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肺动静脉畸形:治疗选择

Pulmonary arteriovenous malformations: therapeutic options.

作者信息

Puskas J D, Allen M S, Moncure A C, Wain J C, Hilgenberg A D, Wright C, Grillo H C, Mathisen D J

机构信息

General Thoracic Surgical Unit, Massachusetts General Hospital, Harvard Medical School, Boston 02114.

出版信息

Ann Thorac Surg. 1993 Aug;56(2):253-7; discussion 257-8. doi: 10.1016/0003-4975(93)91156-h.

Abstract

We have treated 21 patients (13 female, 8 male) with pulmonary arteriovenous malformations (PAVMs). Mean age at diagnosis was 37.5 years (range, 15 to 72 years). Presenting symptoms included dyspnea on exertion (67%), hereditary hemorrhagic telangiectasia (57%), and major neurologic events (33%). In our early experience, 8 patients had no specific treatment; their case histories illustrate the major neurologic complications of untreated PAVMs. Nine patients (8 primarily, 1 after recurrence) underwent conservative surgical excision; 4 had lobectomy, and 5 had segmentectomy or subsegmental excision. One patient underwent staged bilateral thoracotomies for multiple bilateral lesions. The arterial oxygen tension was found to increase after excision of large or solitary PAVMs. All surgically treated patients were relieved of dyspnea, and none had postoperative recurrence of PAVMs or neurologic complications related to PAVMs. Five patients underwent balloon occlusion of PAVMs. Two patients chose to have solitary PAVMs occluded rather than undergo thoracotomy. One underwent surgical excision 5 years later, and the other required repeat balloon embolization 4 years later when recanalization of the PAVMs was documented. Three patients with numerous PAVMs received palliation with multiple balloon embolizations. The high incidence of associated major neurologic complications mandates aggressive treatment of PAVMs whenever feasible. Conservative surgical resection remains the treatment of choice. Balloon embolization offers an alternative therapy for patients who are poor surgical risks or those whose lesions are too numerous to resect.

摘要

我们治疗了21例肺动静脉畸形(PAVM)患者(13例女性,8例男性)。诊断时的平均年龄为37.5岁(范围为15至72岁)。呈现的症状包括劳力性呼吸困难(67%)、遗传性出血性毛细血管扩张症(57%)和重大神经系统事件(33%)。在我们早期的经验中,8例患者未接受特殊治疗;他们的病史说明了未经治疗的PAVM的主要神经系统并发症。9例患者(8例初次治疗,1例复发后治疗)接受了保守性手术切除;4例行肺叶切除术,5例行肺段切除术或亚肺段切除术。1例患者因双侧多发病变接受了分期双侧开胸手术。发现切除大型或孤立性PAVM后动脉血氧张力增加。所有接受手术治疗的患者呼吸困难均得到缓解,且无一例出现PAVM术后复发或与PAVM相关的神经系统并发症。5例患者接受了PAVM的球囊封堵术。2例患者选择封堵孤立性PAVM而非接受开胸手术。1例患者5年后接受了手术切除,另1例患者在记录到PAVM再通时于4年后需要再次进行球囊栓塞。3例有大量PAVM的患者接受了多次球囊栓塞进行姑息治疗。相关重大神经系统并发症的高发生率要求在可行时积极治疗PAVM。保守性手术切除仍然是首选治疗方法。球囊栓塞为手术风险高或病变过多无法切除的患者提供了一种替代治疗方法。

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