Difficulties in the Differential Diagnosis of Tracheobronchomegaly in Children

Tracheobronchomegaly is a rare developmental defect in children, which is characterized by the expansion of the trachea and large bronchi, the diagnosis and treatment of which are made more often in adult practice, when the clinical diagnostic picture acquires the obvious features of Mounier-Kuhn sy...

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Main Authors: N. A. Il’ina, L. E. Vorob’eva, M. D. Bakradze, A. S. Polyakova
Format: Article
Language:English
Published: Luchevaya Diagnostika, LLC 2020-06-01
Series:Вестник рентгенологии и радиологии
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Online Access:https://www.russianradiology.ru/jour/article/view/568
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author N. A. Il’ina
L. E. Vorob’eva
M. D. Bakradze
A. S. Polyakova
author_facet N. A. Il’ina
L. E. Vorob’eva
M. D. Bakradze
A. S. Polyakova
author_sort N. A. Il’ina
collection DOAJ
description Tracheobronchomegaly is a rare developmental defect in children, which is characterized by the expansion of the trachea and large bronchi, the diagnosis and treatment of which are made more often in adult practice, when the clinical diagnostic picture acquires the obvious features of Mounier-Kuhn syndrome. In this connection, the need arises for the early detection of this pathology in children and its differentiation from other diseases and malformations of the tracheobronchial tree, which mask a recurrent inflammatory process in the lung and upper respiratory tract, and primarily with those, such as cystic fibrosis and primary ciliary dyskinesia.The paper describes a case of secondary tracheobronchomegaly in a child with obvious clinical manifestations of Mounier-Kuhn syndrome in the presence of bronchiectasis, pulmonary fibrosis, and signs of systemic connective tissue disease. It discusses the possibilities of chest computed tomography in the diagnosis of this defect in children, as well as its causes. The sizes of the trachea and main bronchi in this patient are compared with those in the groups of children of the same age and sex who have cystic fibrosis or diffuse pulmonary fibrosis to identify a more significant role of hereditary connective tissue diseases in the development of secondary tracheobronchomegaly. The authors propose to separate the concepts of tracheobronchomegaly and Mounier-Kuhn syndrome in children and to recognize the threshold for tracheal expansion in these patients having +3SD or more.
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spelling doaj-art-d8a380d101a840ad829af0bf593b948e2025-08-20T03:35:08ZengLuchevaya Diagnostika, LLCВестник рентгенологии и радиологии0042-46762619-04782020-06-01101317518210.20862/0042-4676-2020-101-3-175-182338Difficulties in the Differential Diagnosis of Tracheobronchomegaly in ChildrenN. A. Il’ina0L. E. Vorob’eva1M. D. Bakradze2A. S. Polyakova3I.I. Mechnikov North-Western State Medical University, Ministry of Health of the Russian FederationNational Medical Research Center of Children’s Health, Ministry of Health of the Russian FederationNational Medical Research Center of Children’s Health, Ministry of Health of the Russian Federation; Pirogov Russian National Research Medical University, Ministry of Health of the Russian FederationNational Medical Research Center of Children’s Health, Ministry of Health of the Russian Federation; Pirogov Russian National Research Medical University, Ministry of Health of the Russian FederationTracheobronchomegaly is a rare developmental defect in children, which is characterized by the expansion of the trachea and large bronchi, the diagnosis and treatment of which are made more often in adult practice, when the clinical diagnostic picture acquires the obvious features of Mounier-Kuhn syndrome. In this connection, the need arises for the early detection of this pathology in children and its differentiation from other diseases and malformations of the tracheobronchial tree, which mask a recurrent inflammatory process in the lung and upper respiratory tract, and primarily with those, such as cystic fibrosis and primary ciliary dyskinesia.The paper describes a case of secondary tracheobronchomegaly in a child with obvious clinical manifestations of Mounier-Kuhn syndrome in the presence of bronchiectasis, pulmonary fibrosis, and signs of systemic connective tissue disease. It discusses the possibilities of chest computed tomography in the diagnosis of this defect in children, as well as its causes. The sizes of the trachea and main bronchi in this patient are compared with those in the groups of children of the same age and sex who have cystic fibrosis or diffuse pulmonary fibrosis to identify a more significant role of hereditary connective tissue diseases in the development of secondary tracheobronchomegaly. The authors propose to separate the concepts of tracheobronchomegaly and Mounier-Kuhn syndrome in children and to recognize the threshold for tracheal expansion in these patients having +3SD or more.https://www.russianradiology.ru/jour/article/view/568tracheobronchomegalymounier-kuhn syndromecongenital lung malformationscongenital bronchial malformationsbronchiectasiscystic fibrosiskartagener’s syndromeprimary ciliary dyskinesiapulmonary fibrosischronic lung diseasescomputed tomography
spellingShingle N. A. Il’ina
L. E. Vorob’eva
M. D. Bakradze
A. S. Polyakova
Difficulties in the Differential Diagnosis of Tracheobronchomegaly in Children
Вестник рентгенологии и радиологии
tracheobronchomegaly
mounier-kuhn syndrome
congenital lung malformations
congenital bronchial malformations
bronchiectasis
cystic fibrosis
kartagener’s syndrome
primary ciliary dyskinesia
pulmonary fibrosis
chronic lung diseases
computed tomography
title Difficulties in the Differential Diagnosis of Tracheobronchomegaly in Children
title_full Difficulties in the Differential Diagnosis of Tracheobronchomegaly in Children
title_fullStr Difficulties in the Differential Diagnosis of Tracheobronchomegaly in Children
title_full_unstemmed Difficulties in the Differential Diagnosis of Tracheobronchomegaly in Children
title_short Difficulties in the Differential Diagnosis of Tracheobronchomegaly in Children
title_sort difficulties in the differential diagnosis of tracheobronchomegaly in children
topic tracheobronchomegaly
mounier-kuhn syndrome
congenital lung malformations
congenital bronchial malformations
bronchiectasis
cystic fibrosis
kartagener’s syndrome
primary ciliary dyskinesia
pulmonary fibrosis
chronic lung diseases
computed tomography
url https://www.russianradiology.ru/jour/article/view/568
work_keys_str_mv AT nailina difficultiesinthedifferentialdiagnosisoftracheobronchomegalyinchildren
AT levorobeva difficultiesinthedifferentialdiagnosisoftracheobronchomegalyinchildren
AT mdbakradze difficultiesinthedifferentialdiagnosisoftracheobronchomegalyinchildren
AT aspolyakova difficultiesinthedifferentialdiagnosisoftracheobronchomegalyinchildren