Background: Mucopolysaccharidosis type VI (MPS VI), also known as Manteaux-Lamy syndrome, is an autosomal recessive lysosomal storage disorder caused by deficiency of the enzyme arylsulfatase B(ARSB). This syndrome is progressive and affects many tissues and organs, leading to inflammation and scarring. The classic clinical features of Maroteaux-Lamy syndrome are significant impairment of the osteoarticular system with dysostosis multiplex, short stature and motor dysfunction.
View Article and Find Full Text PDFIntroduction And Importance: Concurrent ipsilateral femoral malunion and nonunion present substantial clinical challenges requiring comprehensive surgical interventions. We describe a unique case of a 65-year-old male with these complications who was treated with a proximal femoral osteotomy, radical sequestrectomy, and free fibula graft.
Case Presentation: The patient underwent over 10 years of multiple surgical interventions, including hardware removal, local debridement, antibiotic-loaded cement spacer placement, autologous bone grafting, and external fixator applications, yet infectious non-union persisted.