단일융모막쌍태임신에서 일측태아의 우측폐무발생증
RIGHT PULMONARY AGENESIS OF ONE FETUS IN A MONOCHORIONIC TWIN PREGNANCY: A CASE REPORT
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초록
일측폐무형성증은 편측에 폐실질, 혈관조직 및 기관지가 완전히 없는 드문 질환으로 산전진단되는 경우는 매우 드물다. 산전초음파에서태아의 심장과 중격의 이동이 있고 횡격막 결손이 없는 경우에 의심해 볼 수 있으며, 도플러검사로 폐동맥의 분지가 없는 것을 확인하여진단할 수 있다. 예후는 동반이상 여부에 따라 다른데, 초음파 소견의 심각함과는 달리 증상이 없을 수 있고 정상적인 성장이 가능하여 성인이 된 후에 우연히 발견되는 경우도 있다. 이에 산전진단되는 경우에도 선택적 유산이 권고되지 않는다. 저자들은 우측 폐무형성증으로산전진단되었고 분만 후 신생아 경과 양호하였던 예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
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 fi ndings 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 fi nding 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.
참고문헌 (13)
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