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DYSCHONDROPLASIA WITH HAEMANGIOMATA (MAFFUCCI'S SYNDROME)

Christopher Strang 1; and Ian Rannie 1

1 The Departments of Medicine and Pathology, The Royal Victoria Infirmary, Newcastle-upon-Tyne, England

1. Maffucci's syndrome consists of dyschondroplasia (Ollier's disease) in association with cavernous haemangiomata and phlebectasia. Twenty-two cases have hitherto been described.

2. A further case is recorded with autopsy findings showing the characteristic features of the syndrome. Death was caused by a chondrosarcoma of the sphenoid, which led to subarachnoid haemorrhage, cranial nerve palsies, crossed hemiplegia and cavernous sinus thrombosis with almost total pituitary necrosis.






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Hip, Knee, Trauma, Upper limb, Foot & Ankle, Paediatrics, Oncology, Spine, Arthroplasty, General