Persistent Primitive Hypoglossal Artery (PPHA) – A Rare Anomaly with Literature Review

Abstract

Persistent primitive hypoglossal artery (PPHA) is a rare embryonic carotid vertebrobasilar artery anastomosis. Hypoglossal artery arises from the internal carotid artery (ICA) between the C1 and C2 vertebral levels and traverses through the hypoglossal canal to join the vertebro-basilar system. We present a rare case of an anomalous right sided PPHA as a sole supply to posterior circulation of brain with absent/hypoplastic bilateral vertebral arteries in a two year child who had presented with acute left sided haemiplegia. Three dimensional time of flight magnetic resonance angiography identified an anomalous vessel arising from the right internal carotid artery at the level of axis vertebra and joining the vertebra-basilar arterial system after coursing through the right hypoglossal canal. This anomaly when present may predispose the person to aneurysm formation, ischaemia in the posterior circulation and atherosclerotic disease of the intracranial vessels.

Benign paroxysmal positional vertigo in a patient with persistent hypoglossal artery and bilateral madelung deformity

“…PHA arises during the early stages of embryonic development ( Avcu et al, 2009 , Coulier, 2018 , MR, 2016 , Fantini et al, 1994 ). PHA represents an off-branching from the internal carotid artery, situated at C1-C3 ( Avcu et al, 2009 , Coulier, 2018 , MR, 2016 , Fantini et al, 1994 ).…”

“…PHA arises during the early stages of embryonic development ( Avcu et al, 2009 , Coulier, 2018 , MR, 2016 , Fantini et al, 1994 ). PHA represents an off-branching from the internal carotid artery, situated at C1-C3 ( Avcu et al, 2009 , Coulier, 2018 , MR, 2016 , Fantini et al, 1994 ). Pre-segmental arteries form a network of anastomoses that play an integral role in neurological maturation in the form of vascular accommodation for the developing fetal brain ( Coulier, 2018 , MR, 2016 ).…”

“…PHA represents an off-branching from the internal carotid artery, situated at C1-C3 ( Avcu et al, 2009 , Coulier, 2018 , MR, 2016 , Fantini et al, 1994 ). Pre-segmental arteries form a network of anastomoses that play an integral role in neurological maturation in the form of vascular accommodation for the developing fetal brain ( Coulier, 2018 , MR, 2016 ). In particular, the forebrain and hindbrain serve as beneficiaries of this vascular network ( Coulier, 2018 , MR, 2016 ).…”

“…Pre-segmental arteries form a network of anastomoses that play an integral role in neurological maturation in the form of vascular accommodation for the developing fetal brain ( Coulier, 2018 , MR, 2016 ). In particular, the forebrain and hindbrain serve as beneficiaries of this vascular network ( Coulier, 2018 , MR, 2016 ). After 7–10 days, such anastomoses are typically obliterated.…”

“…Persistent hypoglossal artery (PHA) is a rare congenital carotid-basilar anastomosis ( Avcu et al, 2009 , Coulier, 2018 , MR, 2016 , Fantini et al, 1994 , Jin et al, 2018 ). While persistent trigeminal artery (PTA) is the most common carotid-vertebrobasilar anastomoses with prevalence estimated to be 0.1–0.2%, PHA is the second most common with reported prevalence of 0.03–0.09% PHA has been associated with various cardiovascular events, including embolic stroke ( Avcu et al, 2009 , Jin et al, 2018 ).…”