Yakup CANITEZ, Şükrü ÇEKİÇ, Arif GÜRPINAR, Nihat SAPAN

Sağ Akciğer Agenezisi; İzole ve Eşlik Eden Anomalilerle Birlikte

Sağ akciğer agenezisi; izole veya kalp, iskelet ve üriner sistem gibi sistemlere ait anomalilerle birlikte görülebilen nadir bir anomalidir. Olgu 1, Dört aylık bir kız çocuğu olup solunum sıkıntısı nedeniyle getirilmişti. Hastada sağ el başparmakta sindaktili ve polidaktili, sağda mandibuler hipoplazisi ile dismorfik düşük kulak vardı. Akciğer grafisi ve toraks bilgisayarlı tomografisi (BT) sağ pulmoner agenezi ile uyumluydu ve 7. torakal seviyede kelebek vertebra vardı. Toraks BT anjiografide; aort, sol pulmoner arter ve sağ atriyum basılarına bağlı sol ana bronşta ve özofagusda daralma saptandı. Bronkoskopide karina düzeyinde basıya bağlı darlık görüldü ve sağ ana bronş izlenmedi. Batın ultrasonografisinde sağ böbrek yerleşim anomalisi (pelvik ektopi) ve renal sintigrafide iki böbrek arasında füzyonla uyumlu görünüm vardı. Bu bulgularla hastada, sağ pulmoner ageneziye; ipsilateral radyal ray anomalisi, renal anomali, vertebral anomali ve hemifasyal mikrozominin eşlik ettiği tespit edildi. Olgu 2, On beş yaşında erkek çocuk olup, 2 aylıkken hışıltı atağı sonrası akciğer grafisi, toraks BT ve bronkoskopi ile pulmoner agenezi tanısı almıştı. Hastanın son bir yılda belirginleşen egzersizle çabuk yorulma dışında şikayeti yoktu. Fizik muayenesinde; açıklığı sağa bakan hafif skolyozu vardı, sağ hemitoraksta solunum sesleri azalmıştı, kalp sesleri sağda duyuluyordu. Akciğer grafisi ve toraks BT sağ akciğer agenezisi ile uyumluydu. Ekokardiyografide anomali yoktu, batın ultrasonografisi normaldi. Unilateral akciğer agenezilerinde; eşlik eden kardiyovasküler anomaliler, toraks içi yapılardaki distorsiyonlar ve tekrarlayan enfeksiyonlar, morbidite ve mortaliteyi etkileyen başlıca faktörlerdir. Burada izole ve multipl anomalilerle birlikte iki sağ akciğer agenezili olgu sunulmuştur. (Güncel Pediatri 2013; 11: 134-7)

Anomalilerle Birlikte Right Lung Agenesis; Isolated and with Accompanied Anomalies

SUMMARY Right lung agenesis is a rare anomaly that can be isolated or accompanied by system anomalies such as cardiac, skeletal or urinary systems. Case 1, a four-month-old girl, was brought because of respiratory distress. Patient had polydactyly, syndactyly of right thumb, right mandibular hypoplasia and low-set dysmorphic ears. Lung x-ray and thorax computerized tomography (CT) were consistent with right pulmonary agenesis and butterfly vertebra was evident in the 7th thoracic level. Thoracic CT angiography revealed narrowing of the left main bronchus and esophagus due to compression of aorta, left pulmonary artery and right atrium. In bronchoscopy, narrowing due to compression at the carina level was seen and right main bronchus was not seen. In abdominal ultrasonography, right kidney placement anomaly (pelvic ectopia) was present and renal scintigraphy revealed fusion in both kidneys. With these findings, it was found that right pulmonary agenesis was accompanied by ipsilateral radial ray anomaly, renal anomaly, vertebral anomaly and hemifacial microsomia. Case 2, a fifteen-year-old male patient, was diagnosed as pulmonary agenesis via chest x-ray, pulmonary CT and bronchoscopy after a wheezing episode when he was 2 months old. Patient had no complaint except for exhaustion that is aggravated by exercise during last year. In physical examination, he had a mild scoliosis toward right, respiratory sounds were diminished on right hemithorax and heart sounds were heard on right side. Chest x-ray and thoracic CT were consistent with right lung agenesis. No abnormality was found in echocardiography and abdominal ultrasonography was normal. Accompanied cardiovascular anomalies, distortions of intrathoracic structures and recurrent infections are main factors that affect mortality and morbidity. Here, two cases with right lung agenesis, isolated and accompanied by multiple anomalies, were presented. (Journal of Current Pediatrics 2013; 11: 134-7)

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