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

• Published in: Journal of pediatric surgery 2004-08, Vol.39 (8), p.e13-e17
• Main Authors: McLean, Sean E., Pfeifer, John D., Siegel, Marilyn J., Jensen, Eric R., Schuler, Pamela M., Hirsch, Russel, Mychaliska, George B.
• Format: Article
• Language: English
• 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
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2004.04.044

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.