Zusammenfassung
Die familiäre adenomatöse Polyposis coli (FAP) kann heute nicht mehr als einheitliche Krankheitsentität mit standardisiertem Therapiekonzept gesehen werden. Grundsätzlich ist jedoch die prophylaktische Kolektomie nach der phänotypischen Manifestation kolorektaler Polypen, aber vor der Entwicklung eines kolorektalen Karzinoms zu fordern. Die Festlegung des optimalen Operationszeitpunktes ist eine klinische Entscheidung, die unabhängig von der molekularen Diagnose getroffen wird. Bei Vorliegen einer klassischen FAP steht die Indikation zur restaurativen Proktokolektomie und ileoanalen Pouchanlage außer Frage. Dagegen ist für die attenuierten FAP-Varianten zusätzliche Evidenz aus klinischen Studien erforderlich, um die chirurgische Taktik und den Vorteil der prophylaktischen Chirurgie im Vergleich zum regelmäßigen endoskopischen Screening im Sinne einer Reduktion der Karzinomrate und der Mortalität zu belegen.
Abstract
Familial adenomatous polyposis coli (FAP) may not be considered a single disease entity with standardized guidelines for operative treatment. However, prophylactic colectomy after the manifestation of polyps but prior to the development of colorectal cancer is essential. The optimal timing of prophylactic surgery remains a clinical decision taken independently of mutation analysis. In case of the classic FAP phenotype, restorative proctocolectomy and ileal pouch-anal anastomosis is the procedure of choice. The development of reliable guidelines for attenuated FAP variants requires further evidence from clinical studies on surgical strategy and the advantages of prophylactic surgery over regular endoscopic screening with removal of polyps.
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Kadmon, M. Prophylaktische Chirurgie der familiären adenomatösen Polyposis coli. Chirurg 76, 1125–1134 (2005). https://doi.org/10.1007/s00104-005-1115-x
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DOI: https://doi.org/10.1007/s00104-005-1115-x
Schlüsselwörter
- Familiäre adenomatöse Polyposis coli
- Restaurative Proktokolektomie
- Ileoanale Pouchanlage
- Subtotale Kolektomie
- Klassische FAP
- Attenuierte FAP
- Genotyp-Phänotyp-Beziehungen