Korean J Obstet Gynecol.  2011 May;54(5):260-264. 10.5468/KJOG.2011.54.5.260.

Right pulmonary agenesis of one fetus in a monochorionic twin pregnancy: A case report

• Choi MR
• Kwak SH
• Cheun JY
• We JS
• Lee Y
• Shin JC

• KMID: 2078022
• DOI: http://doi.org/10.5468/KJOG.2011.54.5.260

Abstract

Unilateral pulmonary agenesis is uncommon congenital malformation which refers to total absence of pulmonary parenchyma and blood vessels as well as bronchia beyond the tracheal bifurcation. And, it is rarely to make prenatal diagnosis. Indirect prenatal sonographic findings in this malformation include mediastinal shift of the heart together with lack of evidence of diaphragmatic hernia. And more reliable diagnosis is obtained by Doppler sonographic finding with lack of branching of the pulmonary artery. Its prognosis depends largely on the presence of associated other congenital anomalies. But unilateral pulmonary agenesis does not result in the death of the infant and is compatible with long-term survival. So, selective termination is not recommended. In the case herein, right pulmonary agenesis of one fetus in a monochorionic twin pregnancy was diagnosed by prenatal sonography. We describe this case with a brief review of the literature.

Keyword

Unilateral pulmonary agenesis; Mediastinal shift; Prenatal diagnosis

MeSH Terms

Blood Vessels
Fetus
Heart
Hernia, Diaphragmatic
Humans
Infant
Lung
Lung Diseases
Pregnancy, Twin
Prenatal Diagnosis
Prognosis
Pulmonary Artery
Lung
Lung Diseases

Figure

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  Fig. 1. Transverse axial ultrasonography image at 35 gestational weeks shows dextroposition of the heart in the absence of intrathoracic mass. The septal axis is normal. The heart is shifted into the right chest owing to a right lung hypoplasia or agenesis.

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  Fig. 2. X-ray scan of the neonate's chest imaged with total haziness of Right lung field and right side deviation of mediastinal structure.

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  Fig. 3. Axial CT scan of the infant's head imaged with no visualization of right lung parenchyma and airway. Left lung shows normal structure. But, it is over-distended. Heart and mediastinal structures are displaced to the right side.

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