clinical features

Last reviewed 01/2018

The clinical features are diverse and may be the result of the primary haemangiomata or the associated disorders.

Patients usually present after adolescence with:

  • retinal haemangioblastoma, often an early sign and sometimes resulting in visual loss
  • cerebellar signs - progressive ataxia
  • compression of the fourth ventricle causing hydrocephalus
  • spinal cord haemangioblastomas present with features of cord or root compression

Fluorescein angiography confirms visual signs.