Sturge-Weber sequence (SWS) is a neurocutaneous syndrome present at birth and related to migration of cells arising from the cephalic end of the neural crest during embryonic development.


The primary finding in the Sturge-Weber Sequence is a port-wine vascular malformation with a distribution that matches that of the ophthalmic and/or maxillary branches of the trigeminal nerve. It is usually unilateral but may be bilateral.


Common concomitant findings:

(1) vascular malformation involving the ipsilateral pia and arachnoid over the temporal, parietal and/or occipital lobes, which may be associated with cerebral atrophy, serpentine intracranial calcifications ("railroad tracks") and/or seizures

(2) involvement of the ipsilateral ocular choroid with buphthalmos and/or glaucoma


Additional findings may include:

(1) overgrowth of the underlying facial bones

(2) mental retardation

(3) hemiparesis

(4) cutaneous hemangiomas elsewhere in the body, including extension to the lower face, upper trunk, oral mucosa and pharynx

(5) macrocephaly

(6) microgyria

(7) abnormalities of the retinal blood vessels

(8) colobomata of the iris

(9) iris heterochromia

(10) retinal detechment

(11) strabismus

(12) coarctation of the aorta

(13) enlargement of the external ear

(14) ipsilateral dilatation of a lateral ventricle


Occassionally a patient will have a meningeal vascular malformation without visible skin involvement.


Differential diagnosis:

(1) isolated port-wine capillary hemangioma over the face


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