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      Anomalous systemic arterial supply of pulmonary sequestration in adult patients

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          Abstract

          OBJECTIVES:

          This study described the characteristics of the systemic arterial supply of pulmonary sequestration (PS) in an attempt to better distinguish PS from other acquired lesions.

          METHODS:

          We identified 25 patients hospitalized at the Peking Union Medical College Hospital during January 2013 to December 2015 with the assistance of medical catalogers. Twenty-three patients with a definite diagnosis of “pulmonary sequestration” clinically or pathologically were included in the study. The medical records, imaging information, and pathological data were reviewed retrospectively. The general characteristics of the patients and the features of the anomalous arteries were summarized.

          RESULTS:

          Aberrant arterial supply of PS was found in all 23 (100%) cases. Among them, twenty patients received surgery, including 14 (70%) with aberrant arterial supply found before surgery, and the other 6 (30%) found during surgery. Nineteen (82.6%) patients had a single systematic arterial supply, with a median diameter of 8 mm. More than one arterial supplies were found in four (17.4%) cases. In 21 (91.3%) cases, the anomalous systemic artery originated from the descending thoracic aorta just adjacent to the sequestrated lung which it supplied, without the presence of accompanying bronchi. In twenty (87.0%) patients who received the surgical intervention, samples of 12 (85.7%) were proved to have elastic vessel walls, out of the 14 samples in which the anomalous systemic arteries were available for analysis.

          CONCLUSIONS:

          There are no certain pathology diagnostic criteria for the diagnosis of PS. The detecting of the aberrant systematic artery and distinguishing it from the bronchial arteries corresponded to certain lung abnormalities are the keys to the accurate diagnosis of pulmonary sequestration in adult patients. We propose that the characteristic features of the anomalous arteries include: Originating from aorta and its main branches, adjacent to the sequestrated area, directly running into the sequestrated mass without accompanying bronchus branch, being large in diameter, and having elastic vessel wall.

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          Most cited references16

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          Pulmonary sequestration: a retrospective analysis of 2625 cases in China.

          Pulmonary sequestration is a congenital lung malformation, which is often misdiagnosed as lung cancer, pulmonary cysts, mediastinal tumors, etc. Therefore, more research on the clinical characteristics of pulmonary sequestration should be carried out to improve the preoperative diagnosis rate. The study used was a retrospective analysis of 2625 cases of pulmonary sequestration well documented in the Chinese National Knowledge Infrastructure from 1998 to 2008. Analysis was performed on the patients' age, gender, symptom, chest computed tomography (CT) scan, chest radiograph, lesion localization, arterial supply, venous drainage, and incorrect preoperative diagnosis. A total of 2625 cases of pulmonary sequestration was reported in the Chinese National Knowledge Infrastructure from 1998 to 2008, and the male:female ratio was 1.58:1. The symptoms of pulmonary sequestration were cough, sputum, fever, hemoptysis, and chest pain, with 13.36% of patients being asymptomatic. Chest CT scan showed mass lesions (49.01%), cystic lesions (28.57%), cavitary lesions (11.57%), and pneumonic lesions (7.96%). The sequestration was mainly located in the lower lobe, primarily in the left posterior basal segment (66.43%) and in the right posterior basal segment (20.16%). Pulmonary sequestrations were divided into two types, intralobar sequestration (83.95%) and extralobar sequestration (16.05%). Bilateral sequestrations were rare--only three cases had been reported. The arterial supply was mainly provided by branches of thoracic aorta (76.55%) and abdominal aorta (18.47%). The mean incorrect preoperative diagnosis rate was as high as 58.63%. A comparison between pediatric and adult patients indicated that the subtype ratio (intralobar/extralobar) was higher in the adult group than that in the pediatric group (P < 0.001). Clinical manifestations of pulmonary sequestration varied and preoperative diagnosis was often incorrect. To improve the preoperative diagnosis rate, we should take full advantage of symptoms, image performance, and localization characteristics. A certain early-onset age, recurrent pneumonias, mass or cyst lesion located in the lower lobe, and aberrant arterial supply are indicators for the diagnosis. Crown Copyright © 2011. Published by Elsevier B.V. All rights reserved.
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            Airway and blood vessel interaction during lung development.

            In the adult lung the pulmonary arteries run alongside the airways and the pulmonary veins show a similar branching pattern to the arteries, though separated from them. During early fetal development the airways act as a template for pulmonary blood vessel development in that the vessels form by vasculogenesis around the branching airways. In later lung development the capillary bed is essential for alveolar formation. This paper reviews evidence for the interaction of the airways and blood vessels in both normal and abnormal lung development.
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              Imaging in bronchopulmonary sequestration.

              Bronchopulmonary sequestration is an uncommon pulmonary disorder characterized by an area of non-functioning abnormal lung tissue, which receives its blood supply from a systemic artery and characteristically has no connection with the tracheobronchial tree. The abnormal lung tissue is located within the visceral pleura of a pulmonary lobe in the intralobar variety, whereas the extralobar form has its own visceral pleura. The venous drainage of the extralobar type is usually into the systemic veins, whereas the intralobar type drains into the pulmonary veins. Radiological imaging plays a vital role in establishing the diagnosis, and even more importantly, in providing to the clinician a vascular roadmap essential for surgical planning. We present here a review of bronchopulmonary sequestration and also discuss the role of various imaging methods in the early diagnosis and management of these cases.
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                Author and article information

                Journal
                Ann Thorac Med
                Ann Thorac Med
                ATM
                Annals of Thoracic Medicine
                Medknow Publications & Media Pvt Ltd (India )
                1817-1737
                1998-3557
                Jan-Mar 2017
                : 12
                : 1
                : 46-50
                Affiliations
                [1] Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
                [1 ] Department of Pathology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
                [2 ] Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
                Author notes
                Address for correspondence: Prof. Jing Li, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100032, China. E-mail: lijing6515@ 123456pumch.cn Prof. Baiqiang Cai, Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, 100730, China. E-mail: caibq2009@ 123456hotmail.com
                Article
                ATM-12-46
                10.4103/1817-1737.197778
                5264173
                a07f6e81-f202-47f9-b553-ef0f09861eac
                Copyright: © 2017 Annals of Thoracic Medicine

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                : 24 May 2016
                : 30 August 2016
                Categories
                Original Article

                Respiratory medicine
                aberrant systematic artery,congenital anomalous artery,pulmonary sequestration

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