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[先天性支气管肺发育不良的诊断与治疗。32例病例回顾]

[Diagnosis and treatment of congenital bronchopulmonary malformations. A review of 32 cases].

作者信息

Salles M, Deschildre A, Bonnel C, Dubos J P, Bonnevalle M, Devismes L, Errera S, Sfeir R, Glowacki M, Santos C, Thumerelle C

机构信息

Unité de pneumoallergologie pédiatrique, clinique de pédiatrie, hôpital-Jeanne-de-Flandre, CHRU de Lille, 59037 Lille cedex, France.

出版信息

Arch Pediatr. 2005 Dec;12(12):1703-8. doi: 10.1016/j.arcped.2005.09.013. Epub 2005 Oct 14.

Abstract

OBJECTIVES

Analysis of our experience and propositions on the diagnosis and treatment of congenital bronchopulmonary malformations (BPM).

POPULATION AND METHODS

Retrospective study of BPM diagnosed between 1997 and 2001. Analysis of clinical spectrum, diagnosis tools, treatment, and clinical outcome.

RESULTS

Thirty-two cases of BPM have been investigated (11 cystic adenomatoid malformations, 7 pulmonary sequestrations, 7 bronchogenic cysts, 4 congenital lobar emphysema, and 3 complex emphysematous malformations). Nineteen patients had a prenatal diagnosis. For 9 others, symptoms occurred before 4 years of age. Evaluation included a CT-scan in all patients (BPM involution in one). Surgical treatment was performed in 30 patients (lobectomy in 18), with a mean age of 7 months for asymptomatic patients. During the follow-up (mean: 3 years), respiratory symptoms were reported in 10 cases, 3 of them were related to the BPM.

DISCUSSION

Improvement in prenatal ultrasound diagnosis modified the management strategy. Considering the risk of pulmonary complications, surgical treatment is required during the first months of life. For congenital lobar emphysema, and some pulmonary sequestrations or small cystic adenomatoid malformations (<3 cm), conservative attitude may be preferred. BPM justify a multidisciplinary management.

摘要

目的

分析我们在先天性支气管肺畸形(BPM)诊断和治疗方面的经验及建议。

研究对象与方法

对1997年至2001年间诊断的BPM进行回顾性研究。分析临床症状谱、诊断工具、治疗方法及临床结果。

结果

共研究了32例BPM(11例囊性腺瘤样畸形、7例肺隔离症、7例支气管囊肿、4例先天性大叶性肺气肿和3例复杂性肺气肿畸形)。19例患者进行了产前诊断。另外9例患者在4岁前出现症状。所有患者均接受了CT扫描(1例BPM自行消退)。30例患者接受了手术治疗(18例行肺叶切除术),无症状患者的平均年龄为7个月。在随访期间(平均3年),10例患者出现呼吸道症状,其中3例与BPM有关。

讨论

产前超声诊断的改善改变了治疗策略。考虑到肺部并发症的风险,在生命的最初几个月需要进行手术治疗。对于先天性大叶性肺气肿以及一些肺隔离症或小的囊性腺瘤样畸形(<3 cm),可能更倾向于采取保守态度。BPM需要多学科管理。

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