Nutzen und Wertigkeit der Adeno-Tonsillektomie bei Kindern und Jugendlichen mit PFAPA-Syndrom

Zusammenfasung

Hintergrund

Das PFAPA-Syndrom kennzeichnet sich durch das Auftreten von periodischem Fieber, aphthöser Stomatitis, Pharyngitis und zervikaler Lymphadenopathie. Die Tonsillektomie (TE) bzw. Adenotonsillektomie (ATE) stellt eine operative Therapieoption dieses periodischen Fiebersyndroms dar, deren Stellenwert wir diskutieren.

Material und Methoden

Im Zeitraum von 2004 bis 2010 erfassten wir die Daten von 36 Patienten mit der Diagnose PFAPA-Syndrom. Die Auswertung erfolgte anhand von struktuierten Erhebungsbögen, Krankenakten sowie einer systematischen Aufarbeitung der internationalen Literatur bis April 2011.

Ergebnisse

Die PFAPA-Episoden begannen durchschnittlich im Alter von 22 Monaten und traten bei einer durchschnittlichen Dauer von 3–5 Tagen etwa alle 14–33 Tage auf. Im Fieberschub kam es bei 70% der Patienten zu oralen Aphthen, bei 93% zu einer Pharyngitis und bei 96% zu einer zervikalen Lymphadenopathie. Bei 4 Patienten lag eine positive Familienanamnese bezüglich rezidivierender Fieberschübe vor. Als einzige medikamentöse Therapieoption bewirkten Kortikosteroide bei 85% eine anhaltende Entfieberung bis zum nächsten Schub. Die TE/ATE (n=16) führte bei 10 Patienten zu einem vollständigen Sistieren der Fieberschübe, bei 2 Patienten zu einer Verminderung der Schubfrequenz, 3 Patienten profitierten nicht von der TE/ATE und ein Kind verstarb infolge einer Nachblutung. Bei 8 Patienten zeigte sich eine spontane Remission nach einer durchschnittlichen Krankheitsdauer von 4 Jahren.

Schlussfolgerungen

Die TE stellt eine effektive Methode in der Behandlung des PFAPA-Syndroms dar. Entgegen einer pauschalisierten Indikationsstellung sollte immer individuell unter Abwägung des Nutzens gegenüber dem Operationsrisiko mit HNO-ärztlichen Beratung entschieden werden.

Abstract

Benefits and Importance of Tonsillectomy in Children and Youth with PFAPA Syndrome

Background

PFAPA syndrome is characterized by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Tonsillectomy and adenotonsillectomy are surgical treatment options for this periodic fever syndrome, the significance of which will be discussed.

Material and Methods

Between 2004 and 2010 we collected data of 36 patients with the diagnosis of PFAPA syndrome. Data analysis was carried out on the basis of structured questionnairs, patients’ files as well as a systematic evaluation of international literature up to April 2011.

Results

The average age for the appearance of PFAPA episodes was 22 months and they recurred for an average duration of 3–5 days every 14–33 days. During a PFAPA attack, aphthous stomatitis was present in 70% of the patients, pharyngitis was present in 93% and cervical adenitis in 96%. The family history for recurrent fever was positive in 4 of the patients. In 85% cortikosteroids were the only effective medicative treatment with no further symptoms until the next attack. Surgery (tonsillectomy±adenoidectomy) aborted the PFAPA episodes in 10 of 16 patients, in 2 patients the frequency of episodes decreased, 3 patients had no noticeable change and 1 patient died as a result of postsurgical bleeding at another institution. After an average duration of illness of 4 years the PFAPA syndrome was in spontaneous remission in 8 patients.

Conclusions

Tonsillectomy is an effective treatment option for the PFAPA syndrome. Contrary to a general indication the decision should be personalized considering the benefit vs. the risk of operation with the advice of an ENT specialist.

Schlüsselwörter

PFAPA-Syndrom - periodisches Fieber - Lymphadenitis colli - Pharyngitis - Tonsillitis - Tonsillektomie

Key words

pharyngitis - stomatitis/aphthous - tonsillitis/diagnosis - PFAPA syndrome - lymphatic diseases - fever

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