背景:肺动静脉畸形是一种相对罕见的医学疾病,影响大约每2500个人中的1人。在患有肺动静脉畸形的人中,80%有潜在的遗传病:遗传性出血性毛细血管扩张症。
方法:我们介绍了一名20岁的巴基斯坦男性,他有持续性慢性发作性额叶头痛的病史,在一天的过程中严重程度增加。他的血红蛋白是18g/dl,表明红细胞增多症,为此,他在一个月内接受了七次手术。他的身体检查平淡无奇。他的计算机断层扫描扫描描绘了多个扩张的曲折血管,在肺的右下叶具有分支的线性混浊。多条供血动脉由右主肺动脉供血,大的引流静脉通向右下肺静脉。这被确定为弥漫性肺动静脉畸形。建议他做右肺动脉血管造影。它显示出多个曲折的血管,有一个鼻孔和大的引流静脉-在肺的右下叶具有弥漫性动静脉畸形的特征,与计算机断层扫描扫描一致。对供应动静脉畸形的这些血管中的两个进行了栓塞,使用Amplatzer血管塞2,而多个可推压线圈(五个线圈)用于栓塞第三个喂食血管。这实现了70-80%的成功栓塞右肺AVM;然而,考虑到病变的复杂性,在动静脉畸形中仍然可以看到一些残余血流。紧接着,他的氧饱和度从78%提高到96%.
结论:弥漫性肺动静脉畸形,正如在这个病人身上看到的,是罕见的,占诊断的肺动静脉畸形总数的不到5%。病人主诉进行性额叶头痛,这可以归因于低氧饱和度或脑动静脉畸形的存在。患者家属无遗传性出血性毛细血管扩张症病史。此外,尽管大多数遗传性出血性毛细血管扩张症和肺动静脉畸形的患者都有缺铁性贫血的主诉,相反,我们的患者患有红细胞增多症。这可以解释为低氧血症的补偿机制。此外,患者没有咯血或鼻出血的主诉,与典型的肺动静脉畸形相比,表现多种多样。
BACKGROUND: Pulmonary arteriovenous malformations are a relatively uncommon medical condition, affecting roughly 1 in every 2500 individuals. Of those suffering from pulmonary arteriovenous malformations, 80% have an underlying genetic condition: hereditary hemorrhagic telangiectasia.
METHODS: We present the
case of a 20-year-old Pakistani male with a history of persistent slower-onset frontal headaches that increased in severity within the course of the day. His hemoglobin was 18 g/dl, indicating polycythemia, for which he had undergone seven venesections in a month previously. His physical examination was unremarkable. His computed tomography scan depicted multiple dilated tortuous vessels with branching linear opacities in the right lower lobe of the lungs. The multiple feeding arteries were supplied by the right main pulmonary artery, and the large draining veins led to the right inferior pulmonary vein. This was identified as a diffuse pulmonary arteriovenous malformation. He was recommended for a right pulmonary artery angiogram. It showed multiple tortuous vessels with a nidus and large draining veins-features of a diffuse arteriovenous malformation in the right lower lobe of the lung consistent with the computed tomography scan. Embolization of two of these vessels feeding the arteriovenous malformation was conducted, using Amplatzer Vascular plug 2, whereas multiple pushable coils (five coils) were used for embolizing the third feeding vessel. This achieved 70-80% successful embolization of right pulmonary AVM; however, some residual flow was still seen in the arteriovenous malformation given the complexity of the lesion. Immediately after, his oxygen saturation improved from 78% to 96%.
CONCLUSIONS: Diffuse pulmonary arteriovenous malformations, as seen in this patient, are rare, accounting for less than 5% of total pulmonary arteriovenous malformations diagnosed. The patient presented with a complaint of progressive frontal headaches, which can be attributed to low oxygen saturation or the presence of a cerebral arteriovenous malformation. There was no history of hereditary hemorrhagic telangiectasia in the patient\'s family. Furthermore, although most patients with hereditary hemorrhagic telangiectasia and hence pulmonary arteriovenous malformation have complaints of iron-deficiency anemia, our patient in contrast was suffering from polycythemia. This can be explained as a compensatory mechanism in hypoxemic conditions. Moreover, the patient had no complaint of hemoptysis or epistaxis, giving a varied presentation in comparison with a typical pulmonary arteriovenous malformation.