic disorders due to its related chondrodysplasia and abnormal calcification. Here, we report a child with RCDP presented with empyema thoracis. A thoracotomy was attempted for drainage and decortication. However, it failed due to the ossified intercostal spaces. The fixed right shell was inpenetrable. The procedure was abandoned in view of the risk of destruction to the chest wall from excessive traction and to avoid excessive bleeding. The clinical presentations, anaesthetic and surgical challenges will be discussed. To date, there is no similar case reported.