Severe haemorrhage in neurofibromatoma: a lesson

Neurofibromatosis is a relatively common inherited disease of the nervous system, with a frequency of almost 1 in 3000. It is associated with a wide range of vascular abnormalities. A 62-year-old man with neurofibromatosis presented to us with a sacral haematoma. This was due to spontaneous rupture of a pre-existing neurofibromata. Upon admission the patient was in hypovolaemic shock and required aggressive resuscitation prior to surgery. Haemorrhage following trauma or spontaneous rupture is an uncommon complication of neurofibromatosis. The management is discussed with emphasis on the cause and control of bleeding from these lesions.