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先天性支气管肺前肠畸形。叶内型和叶外型肺隔离症与前肠相通。

Congenital bronchopulmonary foregut malformations. Intralobar and extralobar pulmonary sequestrations communicating with the foregut.

作者信息

Hruban R H, Shumway S J, Orel S B, Dumler J S, Baker R R, Hutchins G M

机构信息

Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, Maryland.

出版信息

Am J Clin Pathol. 1989 Apr;91(4):403-9. doi: 10.1093/ajcp/91.4.403.

Abstract

Two unusual variants of bronchopulmonary foregut malformations are presented. The first case was that of a 12-year-old male with a history of pectus excavatum in whom severe lobar emphysema developed secondary to an intralobar pulmonary sequestration that communicated with the esophagus. This case was unusual in that foregut communications and associated congenital anomalies are generally believed to be restricted to extralobar pulmonary sequestrations. The second case was that of a 27-year-old woman with an extralobar pulmonary sequestration that communicated with the esophagus. The sequestration was unusual in that it arose in the anterior mediastinum and received the bulk of its blood supply from the pulmonary artery. The occurrence of mixed forms of pulmonary sequestrations supports the hypothesis that extralobar and intralobar sequestrations and sequestrations with foregut communication are related thoracic disorders that are best considered bronchopulmonary foregut malformations.

摘要

本文介绍了支气管肺前肠畸形的两种罕见变异型。第一例是一名12岁男性,有漏斗胸病史,因叶内型肺隔离症与食管相通继发严重的大叶性肺气肿。该病例不同寻常之处在于,一般认为前肠相通及相关先天性异常仅限于叶外型肺隔离症。第二例是一名27岁女性,患有与食管相通的叶外型肺隔离症。该隔离症不同寻常之处在于其起源于前纵隔,主要血供来自肺动脉。肺隔离症混合形式的出现支持了这样一种假说,即叶外型和叶内型隔离症以及与前肠相通的隔离症是相关的胸部疾病,最好将其视为支气管肺前肠畸形。

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