Congenital cystic adenomatoid malformation connected to an extralobar pulmonary sequestration in the contralateral chest: Common origin?

Extralobar pulmonary sequestration (ELS) and congenital cystic adenomatoid malformation have been reported to coexist in several variations. This suggests a common embryologic origin. A 6-month-old boy presented with a history of recurrent pneumonias. The patient was diagnosed with a right lower lob...

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Veröffentlicht in:Journal of pediatric surgery 2004-08, Vol.39 (8), p.e13-e17
Hauptverfasser: McLean, Sean E., Pfeifer, John D., Siegel, Marilyn J., Jensen, Eric R., Schuler, Pamela M., Hirsch, Russel, Mychaliska, George B.
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container_end_page e17
container_issue 8
container_start_page e13
container_title Journal of pediatric surgery
container_volume 39
creator McLean, Sean E.
Pfeifer, John D.
Siegel, Marilyn J.
Jensen, Eric R.
Schuler, Pamela M.
Hirsch, Russel
Mychaliska, George B.
description Extralobar pulmonary sequestration (ELS) and congenital cystic adenomatoid malformation have been reported to coexist in several variations. This suggests a common embryologic origin. A 6-month-old boy presented with a history of recurrent pneumonias. The patient was diagnosed with a right lower lobe congenital cystic adenomatoid malformation (CCAM) and a left lower lobe ELS/CCAM. The diagnosis was made with the aid of a multidetector computed tomography (CT). Three-dimensional CT reconstruction showed the presence of a right lower lobe CCAM, a left lower ELS with an aberrant arterial supply from the celiac axis, and possible venous drainage into the right CCAM. The patient underwent a right thoracotomy. Intraoperatively, the lesions were discovered to be connected by a band of tissue. The right lower lobe CCAM and the left ELS were removed from the right chest. Histologic analysis confirmed the presence of a CCAM within the right lower lobe. The ELS had involvement of a type II CCAM within the sequestration. The connection between the right CCAM and left ELS/CCAM showed an anomalous conducting airway, anomalous vein, and anomalous artery connecting the 2 lesions. The authors present the first case of a CCAM connected to an ELS/CCAM in the contralateral hemithorax. The unique anatomic configuration of these lesions suggests a common embryologic origin of ELS and CCAM.
doi_str_mv 10.1016/j.jpedsurg.2004.04.044
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subjects Bronchopulmonary Sequestration - diagnostic imaging
Bronchopulmonary Sequestration - embryology
Bronchopulmonary Sequestration - surgery
congenital cystic adenomatoid malformation
congenital lung malformations
Cystic Adenomatoid Malformation of Lung, Congenital - diagnostic imaging
Cystic Adenomatoid Malformation of Lung, Congenital - embryology
Cystic Adenomatoid Malformation of Lung, Congenital - surgery
Extralobar pulmonary sequestration
Humans
Imaging, Three-Dimensional
Infant
Infant, Newborn
Lung - blood supply
Lung - embryology
lung development
Male
Mesoderm - physiology
Models, Biological
Pneumonia - etiology
Recurrence
Respiratory Distress Syndrome, Newborn - etiology
Thoracotomy
Tomography, Spiral Computed
title Congenital cystic adenomatoid malformation connected to an extralobar pulmonary sequestration in the contralateral chest: Common origin?
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