Pediatric Diffuse Pulmonary Lymphangiomatosis: CT Characteristics

Objective: To explore the typical CT features of diffuse pulmonary lymphangiomatosis in children and improve the diagnostic accuracy. Methods: The radiologic data of 13 children with diffuse pulmonary lymphangiomatosis confirmed by clinical, imaging and pathological biopsy were reviewed retrospectiv...

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Main Authors: Jun GAO, Tong YU, Xiaomin DUAN, Xiaoyan ZHANG, Yun PENG
Format: Article
Language:English
Published: Editorial Office of Computerized Tomography Theory and Application 2022-08-01
Series:CT Lilun yu yingyong yanjiu
Subjects:
Online Access:https://www.cttacn.org.cn/cn/article/doi/10.15953/j.ctta.2022.103
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author Jun GAO
Tong YU
Xiaomin DUAN
Xiaoyan ZHANG
Yun PENG
author_facet Jun GAO
Tong YU
Xiaomin DUAN
Xiaoyan ZHANG
Yun PENG
author_sort Jun GAO
collection DOAJ
description Objective: To explore the typical CT features of diffuse pulmonary lymphangiomatosis in children and improve the diagnostic accuracy. Methods: The radiologic data of 13 children with diffuse pulmonary lymphangiomatosis confirmed by clinical, imaging and pathological biopsy were reviewed retrospectively, including 6 girls and 7 boys, with an average age of 5.8 years. All the 13 cases underwent CT plain scan and enhanced examination. Results: The characteristic CT findings of diffuse pulmonary lymphangiomatosis in this group were as follows: (1) Mediastinum and hilum, peribronchovascular, interlobular septa were involved; (2) The thickening of soft tissue in mediastinum continued with the thickening of soft tissue around hilum and bronchovascular; (3) The soft tissue density in the mediastinum was low, no enhancement was observed under contrast, and there was no compression or shift in the brachiocephalic great vessels; (4) Hilar thickening, smooth thickening of soft tissue around bronchovascular and interlobular septa, most of which involved both sides of the lung, and a few involved one side at the same time; (5) Pleural thickening or pleural effusion; (6) Spleen involvement is common; (7) Cervical and or axillary lymph node enlargement and density reduction; (8) The density of several or single vertebrae wasreduced. Laboratory examination with thrombocytopenia and/or DIC was considered Kaposiform lymphangiomatosis. Conclusion: CT findings of diffuse pulmonary lymphangioma lymphangiomatosis in children show high specificity, when combined with the age of onset and clinical laboratory examinations, can significantly improve the diagnostic coincidence rate of the disease.
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spelling doaj.art-2e1b9a7ad8e34879a4441c713681879e2023-09-03T13:52:26ZengEditorial Office of Computerized Tomography Theory and ApplicationCT Lilun yu yingyong yanjiu1004-41402022-08-0131442543210.15953/j.ctta.2022.1032022-103Pediatric Diffuse Pulmonary Lymphangiomatosis: CT CharacteristicsJun GAO0Tong YU1Xiaomin DUAN2Xiaoyan ZHANG3Yun PENG4Department of Radiology, Beijing Children’s Hospital, Capital Medical University/ National Center for Children's Health, Beijing 100045, ChinaDepartment of Radiology, Beijing Children’s Hospital, Capital Medical University/ National Center for Children's Health, Beijing 100045, ChinaDepartment of Radiology, Beijing Children’s Hospital, Capital Medical University/ National Center for Children's Health, Beijing 100045, ChinaDepartment of Radiology, Beijing Children’s Hospital, Capital Medical University/ National Center for Children's Health, Beijing 100045, ChinaDepartment of Radiology, Beijing Children’s Hospital, Capital Medical University/ National Center for Children's Health, Beijing 100045, ChinaObjective: To explore the typical CT features of diffuse pulmonary lymphangiomatosis in children and improve the diagnostic accuracy. Methods: The radiologic data of 13 children with diffuse pulmonary lymphangiomatosis confirmed by clinical, imaging and pathological biopsy were reviewed retrospectively, including 6 girls and 7 boys, with an average age of 5.8 years. All the 13 cases underwent CT plain scan and enhanced examination. Results: The characteristic CT findings of diffuse pulmonary lymphangiomatosis in this group were as follows: (1) Mediastinum and hilum, peribronchovascular, interlobular septa were involved; (2) The thickening of soft tissue in mediastinum continued with the thickening of soft tissue around hilum and bronchovascular; (3) The soft tissue density in the mediastinum was low, no enhancement was observed under contrast, and there was no compression or shift in the brachiocephalic great vessels; (4) Hilar thickening, smooth thickening of soft tissue around bronchovascular and interlobular septa, most of which involved both sides of the lung, and a few involved one side at the same time; (5) Pleural thickening or pleural effusion; (6) Spleen involvement is common; (7) Cervical and or axillary lymph node enlargement and density reduction; (8) The density of several or single vertebrae wasreduced. Laboratory examination with thrombocytopenia and/or DIC was considered Kaposiform lymphangiomatosis. Conclusion: CT findings of diffuse pulmonary lymphangioma lymphangiomatosis in children show high specificity, when combined with the age of onset and clinical laboratory examinations, can significantly improve the diagnostic coincidence rate of the disease.https://www.cttacn.org.cn/cn/article/doi/10.15953/j.ctta.2022.103radiologychilddiffuse pulmonary lymphangiomatosis
spellingShingle Jun GAO
Tong YU
Xiaomin DUAN
Xiaoyan ZHANG
Yun PENG
Pediatric Diffuse Pulmonary Lymphangiomatosis: CT Characteristics
CT Lilun yu yingyong yanjiu
radiology
child
diffuse pulmonary lymphangiomatosis
title Pediatric Diffuse Pulmonary Lymphangiomatosis: CT Characteristics
title_full Pediatric Diffuse Pulmonary Lymphangiomatosis: CT Characteristics
title_fullStr Pediatric Diffuse Pulmonary Lymphangiomatosis: CT Characteristics
title_full_unstemmed Pediatric Diffuse Pulmonary Lymphangiomatosis: CT Characteristics
title_short Pediatric Diffuse Pulmonary Lymphangiomatosis: CT Characteristics
title_sort pediatric diffuse pulmonary lymphangiomatosis ct characteristics
topic radiology
child
diffuse pulmonary lymphangiomatosis
url https://www.cttacn.org.cn/cn/article/doi/10.15953/j.ctta.2022.103
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AT tongyu pediatricdiffusepulmonarylymphangiomatosisctcharacteristics
AT xiaominduan pediatricdiffusepulmonarylymphangiomatosisctcharacteristics
AT xiaoyanzhang pediatricdiffusepulmonarylymphangiomatosisctcharacteristics
AT yunpeng pediatricdiffusepulmonarylymphangiomatosisctcharacteristics