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DOI: 10.1055/s-0038-1629051
Angeborene Fehlbildungen der Trachea und der Lunge
Congenital malformations of the trachea and lungPublication History
Eingereicht am:
18 May 2009
angenommen am:
03 June 2009
Publication Date:
25 January 2018 (online)
Zusammenfassung
Angeborene Fehlbildungen der Trachea und der Lunge sind seltene Erkrankungen teilweise unklarer Ätiologie. Sie zeigen ein breites Spektrum an Symptomen mit unterschiedlicher Ausprägung und unterschiedlichem Manifestationsalter. Einige Fehlbildungen werden bereits intrauterin klinisch relevant und bedürfen einer Therapie. Anhaltspunkte sind vor allem Stridor und rezidivierende Infekte, häufig ist die Anamnese jedoch leer. Entsprechend vielseitig sind die diagnostischen und therapeutischen Möglichkeiten. Die Mehrzahl aller Fehlbildungen ist nur mit apparativen Methoden endgültig zu diagnostizieren. Bei allen Fehlbildungen der Lunge und des Tracheobronchialbaums ist früher oder später mit Komplikationen zu rechnen. Entscheidend ist die frühzeitige, präventive Erkennung dieser Fehlbildungen. Ein entscheidendes Argument für die frühzeitige operative Korrektur ist das Wachstumspotenzial der reifenden Lunge mit Wachstum und Neubildung von Alveolen bis etwa zum 8. Lebensjahr. Die komplexe pneumologische Abklärung sollte in pädiatrischen Zentren erfolgen, bestehend aus einem interdisziplinären Team aus Pulmologen, Kardiologen, HNO-Ärzten, Anästhesisten, Radiologen und Kinderchirurgen.
Summary
Malformations of the trachea and lung are rare; the ethiology in most cases is unknown. Depending on the kind of malformation they show a broad spectrum of symptoms and a varying age at manifestation. Some malformations are diagnosed prenatally and need intrauterine interventions. A history of stridor and recurrent pulmonary infections are important hints, but often these children have no medical history at first diagnosis. Thus there are many diagnostic and therapeutic options. Most malformations need technical appliances for the definitive diagnosis. One has to calculate that sooner or later most malformations of the trachea and lung develop complications. Decisive is the early diagnosis and treatment of these malformations. A strong argument for the early surgical treatment is the fact that the growth potential of the maturating lung persists until the 8th year of age. Thus, in case of lung resection, the lung does not only regenerate by distension, but also by growing and alveoli formation. The complexity of these cases should lead to early submission of these children to paediatric centres consisting trained paediatric pulmonologists and cardiologists, ENT, anaesthesiologists, radiologists and paediatric surgeons.
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