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成人先天性肺部疾病

Adult congenital lung disease.

作者信息

Shanmugam Ganesh

机构信息

Department of Cardiac Surgery, Royal Hospital for Sick Children, Dalnair Street, Glasgow G3 8SJ, Scotland, UK.

出版信息

Eur J Cardiothorac Surg. 2005 Sep;28(3):483-9. doi: 10.1016/j.ejcts.2005.05.008.

Abstract

Congenital lung anomalies are increasingly diagnosed in adults, because they are missed in childhood, or as a consequence of incomplete resections at a younger age. They have unique manifestations, often mimic other thoracic pathology, and can present acutely and necessitate emergent evaluation and management. Misdiagnosis is common. This article deals with the patterns of presentation, clinical and radiological diagnosis, and the management of these disorders in adults. The lesions analysed include congenital cystic adenomatoid malformation, sequestration, congenital lobar emphysema and bronchogenic cysts. It is important to include these malformations in the differential diagnosis of adult thoracic pathology and to understand the modes of presentation, potential complications and management strategies. The radiological diagnosis is reliably made by computed tomography and/or magnetic resonance imaging. The potential for malignancy in these lesions is an important consideration. The management of asymptomatic lesions was considered controversial, but more lesions are now resected early, in recognition of the potential for subsequent complications. Complete resection is recommended. In contradistinction to childhood malformations, these lesions are more amenable to thoracoscopic resections, provided they are completely benign. Congenital lung malformations call for well-organized cooperation between paediatric and adult pulmonologists to ensure a smooth medical transition from childhood to adulthood.

摘要

先天性肺异常在成人中越来越多地被诊断出来,这是因为它们在儿童期被漏诊,或者是由于年轻时切除不完全所致。它们有独特的表现,常与其他胸部病变相似,可急性发作,需要紧急评估和处理。误诊很常见。本文探讨这些疾病在成人中的表现形式、临床及影像学诊断以及治疗方法。分析的病变包括先天性囊性腺瘤样畸形、肺隔离症、先天性大叶性肺气肿和支气管囊肿。在成人胸部病变的鉴别诊断中纳入这些畸形,并了解其表现方式、潜在并发症及治疗策略很重要。通过计算机断层扫描和/或磁共振成像可可靠地做出影像学诊断。这些病变的恶变可能性是一个重要的考虑因素。无症状病变的治疗曾存在争议,但鉴于后续并发症的可能性,现在更多病变在早期就被切除。建议进行完整切除。与儿童期畸形不同,只要这些病变完全良性,就更适合胸腔镜切除。先天性肺畸形需要儿科和成人肺科医生之间有组织良好的合作,以确保从儿童期到成年期的医疗过渡顺利。

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