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Total Tracheopulmonary Agenesis Associated with Asplenia, Agenesis of Umbilical Artery and Other Anomalies
Author(s) -
DEVI BANTI,
MORE J. R. S.
Publication year - 1966
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1966.tb15215.x
Subject(s) - medicine , agenesis , anatomy , hypoplasia , embryology , azygos vein , asplenia , supernumerary , truncus arteriosus , dextrocardia , truncus , surgery , cardiology , tetralogy of fallot , heart disease , spleen
Summary A case of bilateral pulmonary agenesis and the first of total tracheopulmonary agenesis, is reported. In addition to absence of the lower portion of the larynx, trachea and lungs, there were several cardiac abnormalities, including a common atrio‐ventricular valve, persistent ostium primum, interventricular septa1 defect, truncus arteriosus and absence of both pulmonary trunk and arteries. Additional malformations present included a persistent left superior vena cava, left azygos vein, absence of the spleen, gastric hypoplasia and a hemivertebra. The embryology of the defects in the present case is discussed with particular reference to their time of onset and causation and the important findings compared with those of previously published cases. It is suggested that inception of splenic development may occur earlier than has previously been postulated.