Kargl Simon, Schlader Florian, Scala Mario, Kammel Julian
Medical Faculty, Johannes Kepler University Linz, Linz, Austria.
Department of Pediatric Surgery, Kepler University Hospital, Linz, Austria.
Front Pediatr. 2022 May 12;10:900538. doi: 10.3389/fped.2022.900538. eCollection 2022.
Bronchopulmonary sequestration (BPS) and hybrid lesion of congenital pulmonary airway malformation (CPAM) are congenital lung lesions typically presenting with systemic vascular connection. We describe and categorize this atypical systemic vascular anatomy in congenital lung lesions.
In a medical chart review from 2005 to 2020 patients with systemic vascular connection of congenital lung lesions were identified. Clinical and radiological data were collected and compared. Two experienced pediatric radiologists reviewed postnatal thoracic contrast-enhanced computed tomography scans to describe and categorize atypical vascular anatomy. We completed our findings with a review on vascular anatomy in congenital lung lesions.
A total of 21 patients with congenital lung lesions (nine extralobar BPS, five intralobar BPS, seven hybrid lesions) had systemic arterial supply; with seven of these additionally having systemic venous drainage. Origin of the feeding arteries from the aorta or aortic main branches was described as supra-diaphragmatic (descending thoracic aorta) in nine and infra-diaphragmatic in ten patients (abdominal aorta, celiac trunk). In two patients with hybrid lesions both supra- and infra-diaphragmatic arterial feeders were found. Additional systemic venous connection of supra-diaphragmatic type drains into the azygos-hemiazygos system (4/21) while the infra-diaphragmatic type (3/21) drains into caval vein, portal or splenic vein.
Various variants of systemic arterial and venous connection of congenital lung lesions can be found. Classification of systemic arterial connection as well as venous drainage of congenital lung lesions as supra-diaphragmatic and infra-diaphragmatic types is intuitive, simple and may be important for the surgeon to avoid unanticipated situations and to perform safe resections.
支气管肺隔离症（BPS）和先天性肺气道畸形（CPAM）混合病变是典型的伴有体循环血管连接的先天性肺部病变。我们描述并对先天性肺部病变中这种非典型的体循环血管解剖结构进行分类。
回顾2005年至2020年的病历，确定患有先天性肺部病变且伴有体循环血管连接的患者。收集并比较临床和放射学数据。两位经验丰富的儿科放射科医生对产后胸部增强CT扫描进行评估，以描述和分类非典型血管解剖结构。我们通过回顾先天性肺部病变的血管解剖结构来完善我们的研究结果。
共有21例先天性肺部病变患者（9例叶外型BPS、5例叶内型BPS、7例混合病变）存在体循环动脉供血；其中7例还伴有体循环静脉引流。供血动脉起源于主动脉或主动脉主要分支，9例为膈上型（胸降主动脉），10例为膈下型（腹主动脉、腹腔干）。在2例混合病变患者中，同时发现了膈上和膈下动脉供血。膈上型额外的体循环静脉连接汇入奇静脉 - 半奇静脉系统（4/21），而膈下型（3/21）汇入腔静脉、门静脉或脾静脉。
可发现先天性肺部病变体循环动脉和静脉连接的多种变异。将先天性肺部病变的体循环动脉连接以及静脉引流分为膈上型和膈下型，直观、简单，对外科医生避免意外情况并进行安全切除可能很重要。
