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 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.
Department of Surgery, Washington University School of Medicine, St Louis, MO, USA.
Sean E
SE
McLean
John D
JD
Pfeifer
Marilyn J
MJ
Siegel
Eric R
ER
Jensen
Pamela M
PM
Schuler
Russel
R
Hirsch
George B
GB
Mychaliska
D001998:Bronchopulmonary Sequestration / Q000000981:diagnostic imaging / Q000196:embryology* / Q000601:surgery; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000000981:diagnostic imaging / Q000196:embryology* / Q000601:surgery; D006801:Humans; D021621:Imaging, Three-Dimensional; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000098:blood supply / Q000196:embryology; D008297:Male; D008648:Mesoderm / Q000502:physiology; D008954:Models, Biological; D011014:Pneumonia / Q000209:etiology; D012008:Recurrence; D012127:Respiratory Distress Syndrome, Newborn / Q000209:etiology; D013908:Thoracotomy; D036542:Tomography, Spiral Computed