Congenital Horner Syndrome with Heterochromia Iridis Associated with Ipsilateral Internal Carotid Artery Hypoplasia

BACKGROUND: Horner syndrome (HS), also known as Claude-Bernard-Horner syndrome or oculosympathetic palsy, comprises ipsilateral ptosis, miosis, and facial anhidrosis. CASE REPORT: We report herein the case of a 67-year-old man who presented with congenital HS associated with ipsilateral hypoplasia of the internal carotid artery (ICA), as revealed by heterochromia iridis and confirmed by computed tomography (CT). CONCLUSIONS: CT evaluation of the skull base is essential to establish this diagnosis and distinguish aplasia from agenesis/hypoplasia (by the absence or hypoplasia of the carotid canal) or from acquired ICA obstruction as demonstrated by angiographic CT.

The missed missing hole / Revelações de um canal ausente

At times in clinical neurology, the identification of a subtle clinical or radiological sign can lead to prompt diagnosis of a very rare or difficult case. We report on a patient who presented with untreatable headache and unilateral ptosis. Computed tomography (CT) scan of the head did not reveal any structural cause. Magnetic resonance angiogram showed absence of left internal carotid artery, which was eventually confirmed by a catheter angiography. Reviewing the case, it emerged that a feature on the initial CT scan "bone window" would have confirmed the diagnosis, had it been searched for: the underdeveloped carotid canal, which is a consequence and a marker of internal carotid artery agenesis.

Em algumas circunstâncias, o reconhecimento de um sinal clínico ou radiológico sutil pode tornar simples o diagnóstico de um caso raro ou muito difícil em neurologia clínica. Relatamos o caso de uma paciente que apresentava cefaleia intratável e ptose palpebral unilateral. A tomografia computadorizada (TC) de crânio não permitiu identificar nenhuma causa estrutural. A ressonância magnética evidenciou ausência da artéria carótida interna esquerda, posteriormente confirmada por arteriografia convencional. Retrospectivamente, descobriu-se que um dado da janela óssea da primeira TC de crânio teria confirmado o diagnóstico, tivesse ele sido pesquisado: o hipodesenvolvimento do canal carotídeo, que é uma consequência e um marcador de agenesia da artéria carótida interna.

Common Carotid Artery Agenesis: Duplex Ultrasonographic Findings

BACKGROUND: Agenesis of the common carotid artery (CCA) resulting in separation of the origin of the external carotid artery (ECA) and internal carotid artery (ICA) from the aortic arch is rare. Fewer than 25 cases have been reported, and correlative ultrasound data were available for only 1 of them. CASE REPORT: A 52-year-old woman visited the hospital with a 3-day history of vertigo and headache. Color-coded duplex ultrasonography performed to evaluate the carotid and vertebral arteries revealed a normal configuration on the left side. However, the right CCA could not be found; instead, there were two vessels of approximately equal size in close proximity to each other. The cerebral angiographic findings were consistent with the ultrasonographic findings. The ECA and ICA originated directly from the brachiocephalic trunk, and the ECA arose proximal to the ICA. CONCLUSIONS: The ultrasonographic findings revealed absence of the CCA, the ECA and ICA originating separately from the aortic arch. Color-coded duplex ultrasonography appears to be an effective and sensitive method for detecting absence of the CCA. These findings should help to further our understanding of the embryologic development of the carotid arteries.

Bilateral Agenesis of the Internal Carotid Artery: Case Report

Unilateral or, particularly, bilateral congenital agenesis of the internal carotid artery is a rare anomaly. We report an occurrence of the condition, arising bilaterally, and report the findings of magnetic resonance imaging and magnetic resonance angiography.