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HOME > J Pathol Transl Med > Volume 30(2); 1996 > Article
Case Report Unilateral Pulmonary Agenesis Combined with other Unusual Anomalies: An autopsy report.
Ik Su Kim, Sang Han Lee, In Soo Shu
Journal of Pathology and Translational Medicine 1996;30(2):166-168
DOI: https://doi.org/
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Department of Pathology, Kyungpook National University School of Medicine, 700-422 Taegu, Korea.

Pulmonary agenesis is a very rare anomaly. It is defined as total absence of the pulmonary parenchyma, vascular structures, and bronchi beyond the carina. We experienced a case of right pulmonary agenesis in association with other congenital defects who died at 1 day of age. The other defects included: esophageal atresia, tracheoesophageal fistula, cardiac malformation, anal atresia and a malformed left thumb. The cardiac malformations were a type of Pentalogy of Fallot, composed of right ventricular hypertrophy, ventricular septal defect, an overiding of aorta, pulmonary atresia, and an atrial septal defect. Hand roentgenograms of the malformed left thumb showed an unarticulated metacarpopharyngeal joint. This unique combination of anomalies is extremely rare.

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