Respir Case Rep. 2013; 2(1): 39-43 | DOI: 10.5505/respircase.2013.14622

Bronþektazili Bir Hastada Gecikmiþ Taný: Swyer-James-MacLeod Sendromu

Dilay Ahat Çimen, Aydanur Ekici, Emel Bulcun, Mehmet Ekici
1. Kýrýkkale Üniversitesi, Týp Fakültesi, Göðüs Hastalýklarý Bölümü, Kýrýkkale, Türkiye

Swyer-James/Mac Leod Sendromu (SJMS) pulmoner arter hipoplazisine baðlý unilateral hiperlüsent akciðer görünümü ve bronþektazi ile karakterize nadir görülen bir hastalýktýr. Çocukluk çaðýnda geçirilen viral bronþiolit ve pnömoniler sonrasý akkiz olarak geliþtiði düþünülmektedir. Kýrk sekiz yaþýnda kadýn hasta, nefes darlýðý, öksürük, balgam çýkarma yakýnmalarýyla baþvurdu. Öyküsünden bu þikayetlerinin çocukluk yaþlarýndan beri tekrarladýðý, 10 yýl öncesine kadar kronik bronþit, son 10 yýldýr da bronþektazi ve küçük hava yolu hastalýðý tanýlarýyla takip ve tedavi edildiði öðrenildi. Fizik muayenesinde; solunum seslerinin þiddeti sað alt bölgede sola göre azalmýþ, bilateral orta ve alt bölgede ve anteriorda kaba ralleri mevcuttu. Akciðer grafisinde; sol akciðerde hacim kaybý, radyolusensi artýþý ve alt zonda bronþektazik alanlar izlendi. Bilgisayarlý tomografide; sol pulmoner arter ve dallarýnýn kalibrasyonu saða kýyasla belirgin azalmýþtý. SJMS çocukluk ve genç eriþkin dönemde teþhis edilen bir hastalýk olmasýna karþýn, olgumuzda 48 yaþýna kadar solunum þikayetleri tanýmlamasý nedeniyle birçok kez tetkikler yapýlmasýna raðmen taný konulmamýþtýr. Tek taraflý hiperlüsent akciðer görünümü saptandýðýnda bu sendromun ayýrýcý tanýda düþünülmesi gerektiðini vurgulamak için sunuyoruz.

Anahtar Kelimeler: Bronþektazi, Hiperlüsent akciðer, Swyer-James /Macleod Sendromu.

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Delayed Diagnosis in a Patient with Bronchiectasis: Swyer-James-MacLeod Syndrome

Dilay Ahat Çimen, Aydanur Ekici, Emel Bulcun, Mehmet Ekici
Kirikkale University, Faculty Of Medicine, Department Of Pulmonary Medicine, Kirikkale, Turkey

Swyer-James/MacLeod syndrome (SJMS) is a rare disease, characterized by unilateral hyperlucent lung due to hypoplasia of the pulmonary artery and bronchiectasis. Illnesses such as viral bronchiolitis and pneumonia in childhood are thought to be an acquired form. A 48-year-old woman applied to the hospital with symptoms including shortness of breath, sputum, and cough. Her medical history revealed that these symptoms had been relapsing since her childhood. She had been diagnosed and monitored for chronic bronchitis and bronchiectasis for the past 10 years. A physical examination revealed that the respiratory sounds of the lower left lobe decreased more than the right side, and revealed coarse crackles in the both middle, anterior, and inferior hemithorax. Chest radiography showed a loss of volume of the left lung, hiperlucency, and areas of bronchiectasis in the lower left lobe. Computed tomography showed that calibration of left pulmonary artery and its branches significantly decreased compared to the right side. We present our case simply because the majority of SJMS cases are diagnosed in childhood and young adulthood; however, our patient was examined several times with the same symptoms and not diagnosed until the age of 48. This syndrome should be considered in the differential diagnosis of patients who have hyperlucent lung.

Keywords: Bronchiectasis, Hyperlucent lung, Swyer-James /Macleod Syndrome.

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