Akciğerin konjenital kistik adenomatoid malformasyonu (prenatal tanı ve postnatal tedavi)

Amaç: Akciğerin konjenital kistik adenomatoid malformasyonu (KKAM) embriyonik farklılaşma anomalisi olarak normal akciğer dokusunun yerini değişik boyut ve sayıda kistlerin alması ile ortaya çıkan nadir bir hastalıktır. Prenatal ultrasonografi (USG) ile akciğerinde KKAM olduğu saptanan bir olgunun tanı ve tedavisini sunarak, bu nadir anomalinin tedavi prensiplerini tartışmayı amaçladık. Olgu sunumu: 20 yaşındaki 26 haftalık gebeye yapılan rutin prenatal USG’de dişi fetüsün sol toraksı içinde yerleşimli büyük kistik kitle (40x50x55 mm) tespit edildi. Akciğerin KKAM ön tanısı konularak, konservatif tedavi için fetüs takibe alındı ve 38 haftalık iken vajinal yoldan doğurtuldu. Bebek doğumdan sonraki günlerde sağlıklı olup solunum sıkıntısı yoktu. Fakat, hastaya doğumunun 4. gününde kan gazlarındaki ani bozulma ve solunum sıkıntısı çıkması üzerine acil torasik cerrahi girişim uygulandı. Sol torakotomi yapılarak, sol akciğer alt lobda yerleşik olan makrokistik kitle alt lobla birlikte rezeke edildi. Kistik kitlenin histopatolojik tetkiki ile Stocker’in Tip1 KKAM tanısı doğrulandı. Sonuç: Akciğerin KKAM, yenidoğanda solunum sıkıntısı ve mediastinal kaymaya neden olan konjenital kistik malformasyon olup ayırıcı tanıda akla getirilmelidir.

Anahtar Kelimeler:

Akciğer, Akciğerin kistik adenomatöz malformasyonu, doğumsal, Doğum öncesi tanı

Congenital cystic adenomatoid malformation of the lung (prenatal diagnosis and postnatal management)

Objective: Congenital cystic adenomatoid malformation (CCAM) of the lung which occurred with various forms and numbers of cysts as embryonic differentiation abnormality, is a rare abnormality, and often diagnosed prenatally by ultrasound. We aimed to discuss management principles of this rare abnormality by presenting management and diagnosis of a case which was prenatally diagnosed by ultrasound as CCAM of the lung. Case report: A 20-year-old woman was evaluated by routine prenatal ultrasound at 26 weeks of gestation and was found to have large cystic mass area measuring 40x50x55 mm in left fetal thorax. After prediagnosing as CCAM of the lung, the fetus was followed conservatively until full term, and was delivered vaginally at 38 weeks. The baby was noted as normal on days after birth, and no sign of respiratory distress was present. But, urgent thoracic surgical referral was performed when abrupt deterioration in blood gases and respiratory distress were present on the 4th day of life. Performing left thoracotomy, macrocystic mass located over the left lower lobe of the lung was dissected together with lower lobe. The diagnosis of Stocker’s type I CCAM was confirmed by histopathological examination of cystic mass. Conclusion: CCAM of the lung, which causes respiratory distress and mediastinal shift in newborn, is a congenital cystic malformation, and should be considered in the differential diagnosis of the lung masses.

Keywords:

Lung, Cystic Adenomatoid Malformation of Lung, Congenital, Prenatal Diagnosis,

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