DYSCHONDROPLASIA WITH HAEMANGIOMATA (MAFFUCCI'S SYNDROME)

Abstract

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.