Love B B, Biller J, Landas S K, Hoover W W
Department of Neurology, University of Iowa Hospitals and Clinics, Iowa City.
Angiology. 1992 Jun;43(6):522-8. doi: 10.1177/000331979204300612.
Pulmonary arteriovenous malformations occur in 15-20% of patients with Rendu-Osler-Weber syndrome and can be the source of paradoxical emboli causing cerebral ischemia, septic emboli leading to brain abscesses, or polycythemia causing hyperviscosity and cerebral ischemia. The diagnosis of these malformations may be elusive, since classic clinical or radiologic findings may be absent in some patients. The authors report a patient with Rendu-Osler-Weber syndrome with cerebral ischemia who had normal findings from a pulmonary examination and chest roentgenogram. An ultrafast computed tomography scan of the chest demonstrated, however, a pulmonary arteriovenous malformation in the right upper lobe that was successfully resected. Ultrafast computed tomography of the chest is a relatively noninvasive method of screening for a pulmonary arteriovenous malformation in a patient with Rendu-Osler-Weber syndrome and otherwise unexplained neurologic symptoms.
肺动静脉畸形见于15% - 20%的遗传性出血性毛细血管扩张症患者,可成为反常栓塞的来源,导致脑缺血;成为脓毒性栓子的来源,导致脑脓肿;或导致红细胞增多症,引起血液黏滞度升高和脑缺血。这些畸形的诊断可能较困难,因为部分患者可能没有典型的临床或影像学表现。作者报告1例患有遗传性出血性毛细血管扩张症且有脑缺血的患者,其肺部检查和胸部X线片结果正常。然而,胸部超快速计算机断层扫描显示右上叶有1个肺动静脉畸形,该畸形已成功切除。胸部超快速计算机断层扫描是一种相对无创的方法,用于筛查患有遗传性出血性毛细血管扩张症且有无法解释的神经症状的患者是否存在肺动静脉畸形。