Bronşektazili bir hastada gecikmiş tanı: Swyer-James / Macleod sendromu
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2007
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info:eu-repo/semantics/openAccess
Özet
Swyer-James/MacLeod Sendromu pulmoner arter hipoplazisine bağlı unilateral hiperlüsent akciğer görünümü ve etkilenen tarafta bronşektazi ile karakterize nadir görülen bir hastalıktır. 57 yaşında sigara içmeyen bayan hasta, öksürük, bol balgam çıkarma ve ateş yakınmalarıyla başvurdu. Öyküsünden bu şikayetlerinin çocukluk yaşlarından beri tekrarladığı, hastanemiz dışında, 6-7 yıl öncesine kadar kronik bronşit, son 6-7 yıldır da bronşektazi tanısıyla takip ve tedavi edildiği öğrenildi. Fizik incelemede; solunum seslerinin şiddeti sol orta ve alt zonda sağa göre hafif azalmış, bu alanda kaba raileri mevcuttu. PA akciğer grafisinde; sol hiperlüsent akciğer görünümü, sol akciğerde hacim kaybı ve sol alt zonda bronşektazik alanlar izlendi. Toraksın bilgisayarlı tomografisi (BT) ve yüksek rezolusyonlu BTde; sol pulmoner arterde hipoplazi, sol pulmoner arter dallarının çapında azalma, hiperlüsensi, sol akciğer üst lob posterior ve linguler segment ile alt lobda kistik bronşektazi saptandı. Renkli doppler ekokardiyografide; sol pulmoner arter hipoplazik ve ana pulmoner arter hafif genişlemiş olarak izlendi. Swyer- James/MacLeod Sendromu olguların çoğunda çocukluk ve genç erişkin dönemde teşhis edilen bir hastalık olmasına karşın, olgumuzda 57 yaşına kadar şikayetleri nedeniyle çeşitli defalar tetkik edilmesine rağmen tanımlanamadığı ve hiperlüsent akciğer görünümü saptandığında bu sendromun ayırıcı tanıda düşünülmesi gerektiğini vurgulamak için sunuyoruz.
Swyer-James/MacLeod Syndrome is a rare disease which is characterized by unilateal hyperlucent lung due to hypoplasia of its pulmonary artery. A non-smoker, 57 years old female patient admitted to our clinic with cough, sputum production and fever. Her medical history revealed that these symptoms has been recurrent since her childhood. Furthermore she had been followed up with as a patient of chronic bronchitis or bronchiectasis outside of our clinic so far. Physical examination revealed diminished respiratory sounds and coarse crackles on middle and lower zones of left hemithorax. Chest X Ray showed hyperluceny and volume loss of the left lung and bronchiectasis at the left lower zone. Computerized and High Resolution Computerized Tomography revelaed hypoplasia of left pulmonary artery with reduced diameters of its branches, hyperlucent left lung and cyctic bronchiectasis in left lower, and superior and lingular segments of the left upper lobes .Colored Doppler Echocardiography showed hypoplasia of the left pulmonary artery and enlargement of the main pulmonary artery. Swyer-James/MacLeod Syndrome is diagnosed in childhood or early adulthood of most of the cases. We present this case to emphasize the delayed diagnosis until'the age of 57 although she had been investigatedseveral times because of her recurrent symptoms since her childhood and to emphasize considering this syndrome especially when a hyperlucent lung was detected on chest X ray.
Swyer-James/MacLeod Syndrome is a rare disease which is characterized by unilateal hyperlucent lung due to hypoplasia of its pulmonary artery. A non-smoker, 57 years old female patient admitted to our clinic with cough, sputum production and fever. Her medical history revealed that these symptoms has been recurrent since her childhood. Furthermore she had been followed up with as a patient of chronic bronchitis or bronchiectasis outside of our clinic so far. Physical examination revealed diminished respiratory sounds and coarse crackles on middle and lower zones of left hemithorax. Chest X Ray showed hyperluceny and volume loss of the left lung and bronchiectasis at the left lower zone. Computerized and High Resolution Computerized Tomography revelaed hypoplasia of left pulmonary artery with reduced diameters of its branches, hyperlucent left lung and cyctic bronchiectasis in left lower, and superior and lingular segments of the left upper lobes .Colored Doppler Echocardiography showed hypoplasia of the left pulmonary artery and enlargement of the main pulmonary artery. Swyer-James/MacLeod Syndrome is diagnosed in childhood or early adulthood of most of the cases. We present this case to emphasize the delayed diagnosis until'the age of 57 although she had been investigatedseveral times because of her recurrent symptoms since her childhood and to emphasize considering this syndrome especially when a hyperlucent lung was detected on chest X ray.
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