Kageyama Hiroshi, Toyooka Terushige, Osada Hideo, Tsuzuki Nobusuke
Department of Neurosurgery, Kuki General Hospital, 418-1 Kamihayami Kuki, Japan.
Department of Neurosurgery, National Defense Medical College, Namiki 3-2, Tokorozawa, Saitama, Japan.
Surg Neurol Int. 2015 May 7;6:71. doi: 10.4103/2152-7806.156633. eCollection 2015.
We report a case of infratentorial arteriovenous malformation (AVM) associated with persistent primitive hypoglossal artery (PPHA). To our knowledge, this is the second reported case of these combined anomalies in the English literature. We discuss the embryological relationship between these two congenital vascular anomalies.
An 18-year-old girl, who suddenly developed severe headache and vomiting followed by loss of consciousness, was admitted to our hospital. A computed tomography scan showed intracerebellar hemorrhage with obstructive hydrocephalus. Digital subtraction angiography revealed an AVM in the left cerebellar hemisphere and an ipsilateral PPHA. After the intracranial pressure was stabilized, the AVM was surgically removed. AVMs develop during the 4(th) to 8(th) week of embryonic life. In contrast, carotid-basilar anastomoses (CBAs) including primitive hypoglossal arteries appear and close spontaneously by the 6(th) week of embryonic life. Thus, AVMs precede CBAs, and a large amount of blood flows into the adjoining AVM via ipsilateral CBAs. As a result, spontaneous closure of a CBA may be disturbed.
We speculate that coexistence of infratentorial AVMs and ipsilateral CBAs is not incidental but inevitable.
我们报告一例幕下动静脉畸形（AVM）合并永存原始舌下动脉（PPHA）的病例。据我们所知，这是英文文献中第二例报道的这两种联合畸形病例。我们讨论了这两种先天性血管畸形之间的胚胎学关系。
一名18岁女孩突然出现严重头痛、呕吐，随后意识丧失，被收入我院。计算机断层扫描显示小脑内出血伴梗阻性脑积水。数字减影血管造影显示左小脑半球有一个AVM和同侧的PPHA。颅内压稳定后，手术切除了AVM。AVM在胚胎生命的第4至8周发育。相比之下，包括原始舌下动脉在内的颈基底吻合支（CBA）在胚胎生命的第6周出现并自发闭合。因此，AVM先于CBA出现，大量血液通过同侧CBA流入相邻的AVM。结果，CBA的自发闭合可能受到干扰。
我们推测幕下AVM与同侧CBA的共存并非偶然而是必然。
