Multiple intracranial aneurysms and moyamoya disease associated with microcephalic osteodysplastic primordial dwarfism type II: surgical considerations.
Multiple intracranial aneurysms and moyamoya disease associated with microcephalic osteodysplastic primordial dwarfism type II: surgical considerations.
复制标题
与小头骨发育不良原始侏儒症 II 型相关的多发性颅内动脉瘤和烟雾病：手术注意事项。
DOI：
10.3171/2009.6.peds08137
复制
发表时间：
2009
期刊：
影响因子：
--
通讯作者：
M. Lawton
中科院分区：
文献类型：
--
作者：
J. Waldron;S. Hetts;Jennifer Armstrong‐Wells;C. Dowd;H. Fullerton;N. Gupta;M. Lawton
小头骨发育不良性原始侏儒症II型（MOPD II）是一种罕见的遗传综合征，其特征是身材极小和小头畸形，25%的患者与颅内动脉瘤和烟雾病相关。虽然蛛网膜下腔出血和卒中是这些患者发病和死亡的主要原因，但在神经外科文献中很少研究MOPD II。作者报告了3例MOPD II患者的经验，其中包括1例8个动脉瘤（文献中报告的最多动脉瘤）的患者，以及1例烟雾病和多发性动脉瘤患者的首次报告。这些病变的不良自然史提示积极的显微外科和/或血管内治疗。由于这些患者的手术通道狭窄且动脉较小，无论是动脉瘤夹置入还是颅外-颅内搭桥，显微手术都具有挑战性，但在技术上是可行的，并且在必须治疗多发动脉瘤或需要进行脑血运重建时具有强烈的适应症。
Microcephalic osteodysplastic primordial dwarfism type II (MOPD II) is a rare genetic syndrome characterized by extremely small stature and microcephaly, and is associated in 25% of patients with intracranial aneurysms and moyamoya disease. Although aneurysmal subarachnoid hemorrhage and stroke are leading causes of morbidity and death in these patients, MOPD II is rarely examined in the neurosurgical literature. The authors report their experience with 3 patients who presented with MOPD II, which includes a patient with 8 aneurysms (the most aneurysms reported in the literature), and the first report of a patient with both moyamoya disease and multiple aneurysms. The poor natural history of these lesions indicates aggressive microsurgical and/or endovascular therapy. Microsurgery, whether for aneurysm clip placement or extracranial-intracranial bypass, is challenging due to tight surgical corridors and diminutive arteries in these patients, but is technically feasible and strongly indicated when multiple aneurysms must be treated or cerebral revascularization is needed.