Farhat Wassim, Ahdab Rechdi, Hosseini Hassan
Service de Neurologie, Hôpital Henri Mondor, APHP, Faculté de Médecine, Université Paris XII, Créteil, France.
Vasc Health Risk Manag. 2011 Jan 26;7:37-40. doi: 10.2147/VHRM.S16642.
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.
颈内动脉（ICA）发育不全是一种罕见的发育异常，多数情况下无症状，但也可能表现为脑血管意外。与霍纳综合征相关的情况极为罕见。我们报告3例颈内动脉发育不全合并霍纳综合征及虹膜淡色，并表现为局灶性神经症状。颈内动脉发育不全可导致侧支循环形成，多数病例因此无症状。已描述了三种类型的侧支循环。这些侧支循环增加了动脉瘤形成的风险以及危及生命的蛛网膜下腔出血的发生几率。先天性霍纳综合征与无汗的虹膜淡色相关，强烈提示早年交感神经通路受损。这些体征应促使进一步的诊断评估，以证实颈动脉管发育不全的存在。早期诊断对于排除潜在的危及生命的相关血管异常至关重要。
