Agenesis of Internal Carotid Artery in a Child with Ipsilateral Horner's Syndrome

Fons, Carmen; Vasconcelos, Mônica Miranda; Vidal, Mariona; Vilallonga, Ramón; Capdevila, Antonio; Sánchez, Laia; Campistol, J

doi:10.1177/0883073808321049

Cited by 12 publications

(3 citation statements)

References 7 publications

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“…26 According to other reports, ICA agenesis is associated with developmental abnormalities in multiple organs (such as congenital Horner syndrome, Goldenhar syndrome, and Klippel-Feil syndrome). 33,38,39 These diseases are affected by dysplasia of the arterial arch or temporal bone primordium in the 4-to 6-weekold embryo (ICA development stage). 49 Therefore, when young people present with SAH or developmental abnormalities, it is important for them to undergo radiologic examination to rule out ICA agenesis.…”

Section: Discussionmentioning

confidence: 99%

The clinical presentation and collateral pathway development of congenital absence of the internal carotid artery

Zhang

Wang

et al. 2018

Journal of Vascular Surgery

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From analysis of our 10 cases of ICA agenesis and our review of the relevant literature, we conclude that young patients with ICA agenesis may present with developmental delay, subarachnoid hemorrhage, or other developmental abnormalities, whereas older patients most commonly present with transient neurologic events. Complications of carotid agenesis are related to specific anatomic subtypes and the resulting collateral circulation development.

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Section: Discussionmentioning

confidence: 99%

The clinical presentation and collateral pathway development of congenital absence of the internal carotid artery

Zhang

Wang

et al. 2018

Journal of Vascular Surgery

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“…Absence of oculosympathetic stimulation decreases the production of melanin. As such, a hypopigmented iris indicates an oculosympathetic pathway injury early in life 15,16…”

Section: Discussionmentioning

confidence: 99%

Congenital agenesis of internal carotid artery with ipsilateral Horner presenting as focal neurological symptoms

Farhat¹,

Ahdab²,

Hosseini

2011

VHRM

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Internal carotid artery (ICA) agenesis is a rare developmental anomaly and is most frequently asymptomatic, but it may also present as cerebrovascular accidents. The association with Horner’s syndrome is exceptional. We present three cases of agenesis of ICA associated with Horner’s syndrome and hypochromia iridum presenting as focal neurological symptoms. A system of collaterals develops as a consequence of agenesis of the ICA, making the majority of cases asymptomatic. Three types of collateral circulations have been described. These collaterals increase the risk of aneurysm formation and the occurrence of life-threatening subarachnoid hemorrhages. The association of congenital Horner’s syndrome and hypochromia iridum without anhidrosis is highly suggestive of sympathetic pathway injury early in life. Such signs should prompt further diagnostic evaluation to demonstrate the presence of the agenesis of the carotid canal. Early diagnosis is essential to rule out potentially life-threatening associated vascular anomalies.

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“…ICA agenesis can be one of the vascular abnormalities constituting the PHACES (posterior fossa malformations, hemangiomas, arterial malformations, coarctation of the aorta/ cardiac defects, eye abnormalities and sternal defects) syndrome 9 , and there also are reports on its associations with neurofibromatosis types I and II 10,11 . Congenital Horner's syndrome is rarely described in association with ICA agenesis 7,12,13 . The Horner's syndrome results from interruption of the sympathetic nerve to the eye, upper lid and facial glands at any point in its course.…”

Section: Discussionmentioning

confidence: 99%

The missed missing hole

Porto

Silva

Domingues

et al. 2012

Arq. Neuro-Psiquiatr.

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In everyday clinical practice, neurologists frequently encounter complex clinical cases in which a full analysis of several variants are essential to reach the right diagnosis. The resolution of a doubtful case may rely on the recognition of a very specific diagnostic clue. The objective of this report was to describe a case in which the prompt recognition of a very subtle imaging feature in the emergency room could have precluded further extensive diagnostic investigations. case rePorTA 34-year-old woman came into the Emergency department with a worsening headache. The symptoms had begun two weeks earlier, with a holocranial pulsatile headache that gradually worsened and became unbearable in the preceding three days. In association with the pain, she complained of nausea, vomiting and intolerance to movement, light and sound. As relevant medical history, she had dyslipidemia and was taking anphepramona for obesity. She had suffered occasional headaches in the past, but never of that intensity. She had already used several over the counter analgesics and nonsteroidal anti-inflammatory drugs with no relief.Neurologic examination disclosed left eye ptosis, which she claimed to be preexisting (Fig A), confirmed on an old photograph. Cerebrospinal fluid analysis was normal. A magnetic resonance angiogram (MRA) of the internal carotid artery (ICA) demonstrated no signal within left ICA (Fig B) absTracT 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.Key words: carotid artery agenesis, Horner syndrome, carotid canal underdevelopment, computed tomography. resumo 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.Palavras-Chave: agenesia da artéria carótida, sínd...

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Agenesis of Internal Carotid Artery in a Child with Ipsilateral Horner's Syndrome

Cited by 12 publications

References 7 publications

The clinical presentation and collateral pathway development of congenital absence of the internal carotid artery

The clinical presentation and collateral pathway development of congenital absence of the internal carotid artery

Congenital agenesis of internal carotid artery with ipsilateral Horner presenting as focal neurological symptoms

The missed missing hole

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