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Chirurgische Eingriffe bei Patienten mit systemischer Mastzellaktivierungserkrankung

Empfehlungen für das perioperative Management

Surgical interventions in patients with systemic mast cell activation disease

Recommendations for perioperative management

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Zusammenfassung

Hintergrund

Die systemische Mastzellaktivierungsserkrankung (MCAD; Prävalenz 5–10 %) ist eine multifaktorielle, polygene Erkrankung mit multisystemischer Symptomatik, die durch eine unregulierte erhöhte Freisetzung von Mastzellmediatoren und eine Anreicherung aktivierter Mastzellen in potenziell allen Organen und Geweben gekennzeichnet ist. Aufgrund der weiten Verbreitung der Erkrankung wird der operativ/anästhesiologisch/interventionell tätige Arzt häufig unwissentlich mit mastzellerkrankten Patienten konfrontiert, bei denen prä-, intra- und postoperativ unerwartete Komplikationen auftreten können, wenn keine mastzellspezifischen Behandlungspraktiken angewandt wurden.

Ziel der Arbeit

Die in diesem Beitrag thematisierten Erkenntnisse aus Literaturrecherche, Konsensusempfehlungen der verschiedenen internationalen Expertengruppen sowie umfangreichen eigenen Erfahrungen in der Behandlung von MCAD-Patienten ermöglichen einen empirischen und evidenzbasierten Umgang mit der MCAD in Zusammenhang mit invasiven Eingriffen.

Ergebnisse und Diskussion

Aufgrund der hohen Prävalenz der MCAD in der Bevölkerung ist davon auszugehen, dass Patienten mit MCAD im chirurgischen Patientenkollektiv entsprechend häufig vertreten sind. Werden beim Vorliegen und Verdacht auf das Vorliegen einer Mastzellerkrankung MCAD-spezifische Besonderheiten im anästhesiologischen und operativen Vorgehen berücksichtigt, sind MCAD-Patienten nicht als Risikopatienten einzustufen.

Abstract

Background

Systemic mast cell activation disease (MCAD, prevalence 5–10%) is a multifactorial, polygenic disease with multisystemic symptoms that is characterized by an unregulated increased release of mast cell mediators and an accumulation of activated mast cells potentially in all organs and tissues. Due to the high prevalence of the disease, physicians involved in surgical, anesthesiological and interventional procedures are often unknowingly faced with MCAD patients experiencing unexpected preoperative, intraoperative and postoperative complications, if no mast cell-specific treatment regimens have been applied.

Objective

The findings from a literature search, consensus recommendations of the various international expert groups and extensive own experience in the treatment of MCAD patients enable an empirical and evidence-based care of MCAD patients in association with invasive procedures.

Results and conclusion

Due to the high prevalence of MCAD in the population, it can be assumed that patients with MCAD are correspondingly frequently represented in the surgical patient collective. When MCAD-specific peculiarities are preventively considered in the anesthesiological and surgical procedures in patients with proven or suspected mast cell disease, MCAD patients should not be classified as being at risk.

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Authors and Affiliations

  1. Abteilung für Allgemein- und Viszeralchirurgie, Haus St. Elisabeth, Gemeinschaftskrankenhaus Bonn, Bonn, Deutschland

    B. Sido

  2. Abteilung für Innere Medizin, Haus St. Elisabeth, Gemeinschaftskrankenhaus Bonn, Bonn, Deutschland

    J. Homann

  3. Institut für Experimentelle Hämatologie & Transfusionsmedizin, Universitätsklinikum Bonn, Bonn, Deutschland

    H.-J. Hertfelder

  4. Institut für Pathologie Bonn-Duisdorf, Bonn, Deutschland

    T. Zienkiewicz

  5. Anästhesie, Intensiv- und Schmerzmedizin, Gemeinschaftskrankenhaus Bonn, Bonn, Deutschland

    K.-P. Christians & P. Schablin

  6. Zentrum für Seltene Erkrankungen Bonn (ZSEB), Universitätsklinikum Bonn, Bonn, Deutschland

    M. Mücke

  7. Institut für Humangenetik, Universitätsklinikum Bonn, Sigmund-Freud-Str. 25, 53127, Bonn, Deutschland

    G. J. Molderings

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  1. B. Sido
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Correspondence to G. J. Molderings.

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Interessenkonflikt

B. Sido, J. Homann, H.-J. Hertfelder, T. Zienkiewicz, K.-P. Christians, P. Schablin, M. Mücke und G.J. Molderings geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Sido, B., Homann, J., Hertfelder, HJ. et al. Chirurgische Eingriffe bei Patienten mit systemischer Mastzellaktivierungserkrankung. Chirurg 90, 548–556 (2019). https://doi.org/10.1007/s00104-019-0935-z

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