Agenesis of The Lung
Agenesis of The Lung
Agenesis of The Lung
1989, 2, 690--692
Agenesis of the lung. I. Safa Kaya, U. Dilmen . Dept of Pediatrics, Turkish Health and Therapy
ABSTRACT: Agenesis of the lung is extremely rare. Developmental de- Foundation, Memorial Ahrnet C>rs Hospital, Emck,
fects of the lung are usually associated with other congenital malfor- Ankara, Turkey.
mations. Half of all reported patients die either at birth or within the
Correspondence: I. Safa Kaya, Misket Sk. No: 30,
first 5 yrs of life. We report a case of right lung agenesis with absence 06510 Emek, Ankara, Turkey.
of the left kidney, and fusion anomaly between ribs 4 and 5 on the left
hemithorax. Keywords: Kidney agenesis; lung agenesis.
Eur Respir J., 1989, 2, 690-692.
Received: November, 1988; accepted for publication
February 9, 1989.
Agenesis, affecting both hemithoraces and both sexes in the right lung, and a partial absence of perfusion at
almost equally, should be separated for physiological the apex of the left lung (Fig. 2). The i.v. pyelogram
reasons from hypoplasia by the total absence of lung (Fig. 3) and abdominal ultrasonogram disclosed absence
parenchyma, bronchial tree, and supporting vasculature of the left kidney as well as hydronephrosis and hy-
[I]. This extremely rare condition was first described droureter in the right kidney. Electrocardiographic ex-
by De Pozze who discovered it accidentally at the au- amination and blood chemistry analysis were within
topsy of an adult female in 1673 [2]. Genetic, terato- normal limits.
genic, and mechanical factors may have a bearing on The child expired on the forty-fifth day. The parents
the aetiology. Developmental defects of the lung are did not give consent for postmortem examination.
usually associated with other congenital malformations,
which include the skeletal, cardiovascular, gastrointes-
tinal, and genitourinary systems (1-8].
Case report
reduces the resistance to respiratory infection. This may 8. Sbokos CG, McMillan IKR. - Agenesis of the lung. Br
account for the higher mortality and morbidity [1, 7, J Dis Chest, 1977, 71, 183- 197.
8, 12]. 9. Oyamada A, Gasul BM, Holinger PH.- Agenesis of the
lung. Report of a case with review of all previously recorded
Acknowledgement: We are greatly indebted to cases. Am J Dis Child, 1953, 85, 182-201.
Prof. K.F. Kerrebijn for his editorial assistance. 10. Konishi H, Nakamura H, Mizukami Y, et al.- A case
of pulmonary agenesis associated with congenital tracheal
stenosis and aberrant left pulmonary artery. Rinsho Hoshasen,
1986, 31, 741-744.
References
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