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Laryngorhinootologie 2024; 103(01): 25-34
DOI: 10.1055/a-2160-2777
Übersicht

Klinisches Management der Choanalatresie

Clinical Management of Choanal Atresia
Alexander Philippe Maas 
1   Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universitätsklinikum Bonn, Bonn, Germany (Ringgold ID: RIN39062)
,
Sebastian Strieth
1   Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universitätsklinikum Bonn, Bonn, Germany (Ringgold ID: RIN39062)
,
Thorsten Send 
1   Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universitätsklinikum Bonn, Bonn, Germany (Ringgold ID: RIN39062)
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Zusammenfassung

Die Choanalatresie (CA) ist eine seltene kongenitale Anomalie der nasalen Atemwege mit einer Inzidenz von 1/5000 bis 1/9000, die unilateral (uCA) oder bilateral (bCA) auftreten kann. Die bCA manifestiert sich als akuter Notfall unmittelbar nach der Entbindung durch akute Atemwegsobstruktion und paradoxe Zyanose, während die uCA neben der einseitigen Nasenatmungsbehinderung ein klinisch heterogenes Beschwerdebild aufweisen kann. Die fiberendoskopische Untersuchung ist in Verbindung mit der kranialen Computertomographie der Goldstandard in der diagnostischen Abklärung der CA. Die CA tritt häufig in Assoziation zu angeborenen Fehlbildungssyndromen auf, unter denen das CHARGE-Syndrom hervorzuheben ist. Aufgrund von kardiopulmonaler Instabilität und erschwerten Intubationsbedingungen sind syndromale CA-PatientInnen als eigene Risikogruppe aufzufassen. Nach Sicherung des Atemweges muss die bCA zeitnah operativ versorgt werden, während die Korrektur der uCA erst nach dem sechsten Lebensmonat erfolgen sollte. Die endoskopischen Operationstechniken sind der chirurgische Standard in der Versorgung der CA. Hierbei lassen sich unterschiedliche Herangehensweisen differenzieren: die transnasale Punktion der Atresieplatte mit anschließender Erweiterung nach medial und lateral, die Anlage eines Septumfensters mit anschließender posteriorer Vomer- und Atresieplattenresektion und die Anlage mukoperiostealer Lappen mit anschließender Eröffnung der Atresieplatte. Der transpalatinale Zugangsweg sollte nur noch in anatomisch komplexen Ausnahmefällen gewählt werden. Der Einsatz konventioneller choanaler Stents in der primären Versorgung der CA wird zunehmend zurückgewiesen und sollte Risikokonstellationen vorbehalten bleiben. Ebenso sollte auf die lokale Applikation von Mitomycin C verzichtet werden.

Abstract

Choanal atresia (CA) is a rare congenital anomaly of the nasal airway with an incidence of 1/5000 to 1/9000, which may occur unilateral (uCA) or bilateral (bCA). bCA manifests as an acute emergency immediately after birth by airway obstruction and paradoxical cyanosis, whereas uCA may present with a heterogeneous clinical picture in addition to unilateral nasal airway obstruction. Fiber endoscopic examination and cranial computed tomography are the gold standard in the diagnosis of CA. CA often occurs in association with congenital malformation syndromes, among which CHARGE syndrome stands out. Due to cardiopulmonary instability and difficult intubation conditions, syndromic CA patients should be considered as a separate risk group. After securing the airway, bCA must be treated surgically without delay, whereas correction of uCA should not be performed until after six months of age. Endoscopic techniques are the surgical standard in the treatment of CA. Different approaches can be distinguished: transnasal puncture of the atresia plate with subsequent extension medially and laterally, creation of a septal window with subsequent resection of the posterior vomer and atresia plate, and elevation of mucoperiosteal flaps with subsequent opening of the atresia plate. The transpalatal approach should only be employed in anatomically complex cases. The use of conventional choanal stents in the primary treatment of CA is increasingly rejected and should be reserved for high-risk constellations. Similarly, local application of mitomycin C should be avoided.

Schlüsselwörter

unilaterale Choanalatresie - bilaterale Choanalatresie - CHARGE-Syndrom - Atemwegsmanagement - choanales Stenting - Endoskopisches Operieren - mukoperiosteale Lappen

Keywords

Choanal atresia - Endoscopic Surgery - Mucoperiosteal flap - Airway management - CHARGE syndrome


Publication History

Received: 26 June 2023

Accepted: 16 August 2023

Article published online:
19 September 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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