Roboterassistierte Proktokolektomie mit ileopouchanaler Anastomose bei Verdacht auf hereditäre Polyposis

Zusammenfassung

Zielsetzung In der Literatur werden verschiedene Formen der hereditären Polyposis beschrieben. Die klassische familiäre adenomatöse Polyposis (FAP) ist eine seltene, autosomal-dominant vererbte Erkrankung, die durch eine Keimbahnmutation im Adenomatous-Polyposis-coli-Gen (APC) verursacht wird und sich klinisch durch einen ausgedehnten Befall des Kolons mit multiplen Polypen charakterisiert. Unbehandelt führt die Erkrankung beinahe unweigerlich zur malignen Entartung.

Indikation Wir präsentieren den Fall eines 37-jährigen Patienten mit histologisch gesichertem, stenosierendem Adenokarzinom des Colon descendens sowie initialem Verdacht auf eine hereditäre Polyposis bei multiplen Polypen im Colon descendens und sigmoideum.

Methoden Das Video erläutert die präoperativen bildgebenden sowie endoskopischen Befunde und zeigt die Technik der zweizeitigen, roboterassistierten Proktokolektomie mit ileopouchanaler Anastomose (IPAA) und Anlage eines protektiven Loop-Ileostomas.

Schlussfolgerung Hinsichtlich der chirurgischen Therapie der klassischen FAP kann die restaurative Proktokolektomie (RPC) mit Anlage eines Ileum-J-Pouches – trotz Kontroversen hinsichtlich verschiedener technischer Aspekte – als etabliertes Standardverfahren angesehen werden. Dabei sind die minimalinvasiven Strategien gegenüber den konventionellen Techniken als zumindest gleichwertig zu betrachten.

Abstract

Background Various forms of hereditary polyposis have been described in the literature. Classical familial adenomatous polyposis (FAP) is a rare, autosomal dominantly inherited disease which is caused by a germline mutation in the adenomatous polyposis coli gene (APC). Patients with this diagnosis successively develop multiple polyps of the colon. Left untreated, FAP almost inevitably leads to malignant transformation.

Indication We present the case of a 37-year-old patient with histologically confirmed, stenotic adenocarcinoma of the descending colon and an initially suspected hereditary polyposis due to multiple polyps in the descending and sigmoid colon.

Methods The video describes the preoperative imaging as well as endoscopic findings and demonstrates the technique of a two-stage, robotically assisted proctocolectomy with ileal pouch-anal anastomosis (IPAA) and the creation of a temporary loop ileostomy.

Conclusions With respect to the surgical treatment of classic FAP, restorative proctocolectomy (RPC) with ileal J-pouch construction can be regarded as an established standard procedure, despite controversy regarding various technical aspects. Minimally invasive strategies should be considered as an equivalent option compared to conventional techniques.

Schlüsselwörter

hereditäre Polyposis - familiäre adenomatöse Polyposis (FAP) - Kolonkarzinom - kolorektales Karzinom - roboterassistierte Proktokolektomie - ileopouchanale Anastomose (IPAA)

Key words

hereditary polyposis - familial adenomatous polyposis (FAP) - colon cancer - colorectal carcinoma - robotically assisted proctocolectomy - ileal pouch-anal anastomosis (IPAA)

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