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肺动静脉瘘的外科治疗

目的探讨肺动静脉瘘的临床特点、手术切除及治疗效果。　方法　回顾性分析自 1990年 1月至 2010年 12月同济大学附属上海市肺科医院手术治疗 23例肺动静脉瘘患者的临床资料，其中男 10例，女 13例；年龄 13～ 49（30.5±4.9）岁。行开胸手术 16例，电视胸腔镜手术 7例；肺楔形切除术 10例，肺叶切除术 13例。　结果围术期无死亡， 23例病灶彻底切除，术后无肺部感染、胸腔内出血、低血压、气胸、脓胸及支气管胸膜瘘等并发症。 23例患者手术后动脉血氧分压较术前明显改善［（93.5±2.8）mm Hg vs.（84.1±4.8）mm Hg，P＜ 0.05］。随访 23例，随访时间 5～ 56个月，肺动静脉瘘无复发。　结论　外科手术是治疗肺动静脉瘘非常有效的手段，对孤立性病变应优先考虑行电视胸腔镜手术，其具有创伤小、恢复快等特点。

左下肺先天性肺动静脉瘘两例

左下肺片团影

讨论病案为胸部 CT 平扫显示的肺部片团影像，是呼吸系统常见的影像学改变，临床多考虑感染性疾病、肿瘤或非感染非肿瘤性病变。初诊医师根据临床特征、影像学细微的差异，对患者进行胸部增强 CT 易于发现此病灶为血管结构，并非常见的感染或肿瘤病变。有条件的单位可进行肺血管重建、心室声学造影以及肺动脉造影，就能确诊肺动静脉瘘。临床医师以此为最终诊断是非常普遍的，但专科医师应结合患者受凉感冒后易发生鼻衄且有家族史，全面考虑并深入探究其可能的病因如先天性或遗传性因素的可能，应当熟悉各种罕见病的临床特征，如遗传性出血性毛细血管扩张症。通过家系资料收集、连锁分析，目标基因全外显子测序，最终证实该例患者是遗传性出血性毛细血管扩张症，临床表现出动静脉瘘并有其他部位出血。

Interventional embolization therapy for complex pulmonary arteriovenous fistula: case report and literature review

ObjectiveTo explore the clinical characteristics and improve the knowledge of diagnosis and treatment of complex pulmonary arteriovenous fistula (PAVF) as well as enrich the experience of diagnosis and treatment of the disease.MethodsA retrospective analysis of pathogenetic process clinical manifestations, imaging features and diagnosis and treatment was conducted on one case of complex PAVF. The literature review was carried out with " complex pulmonary arteryovenous fistula (malformation)” as the research terms in English and Chinese respectively in CNKI, WanFang and PubMed database. Search time ranged from January 1997 to April 2018, and the literature was screened and reviewed.ResultsThe patient was a 47-year-old female complained of recurrent epistaxis for 40 years, intermittent hemoptysis for 20 years, headache, dizziness, chest pain, chest tightness for 4 years and the symptoms were aggravated by 3 months, visiting this hospital on January 23, 2018. Pulmonary CT angiography revealed multiple nodules in internal and external segment and outer basal segment of right lung, anterior basal segment and outer basal segment of left lung. CT enhanced scan showed that the thickened pulmonary artery was connected with the above lesion, and the edge was accompanied by large draining veins. Pulmonary artery revascularization showed complex PAVF abnormal branches. The diagnosis was complex PAVF, and interventional embolization therapy was carried out and curative effect was satisfactory during the follow-up. A total of 6 literatures were reviewed in above-mentioned databases, including 4 Chinese literatures and 2 English literatures, containing 10 patients, including 8 males and 2 females, with an average age of (9.7±7.0) years. Most of the clinical manifestations were shortness of breath after exercise, cyanosis and hemoptysis and all patients were cured and discharged after interventional embolization treatment except for 1 patient refused treatment.ConclusionsComplex PAVF is a very rare pulmonary vascular malformation. The clinical manifestations mainly include hypoxemia, dyspnea, hemoptysis, and the preferred treatment is interventional embolization, which has a satisfactory clinical effect at a short-term follow-up.