15岁女童因抽搐1次,同时肺部CT发现肺结节入院。无肢端发绀,无明显杵状指/趾畸形。实验室检查示血红蛋白升高(162 g/L)、动脉血氧分压降低(61.5 mm Hg)。肺部CT提示右肺中叶见不规则稍高密度结节,增强扫描可见明显强化,局部见粗大血管影。追问病史发现患儿母亲有鼻衄、肺部肿物切除史及舌部毛细血管扩张表现。患儿确诊为肺动静脉畸形和遗传性出血性毛细血管扩张症,予介入下行肺动脉栓塞术治疗。术后当日患儿未吸氧下经皮血氧饱和度达98%。但术后3个月复查肺动脉造影提示肺血管畸形复发,因导丝无法进入分支动脉,未行封堵治疗,仍需长期随访。
Abstract:A girl, aged 15 years, was admitted due to sudden convulsion once and multiple pulmonary nodules on lung CT. Acrocyanosis or acropachy/toe deformity was not observed. Laboratory examinations showed an increase in hemoglobin (162 g/L) and a reduction in arterial partial pressure of oxygen (61.5 mm Hg). Lung CT showed irregular slightly high-density nodules in the middle lobe of the right lung, and contrast-enhanced CT scan showed obvious enhancement with thick vascular shadow locally. An investigation of medical history revealed that the girl's mother had a history of epistaxis and resection of pulmonary mass and the girl presented with tongue telangiectasia. The girl was diagnosed with hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformation. she was given interventional embolization therapy. Transcutaneous oxygen saturation reached 98% without oxygen inhalation on the day after surgery. Pulmonary angiography at 3 months after surgery showed the recurrence of pulmonary vascular malformation, and embolization of pulmonary arterial fistula was not performed since the guide wire could not enter the branch artery. There was still a need for long-term follow-up.
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