Clinical features
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.
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