Korean J Obstet Gynecol.  2011 Dec;54(12):798-801. 10.5468/KJOG.2011.54.12.798.

A case of prenatal diagnosis of unilateral pulmonary agenesis

• Lee HJ
• Kim SY
• Park JW
• Kim WO
• Park CW
• Park JS
• Jun JK
• Syn HC

• KMID: 1443945
• DOI: http://doi.org/10.5468/KJOG.2011.54.12.798

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.

Keyword

Pulmonary agenesis; Prenatal diagnosis; Fetal ultasound

MeSH Terms

Female
Humans
Lung
Lung Diseases
Pregnancy
Pregnancy Trimester, Second
Prenatal Diagnosis
Prognosis
Stillbirth
Lung
Lung Diseases

Figure

• 

  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.

Reference

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.