Journal List > Korean J Obstet Gynecol > v.54(12) > 1088379

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Lee, Kim, Park, Kim, Park, Park, Jun, and Syn: A case of prenatal diagnosis of unilateral pulmonary agenesis
Case Report
Korean Journal of Obstetrics & Gynecology

Korean Journal of Obstetrics & Gynecology 2011; 54(12): 798-801.

Published online: 15 December 2011

DOI: https://doi.org/10.5468/KJOG.2011.54.12.798

A case of prenatal diagnosis of unilateral pulmonary agenesis

Hee Jun Lee, MD, Sun Young Kim, MD, Jeong Woo Park, MD, Woo-Oh Kim, MD, Chan-Wook Park, MD, PhD, Joong Shin Park, MD, PhD, Jong Kwan Jun, MD, PhD, Hee Chul Syn, MD, PhD

Department of Obstetrics and Gynecology, Seoul National University College of Medicine, Seoul, Korea.

Corresponding author: Jong Kwan Jun, MD, PhD. Department of Obstetrics and Gynecology, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 110-744, Korea. Tel: +82-2-2072-3744 Fax: +82-2-762-3599, jhs0927@snu.ac.kr

Received 31 May 2011       Accepted 4 July 2011

Copyright © 2011. Korean Society of Obstetrics and Gynecology

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Unilateral pulmonary agenesis is a very rare developmental malformation that is often associated with other anomalies. The prognosis of pulmonary agenesis varies from stillbirth, to neonatal death, to survival without any symptoms depending on the presence of associated congenital malformations. Therefore, it is very important to perform a prenatal diagnosis of unilateral pulmonary agenesis. We describe a case of unilateral pulmonary agenesis diagnosed in the second trimester by transabdominal ultrasound.

Keywords: Pulmonary agenesis, Prenatal diagnosis, Fetal ultasound

Figures and Tables

Fig. 1
Coronal section of fetal thorax (4-chamber view) at 21 weeks' gestation by transabdominal sonogram. The cross-sectional view of the fetal thorax shows normal pulmonary parenchyma on the left side and the absence of pulmonary parenchyma on the right side, resulting in the mediastinal and cardiac shift toward the right side. Mild cardiomegaly can be detected surrounded by a small amount of pericardial fluid. LT, indicates left; RT, right.
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Fig. 2
Fetal echocardiogram at 23 weeks' gestation. Short-axis view of the great vessels in the fetus shows the absence of the right pulmonary artery and pulmonary vein. The right lung could not be detected and the heart was shifting to the right side.
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Fig. 3
Infantogram. Note the right-sided homogeneous opacity with ipsilateral displacement of the mediastinum toward the agenetic lung and hyperexpansion of the left lung. G-tube coiling in the proximal esophagus and distal bowel gas suggests esophageal atresia with tracheoesophageal fistula.
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References

1. Bianchi D, Crombleholme T, D'Alton M. Bianchi D, Crombleholme T, D'Alton M, editors. Pulmonary agenesis. Fetology: diagnosis and management of the fetal patient. 2000. New York: McGraw-Hill;323–328.
2. Engellenner W, Kaplan C, Van de Vegte GL. Pulmonary agenesis association with nonimmune hydrops. Pediatr Pathol. 1989. 9:725–730.
3. Finci V, Beghetti M, Kalangos A, Brundler MA. Unilateral total and contralateral partial pulmonary agenesis associated with total anomalous pulmonary venous drainage. J Pediatr. 1999. 134:510–512.
4. Yancey MK, Richards DS. Antenatal sonographic findings associated with unilateral pulmonary agenesis. Obstet Gynecol. 1993. 81:847–849.
5. Steadland KM, Langham MR Jr, Greene MA, Bagwell CE, Kays DW, Talbert JL. Unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula. Ann Thorac Surg. 1995. 59:511–513.
6. Cunningham ML, Mann N. Pulmonary agenesis: a predictor of ipsilateral malformations. Am J Med Genet. 1997. 70:391–398.
7. Aggarwal AN, Gupta D, Jindal SK. Unilateral pulmonary agenesis with ipsilateral musculoskeletal anomalies. J Assoc Physicians India. 2002. 50:1324–1326.
8. Berkenstadt M, Lev D, Achiron R, Rosner M, Barkai G. Pulmonary agenesis, microphthalmia, and diaphragmatic defect (PMD): new syndrome or association. Am J Med Genet. 1999. 86:6–8.
9. Currarino G, Williams B. Causes of congenital unilateral pulmonary hypoplasia: a study of 33 cases. Pediatr Radiol. 1985. 15:15–24.
10. Fitoz S, Uçar T, Erden A, Günlemez A. DiGeorge syndrome associated with left lung aplasia. Br J Radiol. 2001. 74:764–766.
11. Maltz DL, Nadas AS. Agenesis of the lung. Presentation of eight new cases and review of the literature. Pediatrics. 1968. 42:175–188.
12. Abdullah MM, Lacro RV, Smallhorn J, Chitayat D, van der Velde ME, Yoo SJ, et al. Fetal cardiac dextroposition in the absence of an intrathoracic mass: sign of significant right lung hypoplasia. J Ultrasound Med. 2000. 19:669–676.
13. Wu CT, Chen MR, Shih SL, Huang FY, Hou SH. Case report: agenesis of the right lung diagnosed by three-dimensional reconstruction of helical chest CT. Br J Radiol. 1996. 69:1052–1054.
14. Hansen T, Corbet A. Taeusch H, Ballard R, Gleason C, editors. Anomalies of the airways, mediastinum, and lung parenchyma. Avery's diseases of the newborn. 2005. 8th ed. Philadelphia (PA): Elsevier Saunders;737–757.
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