Abstract
Introduction
Prophylactic colon surgery has increased life expectancy of familial adenomatous polyposis patients. Extracolonic manifestations are life limiting, above all duodenal adenomas. Severe duodenal adenomatosis or cancer may necessitate pancreas-preserving total duodenectomy or partial pancreatico-duodenectomy, mostly after previous proctocolectomy and often after limited local resections of duodenal adenomas.
Scarce information on long-term postoperative outcome and quality of life after surgery for duodenal adenomatosis is available. Aim of the present study was to analyze perioperative and long-term outcome after PD and PPTD for FAP-associated duodenal adenomatosis, including QoL and recurrence of adenomas in the neoduodenum after PPTD.
Material, methods and patients
Thirty-eight patients, 27 after pancreas-preserving duodenectomy and 11 after partial pancreaticoduodenectomy, were included.
Results
Pancreas-preserving total duodenectomy was associated with shorter operation time and less blood loss than partial pancreatico-duodenectomy. Clinically relevant pancreatic fistula occurred in 31.5%. In-hospital mortality was 5.3%. Long-term follow-up revealed recurrent pancreatitis after pancreas-preserving total duodenectomy in 22% of patients, two (7.4%) required re-operation. Recurrent adenomatosis was detected in 26% of patients. Quality of life was comparable to the German normal population after both surgical procedures. Patients with postoperative complications showed worse results than those without complications. Disease-specific 10-year survival rate with respect to duodenal adenomatosis was 100%.
Conclusion
Surgery for FAP-associated duodenal adenomatosis and cancer can be carried out with reasonable morbidity rates despite previous proctocolectomy. Long-term outcome, quality of life, and survival rates are favorable.
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References
Groden J, Thliveris A, Samowitz W et al (1991) Identification and characterization of the familial adenomatous polyposis coli gene. Cell 66:589–600
Bodmer WF, Bailey CJ, Bodmer J et al (1987) Localization of the gene for familial adenomatous polyposis on chromosome 5. Nature 328:614–616
Bisgaard ML, Fenger K, Bülow S, Niebuhr E, Mohr J (1994) Familial adenomatous polyposis coli (FAP): frequency, penetrance, and mutation rate. Hum Mutat 3:121–125
Bülow S (2003) Results of national registration of familial adenomatous polyposis. Gut 52:742–746
Bülow S (1991) Diagnosis of familial adenomatous polyposis. World J Surg 15:41–46
Kadmon M (2005) Prophylactic surgery for patients with familial adenomatous polyposis coli. Chirurg 76:1125–1134
Aziz O, Athanasiou T, Fazio VW et al (2006) Meta-analysis of observational studies of ileorectal versus ileal pouch-anal anastomosis for familial adenomatous polyposis. Br J Surg 93:407–417
Bülow S, Björk J, Christensen IJ et al (2004) DAF Study Group. Duodenal adenomatosis in familial adenomatous polyposis. Gut 53:381–386
Bjoerk J, Åkerbrant H, Iselius L et al (2001) Periampullary adenomas and adenocarcinomas in familial adenomatous polyposis: cumulative risks and APC gene mutations. Gastroenterology 121:1127–1135
Church JM, McGannon E, Hull-Boiner S et al (1992) Gastroduodenal polyps in patients with familial adenomatous polyposis. Dis Colon Rectum 35:1170–1173
Kadmon M, Tandara A, Herfarth C (2001) Duodenal adenomatosis in familial adenomatous polyposis coli. A review of the literature and results from the Heidelberg polyposis register. Int J Color Dis 16:63–75
Spigelman AD, Williams CB, Talbot IC, Domzio P, Philips RKS (1989) Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet 2:783–785
Vasen HFA, Möslein G, Alonso A et al (2008) Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut 57:704–713
Köninger J, Friess H, Wagner M, Kadmon M, Büchler MW (2005) Die Technik der pankreaserhaltenden Duodenektomie. Chirurg 76:273–281
Müller MW, Dahmen R, Köninger J et al (2008) Is there an advantage in performing a pancreas-preserving total duodenectomy in duodenal adenomatosis? Am J Surg 195:741–748
Chung RS, Church JM, van Stolk R (1995) Pancreas-sparing duodenectomy: indications, surgical technique, and results. Surgery 117:254–259
Imamura M, Komoto I, Doi R, Onodera H, Kobayashi H, Kawai Y (2005) New pancreas-preserving total duodenectomy technique. World J Surg 29:203–207
Caillié F, Paye F, Desaint B et al (2012) Severe duodenal involvement in familial adenomatous polyposis treated by pylorus-preserving pancreaticoduodenectomy. Ann Surg Oncol 19:2924–2931
Rückert F, Distler M, Hoffmann S et al (2011) Quality of life in patients after pancreaticoduodenectomy for chronic pancreatitis. J Gastrointest Surg 15:1143–1150
Chan C, Franssen B, Domínguez I, Ramírez-Del A, Uscanga LF, Campuzano M (2012) Impact on quality of life after pancreatoduodenectomy: a prospective study comparing preoperative and postoperative scores. J Gastrointest Surg 16:1341–1346
Bassi C, Dervenis C, Butturini G et al (2005) Postoperative pancreatic fistula: an international study group (ISGPF) definition. Surgery 138(1):8–13
Bassi C, Marcheginai G, Dervenis C et al (2017) The 2016 update of the International Study Group (ISGPS) definition and grading of postoperative pancreatic fisutla: 11 years after. Surgery 161(3):584–591
Wente MN, Veit JA, Bassi C et al (2025) Postpancreatectomy hemorrhage (PPH)—an international study group of pancreatic surgery (ISGPS) definition. Surgery 142(1):2007
Wente MN, Bassi C, Dervenis C et al (2007) Delayed gastric emptying (DGE) after pancreatic surgery: a suggested definition by the International Study Group of Pancreatic Surgery (ISGPS). Surgery 142(5):762–768
Ellert U, Ballach BM (1999) The SF-36 questionnaire in the federal health survey—description. Gesundheitswesen 61:184–190
Kurth BM, Ellert U (2002) The SF-36 questionnaire and its usefulness in population studies: results of the German health interview and examination survey 1998. Soz Praventivmed 47:266–277
Ware JE, Sherbourne CD (1992) The MOS 36-item Short Form Health Survey (SF-36): I. Conceptual framework and item selection. Med Care 30:473–483
Bullinger M (1995) German translation and psychometric testing of the SF-36 health survey: preliminary results from the IQOLA project. Soc Sci Med 41:1359–1366
Al-Sarireh B, Ghaneh P, Gardner-Thorpe J et al (2008) Complications and follow-up after pancreas-preserving total duodenectomy for duodenal polyps. Br J Surg 95:1506–1511
De Castro AMM, van Eijck HJ, Rutten JP et al (2008) Pancreas-preserving total duodenectomy versus standard pancreatoduodenectomy for patients with familial adenomatous polyposis and polyps in the duodenum. Br J Surg 95:1380–1386
Penninga L, Svendson LB (2011) Pancreas-preserving total duodenectomy: a 10-year experience. J Hepatobiliary Pancreat Sci 18:717–723
Lepistö A, Kiviluoto T, Halttunen J, Järvinen HJ (2009) Surveillance and treatment for duodenal adenomatosis in familial adenomatous polyposis. Endoscopy 41:504–509
Wente MN, Shrikande SV, Müller MW et al (2007) Pancreaticojejunostomy versus pancreaticogastrostomy. Systematic review and meta-analysis. Am J Surg 193(2):171–183
O’Leary DP, Fide CJ, Foy C et al (2001) Quality of life after low anterior resection with total mesorectal excision and temporary loop ileostomy for rectal carcinoma. Br J Surg 88:1216–1220
Austin KKS, Young JM, Solomon MJ (2010) Quality of life of survivors after pelvic exenteration for rectal cancer. Dis Colon Rectum 53:1121–1126
Cense HA, Visser MRM, van Sandick JW et al (2004) Quality of life after colon interposition by necessity for esophageal cancer replacement. J Surg Ocnol 88:32–38
Korolija K, Sauerland S, Wood-Dauphinée S et al (2004) Evaluation of quality of life after laparoscopic surgery. Surg Endosc 18:879–897
Smadder NJ, Neklason DW, Boucher KM et al (2016) Effect of sulindac and erlotinib vs placebo on duodenal neoplasia in familial adenomatous polyposis. A randomized clinical trial. JAMA 315(12):1266–1275
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All procedures performed in this study involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
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Ganschow, P., Hackert, T., Biegler, M. et al. Postoperative outcome and quality of life after surgery for FAP-associated duodenal adenomatosis. Langenbecks Arch Surg 403, 93–102 (2018). https://doi.org/10.1007/s00423-017-1625-2
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DOI: https://doi.org/10.1007/s00423-017-1625-2