Persistent second cervical intersegmental artery in a case of possible PHACE syndrome

Area of Science:

• Vascular anatomy
• Embryological development
• Congenital anomalies

Background:

• The proximal vertebral artery (VA) typically arises from the persistent sixth cervical intersegmental artery originating from the subclavian artery.
• Variations in VA origin, including from other cervical or thoracic intersegmental arteries, are documented but less common.
• Persistent second cervical intersegmental arteries are exceptionally rare, with very few cases reported.

Observation:

• A patient presented with multiple complex arterial variations.
• These included a right persistent second cervical intersegmental artery originating from the common carotid artery.
• Additional anomalies observed were distal VA duplication, a circumflex aortic arch, and segmental internal carotid artery agenesis.

Findings:

• The case demonstrates an unusual origin for a persistent second cervical intersegmental artery, arising from the common carotid artery.
• The combination of VA duplication, circumflex aortic arch, and carotid agenesis represents a unique constellation of vascular anomalies.
• This presentation expands the known spectrum of cervical intersegmental artery persistence and associated vascular malformations.

Implications:

• This case contributes to the understanding of rare vertebral artery developmental pathways and anatomical variations.
• The findings may inform the diagnosis and management of patients with complex congenital vascular anomalies, particularly in the context of syndromes like PHACE.
• Further research into the embryological basis of such extensive arterial variations is warranted to elucidate their pathogenesis.