Sturge-Weber syndrome

Schlenun s canal may be seen if blood refluxes into the canal. This might indicate elevated episcleral venous pressure caused by conditions such as a carotid-cavernous fistula, Graves orbitopathy, or Sturge-Weber syndrome. 

Nervous system An infant with drug-resistant epilepsy associated with bilateral Sturge-Weber syndrome became comatose after taking high-dose phenobarbital for a few months and regained consciousness as the serum phenobarbital concentration fell to below 40 [ig/ml. The authors suggested that patients with severe cerebrovascular diseases are more susceptible to the sedative effects of phenobarbital [232 ]. 

Wakamoto H, Nakamura Y, Ebihara T, Tokuda K, Ohmori H. Reversible coma associated with prolonged high-dose phenobarbital therapy in bilateral Sturge-Weber syndrome. J Child Neurol 2009 24(12) 1547-51. 

Capillary malformations (CMs), because of their appearance, have traditionally been referred to as port wine stains and incorrectly as capillary hemangiomas. They represent maldevelopment of capillaries and present as well demarcated skin discoloration. CMs initially have a pink or red color and become more purple as children age. CMs frequently occur with other vascular anomalies. In Klippel-Trenaunay syndrome, they occur on the trunk or lower extremity and are associated with limb hypertrophy and widespread venolymphatic malformations. In Sturge-Weber syndrome, the CM is typically in the VI (first division of the trigeminal nerve) distribution on the face and is associated with underlying ophthalmologic and leptomeningeal VMs and CMs. 

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