Shell-Like Thorax with Intercostal Space Ossification Complicating a Child with Empyema Thoracics and Underlying Rhizomelic Chondrodysplasia Punctata
Journal Title: Scholars Journal of Medical Case Reports - Year 2018, Vol 6, Issue 3
Abstract
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.
Authors and Affiliations
See WS, S H Syed Rasul, J V Simon
Keywords
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Shell-Like Thorax with Intercostal Space Ossification Complicating a Child with Empyema Thoracics and Underlying Rhizomelic Chondrodysplasia Punctata
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