Respir Case Rep. 2013; 2(1): 44-47 | DOI: 10.5505/respircase.2013.96268

Sað Pulmoner Arter Agenezisinin Eþlik Ettiði Scimitar (Pala) Sendromu

Dilaver Taþ, Oðuzhan Okutan, Tuncer Özkýsa, Ömer Ayten, Ersin Demirer, Turgut Öztutgan
GATA Haydarpaþa Eðitim Hastanesi

Scimitar sendromu, nadir bir hastalýktýr ve hipogenetik akciðer sendromu olarak da bilinir. Sendrom sað pulmoner venin, vena kava inferiora drenajý ile karakterizedir. Burada sað pulmoner arter agenezisinin eþlik ettiði Scimitar sendromu sunuldu. Yirmi bir yaþýnda erkek hasta nefes darlýðý, öksürük, balgam çýkarma þikayetleri ile baþvurdu. Akciðer grafisinde sað akciðerde volüm kaybý, kalp ve mediastende saða doðru yer deðiþtirme izlendi. Bronkoskopik incelemede ve toraks BT’de orta lob bronþu izlenmedi. Toraks BT anjiografi görüntülerinde sað akciðer alt loba ait venin sað hepatik vene drene olduðu ve sað pulmoner arter çapýnda azalma izlendi. Akciðer perfüzyon sintigrafisinde sað akciðerde total perfüzyon kaybý izlendi. Scimitar sendromlu hastalarda akciðer grafisinde scimitar (pala) iþaretinin olmasý karakteristiktir. Ancak bu iþaret her zaman görülmeyebilir. Akciðer grafisinde volüm kaybý ve mediastinal þifti olan genç hastalarda diðer hastalýklarýn yanýnda Scimitar Sendromu da akla gelmelidir.

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

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Scimitar Syndrome Associated with Agenesis of Right Pulmonary Artery

Dilaver Taþ, Oðuzhan Okutan, Tuncer Özkýsa, Ömer Ayten, Ersin Demirer, Turgut Öztutgan
GATA Haydarpaþa 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 wasn’t 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.

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