Burnun Konjenital Malformasyonları
Özet
Burnun konjenital malformasyonları, belirgin deformitelerden fark edilmesi güç anomalilere kadar değişen, nadir görülen durumlardır. Bu anomaliler arasında en sık karşılaşılanı, nazal koanaların tıkanmasıyla karakterize olan ve yenidoğanlarda döngüsel siyanoza yol açabilen koanal atrezidir. Diğer önemli orta hat lezyonları arasında kafa tabanındaki defektlerden nöral doku herniasyonu olan nazal ensefaloseller, heterotopik nöral dokulardan köken alan iyi huylu nazal gliomlar ve en sık görülen orta hat anomalisi olan nazal dermoid kistler yer alır. Ayrıca dış burun ve koku duyusunun tamamen yokluğu ile karakterize arini, çift burun yapısı oluşturan polirini, aksesuar burun delikleri, nazal kleftler, tübüler eklentilerle seyreden probozis lateralis ve kraniyofasiyal yarık sendromu olan frontonazal displazi gibi ekstrem anomaliler de mevcuttur. Solunum yollarını doğrudan etkileyen nazolakrimal duktus kistleri, priform apertür stenozu ve çok nadir görülen epignatus gibi teratomlar da klinik tablolar arasında bulunur. Bu malformasyonların tanısında bilgisayarlı tomografi (BT) ve manyetik rezonans görüntüleme (MRG) kritik öneme sahipken, tedavileri genellikle hava yolu güvenliğinin sağlanmasını takiben transnazal endoskopik veya açık cerrahi yaklaşımlarla total eksizyon ve rekonstrüksiyonu içerir.
Congenital malformations of the nose are rare anomalies ranging from distinct deformities to subtle defects. Choanal atresia, characterized by the obstruction of nasal choanae, is the most common congenital nasal anomaly and presents as a neonatal emergency due to cyclic cyanosis. Major midline lesions include nasal encephaloceles, which involve the herniation of neural tissues through skull base defects; nasal gliomas, which are benign heterotopic neural tissue masses; and nasal dermoids, representing the most frequent midline congenital lesions. Extremely rare structural deformities encompass arhinia (complete absence of the external nose and olfactory sense), polyrhinia (double nose), supernumerary nostrils, nasal clefts, proboscis lateralis (tubular appendages), and frontonasal dysplasia. Additionally, conditions like nasolacrimal duct cysts, pyriform aperture stenosis, and nasopharyngeal teratomas (epignathus) can severely impair the neonatal airway. Computed tomography (CT) and magnetic resonance imaging (MRI) are crucial for definitive diagnosis and structural evaluation. While mild cases are managed conservatively, definitive treatment typically requires early airway stabilization followed by precise surgical interventions, including transnasal endoscopic or open rhinoplasty approaches for total excision and reconstruction.
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