{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,1]],"date-time":"2026-11-01T14:14:10Z","timestamp":1793542450900,"version":"4.5.0"},"reference-count":19,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,5,12]],"date-time":"2022-05-12T00:00:00Z","timestamp":1652313600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fped.2022.900538","type":"journal-article","created":{"date-parts":[[2022,5,12]],"date-time":"2022-05-12T08:12:52Z","timestamp":1652343172000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":4,"title":["Vascular Anatomy in Congenital Lung Lesions\u2014Description and Classification"],"prefix":"10.3389","volume":"10","author":[{"given":"Simon","family":"Kargl","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florian","family":"Schlader","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Scala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian","family":"Kammel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,5,12]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"986","DOI":"10.1016\/s0022-3468(97)90383-3","article-title":"Cystic lung lesions with systemic arterial blood supply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration.","volume":"32","author":"Cass","year":"1997","journal-title":"J Pediatr Surg."},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1186\/s40792-020-01063-w","article-title":"Anomalous systemic arterial supply to the basal segment of the lung with giant aberrant artery: a case report.","volume":"6","author":"Utsumi","year":"2020","journal-title":"Surg Case Rep."},{"key":"B3","doi-asserted-by":"publisher","first-page":"S117","DOI":"10.1007\/s00270-010-9798-x","article-title":"Systemic arterial supply to the normal basal segments of the left lower lobe of the lung\u2013treatment by coil embolization\u2013and a literature review.","volume":"34","author":"Abe","year":"2011","journal-title":"Cardiovasc Intervent Radiol."},{"key":"B4","doi-asserted-by":"publisher","first-page":"e39","DOI":"10.1016\/j.ejcts.2011.01.080","article-title":"Pulmonary sequestration: a retrospective analysis of 2625 cases in China.","volume":"40","author":"Wei","year":"2011","journal-title":"Eur J Cardiothorac Surg."},{"key":"B5","doi-asserted-by":"publisher","first-page":"626","DOI":"10.1053\/jpsu.2003.50138","article-title":"Upper thoracic extralobar pulmonary sequestration with anomalous blood supply from the subclavian artery.","volume":"38","author":"Ito","year":"2003","journal-title":"J Pediatr Surg."},{"key":"B6","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1007\/BF02505815","article-title":"Extralobar sequestration with unusual arterial supply and venous drainage.","volume":"17","author":"Gamillscheg","year":"1996","journal-title":"Pediatr Cardiol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"1286","DOI":"10.1016\/j.jpedsurg.2018.08.054","article-title":"Distribution, diagnosis, and treatment of pulmonary sequestration: report of 208 cases.","volume":"54","author":"Zhang","year":"2019","journal-title":"J Pediatr Surg."},{"key":"B8","doi-asserted-by":"publisher","first-page":"392","DOI":"10.6061\/clinics\/2016(07)07","article-title":"Evaluation of pulmonary sequestration with multidetector computed tomography angiography in a select cohort of patients: a retrospective study.","volume":"71","author":"Long","year":"2016","journal-title":"Clinics (Sao Paulo)."},{"key":"B9","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1136\/thx.34.1.96","article-title":"Lung sequestration: report of seven cases and review of 540 published cases.","volume":"34","author":"Savic","year":"1979","journal-title":"Thorax."},{"key":"B10","doi-asserted-by":"publisher","first-page":"46","DOI":"10.4103\/1817-1737.197778","article-title":"Anomalous systemic arterial supply of pulmonary sequestration in adult patients.","volume":"12","author":"Hou","year":"2017","journal-title":"Ann Thorac Med."},{"key":"B11","first-page":"142","article-title":"[Intralobar pulmonary sequestration with three aberrant arteries: a case report and review of the Japanese literature].","volume":"51","author":"Jotsuka","year":"","journal-title":"Kyobu Geka."},{"key":"B12","doi-asserted-by":"publisher","first-page":"741","DOI":"10.1007\/s00383-012-3088-4","article-title":"Intralobar pulmonary sequestration: an uncommon case with triple arterial supply and systemic venous drainage.","volume":"28","author":"Theodoropoulos","year":"2012","journal-title":"Pediatr Surg Int."},{"key":"B13","doi-asserted-by":"publisher","first-page":"611","DOI":"10.1378\/chest.86.4.611","article-title":"A study of pulmonary ligament arteries. 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