Seltene Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis

Zusammenfassung

Seltene Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis stellen aufgrund der niedrigen Inzidenz und den daraus resultierend eingeschränkten diagnostischen Kriterien sowie therapeutischen Optionen eine Herausforderung dar. Der Schwellenwert, ab dem eine Erkrankung als selten zu werten ist, liegt bei höchstens 5 Betroffenen pro 10 000 Personen. Innerhalb dieser Erkrankungen gibt es allerdings extreme Schwankungen. So gehören einige seltene Erkrankungen, wie z.B. das invertierte Papillom, zu den regelmäßig diagnostizierten und therapierten Krankheitsbildern einer größeren Klinik für Hals-Nasen-Ohrenheilkunde. Andererseits liegen bei anderen seltenen Erkrankungen und Fehlbildungen weltweit deutlich unter 100 Fallberichte vor, was die Notwendigkeit einer Bündelung der verfügbaren Erfahrung zu Diagnostik und Therapie unterstreicht.

Die vorliegende Arbeit gibt eine Übersicht zu seltenen Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis aus den übergeordneten Bereichen Erkrankungen / Syndrome des olfaktorischen Systems, Fehlbildungen der Nase und Nasennebenhöhlen, Belüftungs- und Funktionsstörungen sowie benigne und maligne Tumore. Die Einordnung sowie Angaben zu Diagnostik und Therapiemöglichkeiten wurden unter Berücksichtigung der aktuellen Literatur erstellt.

Schlüsselwörter

Seltene Erkrankungen - Nase - Nasenennebenhöhlen - vordere Schädelbasis - Fehlbildungen

Publication History

Article published online:
30 April 2021

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