Aliabadi P, Shafiepoor H
AJR Am J Roentgenol. 1978 Aug;131(2):255-7. doi: 10.2214/ajr.131.2.255.
Congenital cystic bronchiectasis is an uncommon disease. Most of the reported cases have been in infants and young children. This paper presents four cases of older children and younger adults who had congenital cystic bronchiectasis. Distribution of the disease is lobar or may even involve an entire lung. Bronchography plays an important role in the diagnosis of congenital cystic bronchiectasis. The three important bronchographic signs of congenital cystic bronchiectasis are: (1) absence of contrast filling of the bronchial glands duct; (2) contrast filling of numerous cysts which communicate directly with the bronchial tree; and (3) nonvisualization of normal lung parenchyma within the area of involvement.
先天性囊性支气管扩张是一种罕见疾病。大多数报道的病例见于婴幼儿。本文报告了4例年龄较大儿童及青年成人患有先天性囊性支气管扩张的病例。该病分布于肺叶,甚至可能累及整个肺脏。支气管造影在先天性囊性支气管扩张的诊断中起着重要作用。先天性囊性支气管扩张的三个重要支气管造影征象为:(1)支气管腺体导管无造影剂充盈;(2)大量囊肿与支气管树直接相通且有造影剂充盈;(3)受累区域内正常肺实质不显影。