Abstract
Pulmonary agenesis is a rare congenital lung malformation. Although it is thought to occur sporadically, cases reported in the literature suggest the possibility of genetic transmission. The estimated incidence is 1 per 10000-15000 autopsies. Pulmonary agenesis may be unilateral or bilateral. The bilateral form is incompatible with extrauterin life. In 50% of cases, additional anomalies involving cardiovascular, gastrointestinal, genitourinary and musculoskeletal systems can be seen. The chest radiograph and computed tomography are the imaging modalities that should be choosen primarily. Prenatal diagnosis with prenatal diagnostic techniques such as fetal ultrasonography and fetal magnetic resonance imaging facilitates the postnatal diagnosis. In this report, the imaging findings of a newborn with right pulmonary agenesis are described and discussed in view of the existing literature.
Keywords
References
- Ünal S, Koç Esin.Pulmonary Agenesis, Pulmonary Hypoplasia.Turkiye Klinikleri J Pediatr Sci 2013;9(1):76-81
- Malcon MC, Malcon CM, Cavada MN, Caruso PE, Real LF. Unilateral pulmonary agenesis. J Bras Pneumol. 2012;38(4):526-529
- Dembinski J, Kroll M, Lewin M, Winkler P. Unilateral pulmonary agenesis,aplasia and dysplasia.Z Geburtshilfe Neonatol. 2009;213(2):56- 61.
- Boria MB, del Rio Camacho G, Orazco AL, Gil de San Vicente LP. A First Event of Dyspnea in an İnfant. Chest 2000;118 (4):1202-4
- Newman B. Congenital bronchopulmonary foregut malformations: concepts and controversies. Pediatr Radiol.2006;36(8):773-91
- Lee ML, Lue HC, Chiu IS, Chiu HY, Tsao LY, Yang AD. A systematic classification of the congenital bronchopulmonary vascular malformations: Dysmorphogenesis of the primitive foregut system and the primitive aortic arch system. Yonseı Med J.2008; 49(1):90-102
- Yetim TD, Bayaroğullari H, Yalçin HP, Arıca V, Arιca SG.Congenital Agenesis of the Left Lung: A rare case J Clin Imaging: 2011;1:47
- Nandan D, Bhatt GC, Dewan V, Pongener I. Complete right lung agenesis with dextrocardia. An unusual cause of respiratory distress. J Clin Imaging Sci. 2012;2:81
- Jentzsch NS. Unilateral pulmonary agensis. J Bras Pneumol. 2014;40(3): 322-4
- Saskın H, İdiz M, Düzyol C, Macika H, Aksoy R. Mitral valve replacement in an Adult with left pulmonary agenesis. Heart Surg Forum 2015;18(3):109-11
- Şirvancı M, Karaman K, Duran C, Onat L, Özer K. Unilateral Pulmoner Agenesi. Türk Göğüs Kalp Damar Cerrahisi Dergisi. 1999;7(6):486-488
- Canıtez Y, Çekiç Ş, Gürpınar A,Sapan N. Sağ Akciğer Agenezisi: İzole ve Eşlik Eden Anomalilerle Birlikte. Güncel Pediatri 2013;11:134-7
- Abrams ME, Ackerman VL, Engle WA. Primary unilateral pulmonary hypoplasia : neonate through early childhood-case report, radiographic diagnosis and review of the literature. J.Perinatol 2004;24(10):667-70.
- Pahuja A, Lee S.Persistent unilateral white-out in preterm. İnfant.J Pediatr Child Health 2013;49(1):87-89
Abstract
Pulmoner agenezi, nadir bir konjenital akciğer anomalisidir. Sporadik olarak geliştiği düşünülmesine rağmen literatürde yayınlanan ailesel vakalar, genetik geçiş ihtimalini de düşündürür. Tahmini sıklığı 10000-15000 otopsi incelemesinde birdir. Agenezi unilateral ya da bilateral olabilir. Bilateral formu yaşamla bağdaşmaz. Olguların %50’sinde; kardiyovasküler, gastrointestinal, genitoüriner ya da kas iskelet sistemini ilgilendiren ilave anomaliler de görülebilir. Tanıda öncelikle akciğer radyografisi ve bilgisayarlı tomografi BT seçilecek radyolojik görüntüleme yöntemleridir. Fetal ultrasonografi US ve fetal manyetik rezonans görüntüleme MRG teknikleri sayesinde prenatal tanı konulabilmesi, postnatal tanı koymayı kolaylaştırır. Bu yazıda intrauterin dönemde saptanan sağ pulmoner agenezi olgusunda postnatal radyolojik bulgular tanımlanmış ve literatür eşliğinde tartışılmıştır.
Keywords
References
- Ünal S, Koç Esin.Pulmonary Agenesis, Pulmonary Hypoplasia.Turkiye Klinikleri J Pediatr Sci 2013;9(1):76-81
- Malcon MC, Malcon CM, Cavada MN, Caruso PE, Real LF. Unilateral pulmonary agenesis. J Bras Pneumol. 2012;38(4):526-529
- Dembinski J, Kroll M, Lewin M, Winkler P. Unilateral pulmonary agenesis,aplasia and dysplasia.Z Geburtshilfe Neonatol. 2009;213(2):56- 61.
- Boria MB, del Rio Camacho G, Orazco AL, Gil de San Vicente LP. A First Event of Dyspnea in an İnfant. Chest 2000;118 (4):1202-4
- Newman B. Congenital bronchopulmonary foregut malformations: concepts and controversies. Pediatr Radiol.2006;36(8):773-91
- Lee ML, Lue HC, Chiu IS, Chiu HY, Tsao LY, Yang AD. A systematic classification of the congenital bronchopulmonary vascular malformations: Dysmorphogenesis of the primitive foregut system and the primitive aortic arch system. Yonseı Med J.2008; 49(1):90-102
- Yetim TD, Bayaroğullari H, Yalçin HP, Arıca V, Arιca SG.Congenital Agenesis of the Left Lung: A rare case J Clin Imaging: 2011;1:47
- Nandan D, Bhatt GC, Dewan V, Pongener I. Complete right lung agenesis with dextrocardia. An unusual cause of respiratory distress. J Clin Imaging Sci. 2012;2:81
- Jentzsch NS. Unilateral pulmonary agensis. J Bras Pneumol. 2014;40(3): 322-4
- Saskın H, İdiz M, Düzyol C, Macika H, Aksoy R. Mitral valve replacement in an Adult with left pulmonary agenesis. Heart Surg Forum 2015;18(3):109-11
- Şirvancı M, Karaman K, Duran C, Onat L, Özer K. Unilateral Pulmoner Agenesi. Türk Göğüs Kalp Damar Cerrahisi Dergisi. 1999;7(6):486-488
- Canıtez Y, Çekiç Ş, Gürpınar A,Sapan N. Sağ Akciğer Agenezisi: İzole ve Eşlik Eden Anomalilerle Birlikte. Güncel Pediatri 2013;11:134-7
- Abrams ME, Ackerman VL, Engle WA. Primary unilateral pulmonary hypoplasia : neonate through early childhood-case report, radiographic diagnosis and review of the literature. J.Perinatol 2004;24(10):667-70.
- Pahuja A, Lee S.Persistent unilateral white-out in preterm. İnfant.J Pediatr Child Health 2013;49(1):87-89
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | July 1, 2017 |
| Published in Issue | Year 2017 Volume: 14 Issue: 3 |