Zusammenfassung
Einleitung: Das Peutz-Jeghers-Syndrom (PJS) ist charakterisiert durch eine Kombination aus mukokutanen Melaninpigmentierungen und hamartomatösen gastrointestinalen Polypen, die vorwiegend im Bereich des mittleren Dünndarms lokalisiert sind. Die Polypen können zur intestinalen Obstruktion sowie zu Invaginationen und Blutungen führen. Bis vor wenigen Jahren war die primär chirurgische Resektion sowie die intraoperative Polypektomie bei diesen Patienten die einzige Möglichkeit zur Therapie von Polypen im Bereich des mittleren Dünndarms. Die Push-and-Pull-Enteroskopie in Doppelballontechnik (DBE) eröffnet erstmals die Möglichkeit zur nichtchirurgischen Therapie. Methodik: Im Zeitraum von März 2003 bis September 2006 wurden insgesamt 16 Patienten mit PJS mittels DBE untersucht und endoskopisch therapiert. Dokumentiert wurden Größe, Anzahl und Lokalisation der diagnostizierten und endoskopisch resezierten Dünndarmpolypen sowie alle untersuchungs- und therapieassoziierten Komplikationen. Ergebnisse: Insgesamt wurden 47 DBE durchgeführt (oral 39, anal 8). Dabei fanden sich insgesamt 178 Polypen. 47 Polypen wurden aufgrund ihrer Größe und/oder ihres makroskopischen Erscheinungsbildes (Erosionen, Blutung, V. a. Malignität) endoskopisch polypektomiert. Die Größe der resezierten Dünndarmpolypen lag bei maximal 50 mm (Spanne 15 - 50 mm). Insgesamt traten 4 Komplikationen auf (2 Hb-relevante Blutungen, 1 Perforation, 1 Propofol-assoziierter Sättigungsabfall). Schlussfolgerung: Die DBE stellt ein sicheres Verfahren zur Diagnostik von Dünndarmpolypen bei Patienten mit PJS dar. Sie ermöglicht neben dem makroskopischen Eindruck eine Entnahme von Biopsien aus suspekten Arealen sowie eine genau Lokalisationsbestimmung und ggf. Markierung von primär operationswürdigen Befunden. Die DBE revolutioniert die therapeutischen Möglichkeiten für Polypen im Bereich des mittleren Dünndarms und minimiert die Indikation für ein primär chirurgisches Vorgehen.
Abstract
Introduction: Peutz-Jeghers syndrome (PJS) is characterised by a combination of hamartomatous gastrointestinal polyps and mucocutaneous melanin pigmentation. The polyps occur mainly in the small bowel and can lead to intestinal obstruction, intussusception and bleeding. Until only a few years ago, primary surgical resection and intraoperative endoscopy and polypectomy were the only available means of treating polyps in the mid-small bowel in these patients. With the introduction of double-balloon enteroscopy (DBE), we now have not only an improved diagnostic tool but also a non-surgical treatment option. Methods: Between March 2003 and September 2006 a total of 16 patients with PJS were examined by DBE and treated endoscopically. The size, number and location of the diagnosed and endoscopically resected small-bowel polyps were documented as were all complications associated with the examination or treatment. Results: A total of 47 DBE-procedures were performed (oral approach 39, anal approach 8). The examinations revealed a total of 178 polyps. 47 polyps were removed by endoscopic polypectomy on the grounds of their size and/or gross appearance. The largest small-bowel polyp resected was 50 mm (min. 15 mm, max. 50 mm). A total of four complications occurred (2 episodes of bleeding with a fall in Hb, 1 perforation, 1 propofol-associated decrease in oxygen saturation). Conclusion: DBE is a safe and reliable procedure for the diagnosis of small-bowel polyps in patients with PJS. In addition to macroscopic assessment and biopsy of suspicious areas, it permits the exact localisation as well as preoperative marking of polyps that are primary candidates for surgery. DBE revolutionises the therapeutic options for polyps in the region of the mid-small bowel and limits the indications for primary surgical management.
Schlüsselwörter
Peutz-Jeghers-Syndrom - Dünndarmpolypen - Push-and-Pull-Enteroskopie in Doppelballontechnik - endoskopische Polypektomie
Key words
Peutz-Jeghers syndrome - small bowel polyps - push-and-pull enteroscopy in double-balloon technique - endoscopic polypectomy
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Dr. Nicola Plum
Abteilung Innere Medizin II, HSK
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