Introduction
A rare congenital sporadic condition that affects the face, eyes, leptomeninges and is accompanied by vascular hamartomas.
- Has no racial or sex predilection
- Trisymptomatic involvement of skin, eye and CNS.
- Bisymptomatic involvement when skin/CNS or skin/eye.
- Monosymptomatic involvement when either skin or CNS.
Dead Giveaways
Main Diagnostic Triad:
Facial port-wine stain that extends over the trigeminal areas
Ipsilateral vascular anomoly in the brain (leptomeningeal haemangioma) --> can cause seizures
Ocular haemangioma (typically in choroid)
Ocular Giveaways:
The high pressures in episcleral vessels cause tortuosity which obstructs aqueous humour outflow 
Choroidal Haemangioma
diagnostic features
Ocular Signs:
Increased risk of ipsilateral glaucoma (30-70%), related to developmental anomaly of anterior chamber or elevated episcleral venous pressure and aqueous outflow obstruction
Vascular anomalies affecting episcleral, conjunctiva, retina, orbit
Diffuse choroidal haemangioma in 20-70% Px with choroidal thickening or retinal detachment
Port-wine stain on left side of face caan come with buphthalmos and enlarged cornea
