A rare disease; congenital pulmonary airway malformation in an adult

YILMAZ KARA, Bilge; ŞAHİN, Ünal; OZCELIK, Neslihan; ÖZYURT, Songül

doi:10.5578/tt.68095

A rare disease; congenital pulmonary airway malformation in an adult

Neslihan ÖZÇELİK, (Recep Tayyip Erdoğan Üniversitesi, Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Rize, Türkiye)

Songül ÖZYURT, (Recep Tayyip Erdoğan Üniversitesi, Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Rize, Türkiye)

Bilge YILMAZ KARA, (Recep Tayyip Erdoğan Üniversitesi, Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Rize, Türkiye)

Ünal ŞAHİN (Recep Tayyip Erdoğan Üniversitesi, Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Rize, Türkiye)

Tüberküloz ve Toraks

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Yıl: 2019 Cilt: 67 Sayı: 1 Sayfa Aralığı: 71 - 76 Metin Dili: İngilizce DOI: 10.5578/tt.68095 İndeks Tarihi: 29-07-2020

A rare disease; congenital pulmonary airway malformation in an adult

Öz:

Congenital pulmonary airway malformation (CPAM), is a rare disease knownas a developmental abnormality of the lower respiratory tract. It may occurin the neonatal period due to respiratory distress and patients usually die inthe first few months of life. Rarely, it may remain asymptomatic untiladulthood. In this study we describe an adult case and present a review ofthe literature. A 19-year-old male with no relevant medical history wasadmitted to our clinic with cough and wheeze. The patient had a history offrequent lower respiratory tract infection during childhood. Chest radiographrevealed a diffuse opacity and volume loss in the right hemithorax. Highresolution tomography showed hypoplasia of the right hemithorax, multiplecysts in all of the lobes and segments on the right side, ground glass opacityand interlobular septal thickening of the whole right lung parenchyma. Rightpneumonectomy was performed with the pre-diagnosis of congenitalpulmonary airway malformation and the pathological examination wascompatible with CPAM. CPAM is a rare disease in adulthood. We shouldconsider CPAM in the differential diagnosis of patients with frequentrecurrent pulmonary infection and cystic lung lesions. In order to preventinfections and to eliminate the risk of malignancy, surgical treatment shouldbe applied for definite diagnosis and treatment.

Anahtar Kelime:

Nadir bir hastalık; erişkin dönemde rastlanan konjenital pulmoner hava yolu malformasyonu

Öz:

Konjenital pulmoner hava yolu malformasyonu (KPHM), alt solunum yollarında gelişimsel bir anormallik olarak bilinen nadir bir hastalıktır. Yenidoğan döneminde solunum sıkıntısı nedeniyle ortaya çıkabilir ve hastalar genellikle yaşamın ilk birkaç ayında ölür. Nadiren yetişkinliğe kadar asemptomatik kalabilir. Bu çalışmada tanımlanan erişkin bir olgu eşliğinde literatürün gözden geçirilmesini sunuyoruz. Bilinen kronik hastalığı olmayan 19 yaşında erkek öksürük ve hışıltı ile kliniğimize başvurdu. Hastanın çocukluk döneminde sık alt solunum yolu infeksiyonu öyküsü vardı. Akciğer grafisinde sağ hemitoraksta difüz opasite ve hacim kaybı izlendi. Yüksek çözünürlüklü akciğer tomografisinde sağ hemitoraksta hipoplazi, sağda tüm loblarda ve segmentlerde çok sayıda kist, buzlu cam opasitesi ve sağ akciğer parankiminin tamamında interlobüler septal kalınlaşmalar görüldü. KPHM ön tanısı ile sağ pnömonektomi yapıldı ve patoloji sonucu KPHM ile uyumlu bulundu. KPHM erişkin yaşta nadir görülen bir hastalıktır. Sık sık tekrarlayan akciğer infeksiyonu ve kistik akciğer lezyonu olan hastaların ayırıcı tanısında KPHM’yi düşünmeliyiz. İnfeksiyonları önlemek ve malignite riskini ortadan kaldırmak, kesin tanı ve tedavi için cerrahi tedavi uygulanmalıdır.

Anahtar Kelime:

Belge Türü: Makale Makale Türü: Olgu Sunumu Erişim Türü: Erişime Açık

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APA	OZCELIK N, ÖZYURT S, YILMAZ KARA B, ŞAHİN Ü (2019). A rare disease; congenital pulmonary airway malformation in an adult. , 71 - 76. 10.5578/tt.68095
Chicago	OZCELIK Neslihan,ÖZYURT Songül,YILMAZ KARA Bilge,ŞAHİN Ünal A rare disease; congenital pulmonary airway malformation in an adult. (2019): 71 - 76. 10.5578/tt.68095
MLA	OZCELIK Neslihan,ÖZYURT Songül,YILMAZ KARA Bilge,ŞAHİN Ünal A rare disease; congenital pulmonary airway malformation in an adult. , 2019, ss.71 - 76. 10.5578/tt.68095
AMA	OZCELIK N,ÖZYURT S,YILMAZ KARA B,ŞAHİN Ü A rare disease; congenital pulmonary airway malformation in an adult. . 2019; 71 - 76. 10.5578/tt.68095
Vancouver	OZCELIK N,ÖZYURT S,YILMAZ KARA B,ŞAHİN Ü A rare disease; congenital pulmonary airway malformation in an adult. . 2019; 71 - 76. 10.5578/tt.68095
IEEE	OZCELIK N,ÖZYURT S,YILMAZ KARA B,ŞAHİN Ü "A rare disease; congenital pulmonary airway malformation in an adult." , ss.71 - 76, 2019. 10.5578/tt.68095
ISNAD	OZCELIK, Neslihan vd. "A rare disease; congenital pulmonary airway malformation in an adult". (2019), 71-76. https://doi.org/10.5578/tt.68095

APA	OZCELIK N, ÖZYURT S, YILMAZ KARA B, ŞAHİN Ü (2019). A rare disease; congenital pulmonary airway malformation in an adult. Tüberküloz ve Toraks, 67(1), 71 - 76. 10.5578/tt.68095
Chicago	OZCELIK Neslihan,ÖZYURT Songül,YILMAZ KARA Bilge,ŞAHİN Ünal A rare disease; congenital pulmonary airway malformation in an adult. Tüberküloz ve Toraks 67, no.1 (2019): 71 - 76. 10.5578/tt.68095
MLA	OZCELIK Neslihan,ÖZYURT Songül,YILMAZ KARA Bilge,ŞAHİN Ünal A rare disease; congenital pulmonary airway malformation in an adult. Tüberküloz ve Toraks, vol.67, no.1, 2019, ss.71 - 76. 10.5578/tt.68095
AMA	OZCELIK N,ÖZYURT S,YILMAZ KARA B,ŞAHİN Ü A rare disease; congenital pulmonary airway malformation in an adult. Tüberküloz ve Toraks. 2019; 67(1): 71 - 76. 10.5578/tt.68095
Vancouver	OZCELIK N,ÖZYURT S,YILMAZ KARA B,ŞAHİN Ü A rare disease; congenital pulmonary airway malformation in an adult. Tüberküloz ve Toraks. 2019; 67(1): 71 - 76. 10.5578/tt.68095
IEEE	OZCELIK N,ÖZYURT S,YILMAZ KARA B,ŞAHİN Ü "A rare disease; congenital pulmonary airway malformation in an adult." Tüberküloz ve Toraks, 67, ss.71 - 76, 2019. 10.5578/tt.68095
ISNAD	OZCELIK, Neslihan vd. "A rare disease; congenital pulmonary airway malformation in an adult". Tüberküloz ve Toraks 67/1 (2019), 71-76. https://doi.org/10.5578/tt.68095