[Surgical aspects of indications and techniques for adenomatous polyposis variants]

Chirurg. 2016 Aug;87(8):709-22. doi: 10.1007/s00104-016-0217-y.
[Article in German]

Abstract

Due to the advances in molecular genetic diagnostics of adenomatous polyposis variants, identification of patients with a genetic predisposition and their at risk relatives is becoming increasingly important in clinical practice. Precise knowledge of the specific risk profile is gaining significance especially for surgeons and requires a clinically differentiated approach in order to correctly identify the indications for prophylactic surgery. In this article reference will be made to the technical details of the pouch operation rather than the decision-making process per se, since this has become common knowledge for specialized colorectal surgeons. Besides the more commonly known polyposis syndromes, such as familial adenomatous polyposis (FAP), surgeons should nowadays at least be able to clinically distinguish between attenuated and classical variants of FAP, be aware of MUTYH-associated polyposis (MAP) and also the new polyposis syndrome polymerase proofreading-associated polyposis (PPAP). Surgeons should be familiar with the specific indications and extent of surgery for prophylactic organ removal in the lower gastrointestinal tract in order to be able to competently advise patients.

Keywords: Familial adenomatous polyposis; Ileoanal pouch; MYH-associated polyposis; Operative procedures; Peutz-Jeghers syndrome.

Publication types

  • Review

MeSH terms

  • Adenomatous Polyposis Coli / classification
  • Adenomatous Polyposis Coli / genetics*
  • Adenomatous Polyposis Coli / surgery*
  • DNA Glycosylases / genetics
  • DNA Mutational Analysis
  • DNA Polymerase I / genetics
  • DNA Polymerase II / genetics
  • Humans
  • Peutz-Jeghers Syndrome / classification
  • Peutz-Jeghers Syndrome / genetics
  • Peutz-Jeghers Syndrome / surgery

Substances

  • DNA Polymerase I
  • DNA Polymerase II
  • DNA Glycosylases
  • mutY adenine glycosylase