Sharma D, Pandia M P, Bithal P K
Department of Neuroanaesthesiology, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi, India.
Acta Anaesthesiol Scand. 2005 Oct;49(9):1391-4. doi: 10.1111/j.1399-6576.2005.00727.x.
A 9-year-old cyanosed child suffering from Osler-Weber-Rendu syndrome with bilateral pulmonary arteriovenous malformations (PAVMs) was posted for cerebral angiography under general anaesthesia. Careful preanaesthetic evaluation led to the diagnosis of coexisting congenital methaemoglobinaemia. There is no previous report of Osler-Weber-Rendu syndrome coexisting with congenital methaemoglobinaemia. This report emphasizes that a second contributory cause of cyanosis must be suspected and meticulously looked for if the symptomatology in a patient cannot be explained by a single established diagnosis. Positive-pressure ventilation was associated with reduction in arterial oxygenation despite an increasing inspired oxygen concentration, which returned to preanaesthetic levels only after extubation and resumption of spontaneous respiration.
一名9岁患有奥斯勒-韦伯-伦杜综合征且伴有双侧肺动静脉畸形（PAVM）的发绀儿童拟在全身麻醉下进行脑血管造影。仔细的麻醉前评估诊断出同时存在先天性高铁血红蛋白血症。此前尚无奥斯勒-韦伯-伦杜综合征与先天性高铁血红蛋白血症共存的报道。本报告强调，如果患者的症状不能用单一既定诊断来解释，就必须怀疑并仔细寻找导致发绀的第二个原因。尽管吸入氧浓度增加，但正压通气与动脉氧合降低有关，只有在拔管并恢复自主呼吸后，动脉氧合才恢复到麻醉前水平。
