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This syndrome has also being called PHACE syndrome and hemangio-vascular complex syndrome. Pascual Castroviejo type II syndrome is an sporadic condition. It consists of posterior fossa malformations, plaque like facial hemangioma of variable size (Figure 310.1), arterial abnormalities, and eye abnormalities.


Figure 310.1. Pascual Castroviejo type II syndrome. [A] Extensive bilateral facial hemangioma, [B] Raised hemangioma.

The most frequent posterior fossa abnormalities are Dandy-Walker syndrome and partial cerebellar hypoplasia (Figure 310.2 [A] [B]). The most frequent arterial abnormalities include coartation of the aorta, cerebral arteriovenus malformations, hypoplasia of the carotid and vertebrobasilar arterial trunks, and persistent embryonic carotid-vertebrobasilar anastomosis (Figure 310.2 [C]). Cataracts and glaucoma may be present. Subglottic hemangioma may lead to respiratory problems. This syndrome should be suspected in any newborn with plaque facial hemangioma. A patient with plaque-like facial hemagioma should undergo an MRI the brain and an MRA of the brain and neck, ophthalmology evaluation, cardiac evaluation including determination of the blood pressure in the upper and lower limbs, and otolaryngology consultation.


Figure 310.2. Pascual Castroviejo type II syndrome. [A] transverse MRI: cerebellar hemisphere hypoplasia, [B] sagittal MRI: cerebellar hemisphere hypoplasia, [C] persistent of the trigeminal artery.


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