Angeborene Fehlbildungen der Lunge – eine Übersicht

Zusammenfassung

Kongenitale pulmonale Malformationen stellen eine heterogene Gruppe seltener Erkrankungen dar, die auf Fehlentwicklungen während der embryonalen und fetalen Wachstumsphase basieren. Zu ihnen gehören der Trachealbronchus, die bronchiale Atresie, die bronchogene Zyste, die Lungensequestration, das kongenitale lobäre Emphysem sowie die sogenannte Congenital pulmonary Airway Malformation. Eines der Leitsymptome dieser Malformationen ist die durch ihren verdrängenden Effekt bedingte postnatale respiratorische Insuffizienz, welche eine rasche operative Versorgung erfordert. Auch bei asymptomatischen Malformationen wird aufgrund des erhöhten Infektrisikos die Resektion empfohlen.

In der folgenden Übersicht wird auf die Ursachen, das klinische Bild und die therapeutischen Optionen dieser angeborenen Fehlbildungen der Lunge und des Bronchialsystems eingegangen.

Abstract

Congenital pulmonary malformations comprise a heterogenous group of rare developmental diseases. The most common malformations are the tracheal bronchus, bronchial atresia, bronchogenic cyst, pulmonary sequestration, congenital lobar emphysema, and congenital pulmonary airway malformation. Due to their space-consuming effect, patients suffer early postnatal respiratory distress which generally requires immediate surgical resection. The management of asymptomatic lesions remains subject to debate, but early elective surgery is generally recommended to avoid respiratory and infectious complications at a later time point.

We here provide a comprehensive review in which we present causes, clinical presentation and therapeutic options for the most prominent congenital malformations of the airways and lung parenchyma.

Schlüsselwörter

Bronchialatresie - Bronchogene Zyste - Lungensequester - Kongenitales Lobäremphysem - Congenital Pulmonary Airway Malformation

Keywords

bronchial atresia - bronchogenic cyst - pulmonary sequestration - congenital lobar emphysema - congenital pulmonary airway malformation

• Literatur

• 1 Alamo L, Gudinchet F, Reinberg O. et al. Prenatal diagnosis of congenital lung malformations. Pediatr Radiol 2012; 42: 273-283
• 2 Swarr DT, Peranteau WH, Pogoriler J. et al. Novel molecular and phenotypic insights into congenital lung malformations. Am J Respir Crit Care Med 2018; 197: 1328-1339
• 3 Mullassery D, Smith NP. Lung development. Semin Pediatr Surg 2015; 24: 152-155
• 4 Ghaye B, Szapiro D, Fanchamps JM. et al. Congenital bronchial abnormalities revisited. Radiographics 2001; 21: 105-119
• 5 Setty SP, Michaels AJ. Tracheal bronchus: Case presentation, literature review, and discussion. J Trauma 2000; 49: 943-945
• 6 McLaughlin FJ, Strieder DJ, Harris GBC. et al. Tracheal bronchus: Association with respiratory morbidity in childhood. J Pediatr 1985; 106: 751-755
• 7 Wooten C, Patel S, Cassidy L. et al. Variations of the tracheobronchial tree: Anatomical and clinical significance. Clin Anat 2014; 27: 1223-1233
• 8 Moreno M, Castillo-Corullón S, Pérez-Ruiz E. et al. Spanish multicentre study on morbidity and pathogenicity of tracheal bronchus in children. Pediatr Pulmonol 2019; 54: 1610-1616
• 9 Gipson M, Cummings K, Hurth K. Bronchial atresia. Radiographics 2009; 29: 1531-1535
• 10 Wang Y, Dai W, Sun Y. et al. Congenital bronchial atresia: Diagnosis and treatment. Int J Med Sci 2012; 9: 207-212
• 11 Cappeliez S, Lenoir S, Validire P. et al. Totally endoscopic lobectomy and segmentectomy for congenital bronchial atresia. Eur J Cardiothorac Surg 2009; 36: 222-224
• 12 Fowler DJ, Gould SJ. The pathology of congenital lung lesions. Semin Pediatr Surg 2015; 24: 176-182
• 13 Kirmani B, Kirmani B, Sogliani F. Should asymptomatic bronchogenic cysts in adults be treated conservatively or with surgery?. Interact Cardiovasc Thorac Surg 2010; 11: 649-659
• 14 Lesaffer J, Heremans B, de Leyn P. et al. Video-assisted mediastinoscopic resection of a large symptomatic bronchogenic cyst. Interact Cardiovasc Thorac Surg 2011; 12: 1071-1073
• 15 Aravena C, Patel J, Goyal A. et al. Role of endobronchial ultrasound-guided transbronchial needle aspiration in the diagnosis and management of mediastinal cyst. J Bronchology Interv Pulmonol 2020; 27: 142-146
• 16 Corbett HJ, Humphrey GME. Pulmonary sequestration. Paediatr Respir Rev 2004; 5: 59-68
• 17 Mendeloff EN. Sequestrations, congenital cystic adenomatoid malformations, and congenital lobar emphysema. Semin Thorac Cardiovasc Surg 2004; 16: 209-214
• 18 Correia-Pinto J, Gonzaga S, Huang Y. et al. Congenital lung lesions-underlying molecular mechanisms. Semin Pediatr Surg 2010; 19: 171-179
• 19 Kim HM, Hwang JH, Kim MJ. et al. Postnatally diagnosed coexisting congenital diaphragmatic hernia with pulmonary sequestration: A report of two cases. Obstet Gynecol Sci 2020; 63: 529-533
• 20 Sun X, Xiao Y. Pulmonary sequestration in adult patients: A retrospective study. Eur J Cardiothorac Surg 2015; 48: 279-282
• 21 Trabalza Marinucci B, Maurizi G, Vanni C. et al. Surgical treatment of pulmonary sequestration in adults and children: Long-term results. Interact Cardiovasc Thorac Surg 2020; 31: 71-77
• 22 Agarwal R, Sinha D. Endovascular Embolization of Intrapulmonary Sequestration. J Invasive Cardiol 2020; 32: E264
• 23 Liu HS, Li SQ, Qin YZ. et al. Surgical treatment of intralobar pulmonary sequestration. Chin Med Sci J 2010; 25: 53-56
• 24 Munnell ER, Lambird PA, Austin RL. Polyalveolar Lobe Causing Lobar Emphysema of Infancy. Ann Thorac Surg 1973; 16: 624-628
• 25 Demir OF, Hangul M, Kose M. Congenital lobar emphysema: Diagnosis and treatment options. Int J Chron Obstruct Pulmon Dis 2019; 14: 921-928
• 26 Powers JE, Counselman FL. Congenital lobar emphysema: Tube thoracostomy not the treatment. Pediatr Emerg Care 2005; 21: 760-762
• 27 Laberge JM, Puligandla P, Flageole H. Asymptomatic congenital lung malformations. Semin Pediatr Surg 2005; 14: 16-33
• 28 Gupta R, Singhal SK, Rattan KN. et al. Management of congenital lobar emphysema with endobronchial intubation and controlled ventilation. Anesth Analg 1998; 86: 71-73
• 29 Krivchenya DU, Rudenko EO, Dubrovin AG. Congenital emphysema in children: Segmental lung resection as an alternative to lobectomy. J Pediatr Surg 2013; 48: 309-314
• 30 Perea L, Blinman T, Piccione J. et al. Bilateral congenital lobar emphysema: staged management. J Pediatr Surg 2017; 52: 1442-1445
• 31 Bawazir O. Congenital lobar emphysema: Thoracotomy versus minimally invasive surgery. Ann Thorac Med 2020; 15: 21-25
• 32 Wong KKY, Flake AW, Tibboel D. et al. Congenital pulmonary airway malformation: advances and controversies. Lancet Child Adolesc Health 2018; 2: 290-297
• 33 Leblanc C, Baron M, Desselas E. et al. Congenital pulmonary airway malformations: state-of-the-art review for pediatrician’s use. Eur J Pediatr 2017; 176: 1559-1571
• 34 Seear M, Townsend J, Hoepker A. et al. A review of congenital lung malformations with a simplified classification system for clinical and research use. Pediatr Surg Int 2017; 33: 657-664
• 35 Lau CT, Kan A, Shek N. et al. Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program. Pediatr Surg Int 2017; 33: 105-108
• 36 Stocker JT. Cystic lung disease in infants and children. Fetal Pediatr Pathol 2009; 28: 155-184
• 37 Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung – Classification and Morphologic Spectrum. Hum Pathol 1977; 8: 155-171
• 38 Adzick NS, Harrison MR, Glick PL. et al. Fetal cystic adenomatoid malformation: Prenatal diagnosis and natural history. J Pediatr Surg 1985; 20: 483-488
• 39 David M, Lamas-Pinheiro R, Henriques-Coelho T. Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation. Neonatology 2016; 110: 101-115
• 40 Eber E. Antenatal diagnosis of congenital thoracic malformations: Early surgery, late surgery, or no surgery?. Semin Respir Crit Care Med 2007; 28: 355-366
• 41 Stanton M. The argument for a non-operative approach to asymptomatic lung lesions. Semin Pediatr Surg 2015; 24: 183-186
• 42 Jelin EB, O’Hare EM, Jancelewicz T. et al. Optimal timing for elective resection of asymptomatic congenital pulmonary airway malformations. J Pediatr Surg 2018; 53: 1001-1005
• 43 Rothenberg SS, Shipman K, Kay S. et al. Thoracoscopic segmentectomy for congenital and acquired pulmonary disease: A case for lung-sparing surgery. J Laparoendosc Adv Surg Tech A 2014; 24: 50-54
• 44 Nasr A, Bass J. Thoracoscopic vs open resection of congenital lung lesions: A meta-analysis. J Pediatr Surg 2012; 47: 857-861