Other Vascular Malformations
The underlying vascular malformation can be seen to be causing deformity of the nose.
A-V malformation of the hand - causing no functional problems
Features
Deeper blood vessel malformations do occur, some of the most extreme being Klippel-Trenaunay syndrome which comprises of severe lesions on the limbs with bone hypertrophy and port-wine stain.
Some children with trigeminal distribution port-wine stains have corresponding capillary haemangiomas in the brain, sometimes leading to contralateral focal fits. This is known as Sturge-Weber syndrome (incidence 2/100,000).
Investigation and Treatment
MRI scanning is the investigation of choice. Embolisation of some malformations may be possible.
