항문 막힘증을 동반한 Scimitar 증후군 1례
A Rare Case of Scimitar Syndrome Accompanied with an Imperforate Anus in a Newborn
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Scimitar증후군은 우측 폐정맥이 하대정맥을 통하여 좌심방이 아닌 우심방으로 연결되는 부분 폐 정맥 환류이상을 보이면서 우측 폐의 발육부전, 우폐동맥의 발육부전 및 폐 분리증 등과 같은 동반 기형을 보이는 매우 드문 심폐기형 가운데 하나이다. 또한 선천성 항문 막힘증은 출생 후 조기에 잘록 창자 창냄술과 같은 적극적인 수술적 치료가 요구되는 항문 직장 기형의하나로 비뇨기계 이상, 척추 이상, 기관지 식도루, 식도 폐쇄증 혹은 심혈관계 이상 등이 동반될 수 있다. 그러나, 부분 폐 정맥 환류 이상의 하나인 scimitar증후군이 항문 막힘증과 함께 동반됨을 보고한 예는 아직까지 국내외적으로 없었다. 이에 저자들은호흡 곤란 및 선천성 편측 폐 발육부전 및 항문 막힘증을 주소로 입원한 신생아에서 진단된 scimitar 증후군 1례를 경험하였기에 보고하는 바이다.
Abstract
Scimitar syndrome (SS) is a rare congenital anomaly characterized by hypoplasia of the right lung and partially anomalous pulmonary venous drainage to the inferior vena cava. The term scimitar derives from the shadow created by the anomalous pulmonary vein on chest X-ray that closely resembles that of a curved Turkish sword. It rarely presents as an isolated abnormality. Various cardiac and non-cardiac anomalies have been association with SS, such as right lung hypoplasia, dextroposition of the heart, hypoplasia of the right pulmonary artery, systemic arterial blood supply to the right lower lung from the infra-diaphragmatic aorta, and a secundum type of atrial septal defect. However, an imperforate anus has not been reported previously in association with SS. We describe the first case of infantile scimitar syndrome accompanied with an imperforate anus in a newborn who presented with tachypnea and right pulmonary atelectasis.
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