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Laparoscopic Endoscopic Surgical Science Sa Pulmoner Arter Agenezisinin Elik Ettii Scimitar (Pala) Sendromu [Respir Case Rep]
Respir Case Rep. 2013; 2(1): 44-47 | DOI: 10.5505/respircase.2013.96268

Sa Pulmoner Arter Agenezisinin Elik Ettii Scimitar (Pala) Sendromu

Dilaver Ta, Ouzhan Okutan, Tuncer zksa, mer Ayten, Ersin Demirer, Turgut ztutgan
GATA Haydarpaa Eitim Hastanesi

Scimitar sendromu, nadir bir hastalktr ve hipogenetik akcier sendromu olarak da bilinir. Sendrom sa pulmoner venin, vena kava inferiora drenaj ile karakterizedir. Burada sa pulmoner arter agenezisinin elik ettii Scimitar sendromu sunuldu. Yirmi bir yanda erkek hasta nefes darl, ksrk, balgam karma ikayetleri ile bavurdu. Akcier grafisinde sa akcierde volm kayb, kalp ve mediastende saa doru yer deitirme izlendi. Bronkoskopik incelemede ve toraks BTde orta lob bronu izlenmedi. Toraks BT anjiografi grntlerinde sa akcier alt loba ait venin sa hepatik vene drene olduu ve sa pulmoner arter apnda azalma izlendi. Akcier perfzyon sintigrafisinde sa akcierde total perfzyon kayb izlendi. Scimitar sendromlu hastalarda akcier grafisinde scimitar (pala) iaretinin olmas karakteristiktir. Ancak bu iaret her zaman grlmeyebilir. Akcier grafisinde volm kayb ve mediastinal ifti olan gen hastalarda dier hastalklarn yannda Scimitar Sendromu da akla gelmelidir.

Anahtar Kelimeler: Scimitar sendromu, pulmoner arter agenezisi, BT anjiografi.

Scimitar Syndrome Associated with Agenesis of Right Pulmonary Artery

Dilaver Ta, Ouzhan Okutan, Tuncer zksa, mer Ayten, Ersin Demirer, Turgut ztutgan
GATA Haydarpaa Training Hospital

Scimitar syndrome is a very rare disorder and also known as hypogenetic lung syndrome. The syndrome is characterized with anomalous connection of the pulmonary vein with the vena cava inferior. In this text, scimitar syndrome associated with pulmonary artery hypoplasia is presented. A 21-year-old male patient with complaints of cough, shortness of breath and sputum was admitted. Chest X-ray revealed volume loss of right lung and shift to the right of heart and mediastinum. The middle lobe wasnt observed in bronchoscopy and thorax CT. Thoracic CT angi-ography images revealed that vein of right lung lower lobe drains right hepatic vein and right pulmonary artery diameter is reduced. Lung perfusion scintigraphy revealed total loss of perfusion of right lung. Scimitar sign on chest X ray is characteristics for scimitar syndrome. However, this sign cannot be seen in some patients. If there is volume loss of right lung and shift to the right of heart and mediastinum, scimitar syndrome should be considered in addition to other diseases.

Keywords: Scimitar syndrome, agenesis of pulmonary artery, CT angiography.

Dilaver Ta, Ouzhan Okutan, Tuncer zksa, mer Ayten, Ersin Demirer, Turgut ztutgan. Scimitar Syndrome Associated with Agenesis of Right Pulmonary Artery. Respir Case Rep. 2013; 2(1): 44-47

Sorumlu Yazar: Dilaver Ta, Trkiye
Makale Dili: Trke
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