Pascual-Castroviejo type II syndrome (P-CIIS). Importance of the presence of persistent embryonic arteries

Abstract

The presence of embryonic arteries, especially the trigeminal, and connections between branches of the internal and external carotids, mainly through the internal maxillary and ophthalmic arteries, ensure the cerebral supply in the P-CIIS despite the progressive involvement of the cerebral arteries.

Persistent Fetal Intracranial Arteries

“…A persistent trigeminal artery has been reported in the context of neurofibromatosis type 1, Klippel-Feil syndrome (Paksoy et al 2004), and Moyamoya disease (Azab et al 2012). The incidence of this artery is much higher among children with posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, and other cardiac defects and eye abnormalities (PHACE syndrome) (12-16%) than reported from other large cerebral angiographic series (0.1-0.2%; Pascual-Castroviejo et al 2011).…”

Corpus callosum arteriovenous malformation with persistent trigeminal artery

“…33 In reported cases of Pascual-Castroviejo type II syndrome, the presence of embryonal vessels like PTA and collaterals between the external carotid and internal carotid arteries ensure adequate cerebral blood supply and thus prevent cerebrovascular strokes in these patients. 34 Radiological findings of PTA PTA is usually diagnosed with a conventional angiogram or with a MR or CT angiogram. However, PTA may be missed if its diameter is less than 2 mm.…”

Brain Perfusion SPECT in Patients with PHACES Syndrome Under Propranolol Treatment

“…17 At that point the presence of persistent embryonic arteries has a special importance (trigeminals and proatlantals) because these arteries do not undergo any changes and continue their function of anastomosis between the carotid and vertebrobasilar systems, ensuring a better and early cerebral supply when the main cerebral arteries are narrowed. 18 These might not be the only stroke risk factors involved in PS, since others have also been described, such as cardiac malformations, sickle cell anemia, chromosomal aberrations, neurocutaneous syndromes, trauma, drugs or infections (meningitis, encephalitis, sepsis, etc.). 19 For example, heart disease increases the risk of stroke, particularly in the perioperative period, but these patients will still remain at increased risk even years after surgery, implying the existence of an association between the stroke and the cardiopathy itself.…”