PDF(Pubmed)
Abstract：
UNASSIGNED: The disruption of intracranial fluid dynamics due to large unruptured cerebral arteriovenous malformation (AVM) commonly triggers a domino effect within the central nervous system. This phenomenon is frequently overlooked in prior clinic and may lead to catastrophic misdiagnoses. Our team has documented the world\'s first case of so-called AVM Pentalogy (AVMP) induced by a AVM.
UNASSIGNED: A 30-year-old female was first seen 9 years ago with an occasional fainting, at which time a huge unruptured AVM was discovered. Subsequently, due to progressive symptoms, she sought consultations from several prestigious neurosurgical departments in China, where all consulting neurosurgeons opted for conservation treatment due to perceived surgical risks. During the follow-up period, the patient gradually presented with hydrocephalus, empty sella, secondary Chiari malformation, syringomyelia, and scoliosis (we called as AVMP). When treated in our department, she already displayed numerous symptoms, including severe intracranial hypertension. Our team deduced that the hydrocephalus was the primary driver of her AVMP symptoms, representing the most favorable risk profile for intervention. As expected, a ventriculoperitoneal shunt successfully mitigated all symptoms of AVMP at 21-months post-surgical review.
UNASSIGNED: During the monitoring of unruptured AVM, it is crucial to remain vigilant for the development or progression of AVMP. When any component of AVMP is identified, thorough etiological studies and analysis of cascade reactions are imperative to avert misdiagnosis. When direct AVM intervention is not viable, strategically addressing hydrocephalus as part of the AVMP may serve as the critical therapeutic focus.
UNASSIGNED: A 30-year-old female was first seen 9 years ago with an occasional fainting, at which time a huge unruptured AVM was discovered. Subsequently, due to progressive symptoms, she sought consultations from several prestigious neurosurgical departments in China, where all consulting neurosurgeons opted for conservation treatment due to perceived surgical risks. During the follow-up period, the patient gradually presented with hydrocephalus, empty sella, secondary Chiari malformation, syringomyelia, and scoliosis (we called as AVMP). When treated in our department, she already displayed numerous symptoms, including severe intracranial hypertension. Our team deduced that the hydrocephalus was the primary driver of her AVMP symptoms, representing the most favorable risk profile for intervention. As expected, a ventriculoperitoneal shunt successfully mitigated all symptoms of AVMP at 21-months post-surgical review.
UNASSIGNED: During the monitoring of unruptured AVM, it is crucial to remain vigilant for the development or progression of AVMP. When any component of AVMP is identified, thorough etiological studies and analysis of cascade reactions are imperative to avert misdiagnosis. When direct AVM intervention is not viable, strategically addressing hydrocephalus as part of the AVMP may serve as the critical therapeutic focus.
摘要：
■由于大量未破裂的脑动静脉畸形（AVM）引起的颅内流体动力学破坏通常会触发中枢神经系统内的多米诺效应。这种现象在以前的临床中经常被忽视，并可能导致灾难性的误诊。我们的团队记录了世界上第一例由AVM引起的所谓的AVM五联症（AVMP）。
■一名30岁的女性在9年前首次出现昏厥，当时发现了一个巨大的未破裂的AVM。随后，由于进行性症状，她向中国几个著名的神经外科寻求咨询，由于感知到的手术风险，所有咨询神经外科医生都选择了保守治疗。在后续期间,患者逐渐出现脑积水，空的西拉,继发性Chiari畸形,脊髓空洞症,和脊柱侧弯（我们称之为AVMP）。在我们部门接受治疗时，她已经出现了很多症状,包括严重的颅内高压.我们的团队推断，脑积水是她AVMP症状的主要驱动因素，代表最有利的干预风险状况。不出所料,在术后复查21个月时,脑室-腹腔分流术成功缓解了AVMP的所有症状.
■在监测未破裂的AVM期间，对AVMP的发展或进展保持警惕至关重要.当识别出AVMP的任何组件时，彻底的病因研究和级联反应的分析对于避免误诊是必要的。当直接AVM干预不可行时，战略性地解决作为AVMP一部分的脑积水可能是关键的治疗重点.
■一名30岁的女性在9年前首次出现昏厥，当时发现了一个巨大的未破裂的AVM。随后，由于进行性症状，她向中国几个著名的神经外科寻求咨询，由于感知到的手术风险，所有咨询神经外科医生都选择了保守治疗。在后续期间,患者逐渐出现脑积水，空的西拉,继发性Chiari畸形,脊髓空洞症,和脊柱侧弯（我们称之为AVMP）。在我们部门接受治疗时，她已经出现了很多症状,包括严重的颅内高压.我们的团队推断，脑积水是她AVMP症状的主要驱动因素，代表最有利的干预风险状况。不出所料,在术后复查21个月时,脑室-腹腔分流术成功缓解了AVMP的所有症状.
■在监测未破裂的AVM期间，对AVMP的发展或进展保持警惕至关重要.当识别出AVMP的任何组件时，彻底的病因研究和级联反应的分析对于避免误诊是必要的。当直接AVM干预不可行时，战略性地解决作为AVMP一部分的脑积水可能是关键的治疗重点.
