Vaso-vagal Syncope (VVS) after Mydriatic Eyedrops in Cutaneous and Intracranial Vascular Deformations (Pascual-Castroviejo Syndrome Type II)

Purpose : To present a case of vaso-vagal syncope (VVS) after mydriatic eye drops in a patient with cutaneous hemangioma and intracerebral vascular abnormalities. Case report : A 41-year old woman presented to an ophthalmology clinic complaining of near vision disturbance. On examination, there was dilatation and telangiectasis of the conjunctival vessels on the temporal side of the right eye associated with right hemicranial and neck cutaneous involuted hemangioma ipsilateral to the conjunctival lesion. The patient experienced vaso-vagal syncope for approximately 15 minutes after mydriatic eye drops, 2 months prior to presentation. A magnetic resonance angiogram (MRA) showed right hypoplasia of the lateral and sigmoideus venous sinus ipsilateral to the external right hemangioma with predominantly compensatory drainage at the contralateral left system. At the neck vessels, the MRA showed dominant drainage through the lateral and jugular left sinus by the hypoplasia of the contralateral right system. Conclusion: This case shows the association of telangiectasis of the conjunctiva, and cutaneous hemangiomas of the head and neck with anomalies involving the central nervous system (CNS) as described by Pascual-Castroviejo in 1978, which they called cutaneous hemangioma– vascular complex syndrome. The diagnosis was made after the patient experienced VVS after mydriatic eyedrops instillation.