Congenital lobar emphysema

Abstract

Ozet Abstract Bir ya da birden fazla akciger lobunun disaridan bir basi olmadan hiperinflasyonu konjenital lober amfizem olarak tanimlanir. Etyolojisi henuz tam olarak anlasilamamasina ragmen, bir cok yazar bronsiyal anormallikler ya da alveolar defektlerin sonucu olarak olustugunu bildirir. Bazi teoriler yetersiz kikirdak desteginin sonucunda bronsial kollapsi da icerir. Siklikla sol ust lob, ardindan orta ve sag ust loblar, etkilenir, alt loblar ise nadiren etkilenir. En sik klinik prezentasyonu, lokal hava hapsi nedeni ile ipsilateral ya da kontrlateral normal akcigere basi sonrasi yenidoganda akut solunum sikintisi yapmasidir. Konjenital lober amfizem lobektomi ile basarili olarak tedavi edilir. Cocuklarda bir den fazla lob alinsa bile, muhtemelen kompansatuar alveolar buyumenin etkisiyle prognoz iyidir. Bu olgu sunumunda nispeten gec tani alan 3 yasinda bir kiz hasta sunuldu. Congenital lobar emphysema is defined by hyperinflation of one or more lung lobes in the absence of extrinsic bronchial obstruction. Although the etiology has yet to be fully understood, many authors have reported it as the result of bronchial abnormalities or alveolar defects. Some theories include bronchial collapse resulting from inadequate cartilaginous support. The left upper lobe is usually the most frequently affected, followed by the middle and right upper lobes, the lower lobes being rarely affected. The most common clinical presentation is neonatal acute respiratory distress, which is caused by localized air trapping that compresses the ipsilateral and contralateral normal lungs. Congenital lobar emphysema was treated successfully by lobectomy. The prognosis is good even when more than one lobe is resected and probably reflects the potential for compensatory alveolar growth in children. In this case report, 3 years old girl patient who diagnosed relatively late is presented.