Respir Case Rep. 2015; 4(3): 200-203 | DOI: 10.5505/respircase.2015.58070

Scimitar Sendromu: Olgu Sunumu

Emine Aksoy, Dilem Anýl Tokyay, Fatma Tokgöz Akyýl, Ayþe Ýrem Kýlýç, Tülin Sevim
Süreyyapaþa Göðüs Hastalýklarý ve Göðüs Cerrahisi Eðitim ve Araþtýrma Hastanesi, Göðüs Hastalýklarý Kliniði, Ýstanbul

Scimitar sendromu veya pulmoner venolober sendrom, nadir görülen bir konjenital venöz dönüþ anomalisidir. Kýrk yedi yaþýnda kadýn hasta, göðüs aðrýsý ve eforla geliþen nefes darlýðý þikâyeti ile baþvurdu. Fizik muayenesi ve laboratuvar bulgularý normaldi. Postero-anterior akciðer grafisinde sað akciðer alt alanda sað kalp kenarý boyunca nonhomojen opasite artýþý, sað akciðerde volüm kaybý, kalp gölgesinin saða doðru yer deðiþtirdiði izlendi. Ekokardiyografide sistolik pulmoner arter basýncý 55 mmHg ölçüldü. Fiberoptik bronkoskopide sað bronþial sistemde ara bronþun olmadýðý görüldü. Manyetik rezonans anjiografide, Scimitar sendromu ile uyumlu olarak sað pulmoner venlerin subdiafragmatik mesafede inferior vena kavaya drene olduðu izlendi. Anormal pulmoner venöz dönüþ, sað akciðer hipoplazisi, ara bronþ anomalisi ve pulmoner hipertansiyon ile Scimitar sendromu tanýsý konulan olgumuz literatürler eþliðinde sunuldu.

Anahtar Kelimeler: Pulmoner hipertansiyon, pulmoner ven, scimitar sendromu.

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Scimitar Syndrome: A Case Report

Emine Aksoy, Dilem Anýl Tokyay, Fatma Tokgöz Akyýl, Ayþe Ýrem Kýlýç, Tülin Sevim
Department of Chest Diseases, Süreyyapaþa Chest Diseases and Thoracic Surgery Training and Research Hospital, Ýstanbul, Turkey

Scimitar syndrome or pulmonary venolabar syndrome is a rare congenital anomalous pulmonary venous return. A 47-year-old woman presented at our clinic with chest pain and shortness of breath during exertion. The physical examination and laboratory findings were normal. The chest radiograph demonstra-ted non-homogeneous increased opacity along the right cardiac border, and volume loss of the right lung with cardiac silhouette displacement, to the right. Echocardiography pulmonary artery systolic pressure was measured as 55 mmHg. Fiberoptic bronchoscopy revealed the intermediate bronchus was absent. On magnetic resonance angiography, it was observed that the right pulmonary veins drained into the inferior vena cava at a subdiaphragmatic level, consistent with Scimitar Syndrome. The case, diagnosed as Scimitar syndrome, is presented in the light of the literature.

Keywords: Pulmonary hypertension, pulmonary vein, scimitar syndrome.

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ATIF KOPYALA