Endoscopic techniques for surveillance and treatment of FAP
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1 Endoscopic techniques for surveillance and treatment of FAP Evelien Dekker MD PhD Department of Gastroenterology & Hepatology Academic Medical Center Amsterdam The Netherlands
2 FAP: endoscopic surveillance & treatment Colon Duodenum Stomach Small bowel
3 Questions for GE in FAP: Who do we surveil? What age to start Frequency of surveillance Method of surveillance When to intervene Which treatment-options
4 Colon: who do we surveil? Presymptomatic: Persons with familiar risk APC-mutation carriers If no mutation is detected in proband: all 1 st degree family members Symptomatic..
5 Symptomatic 52 yr old man
6 Colon: Age, frequency & method Classic FAP: from 12 yrs 2-yearly sigmoidoscopy Attenuated FAP (AFAP): from 18 yrs 2-yearly colonoscopy
7 Colon: Method Careful non-magnified endoscopy All general quality measures must be taken into account: adequate bowel prep, withdrawal time etc Role for advanced imaging?
8 Chromoendoscopy Contrast stains Indigo-carmine (0,1 0,5%) Absorptive stains Methylene blue (0,1%)
9 Chromoendoscopy Targetted Directly through the workingchannel Segment or full colon With use of a spraycatheter
10 Chromoendoscopy in patient with AFAP
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13 Colon: Method Use chromo (targeted or through a spraycatheter) At any discolouration If there is suspicion on genetic or familial basis
14 Colon: when to intervene? No guidelines.. If burden of polyps is severe and cancer cannot be ruled out
15 Colon: when to intervene? Classic FAP: Try to wait until adulthood Attenuated FAP: Possible to manage endoscopically??
16 Which treatment-options? Surgical Subtotal colectomy with ileorectal anastomosis Proctocolectomy with ileoanal pouch-reconstruction with ileostomy
17 Which operation?? Subtotal colectomy Best functional outcome However, chance of need for secondary proctectomy Related to genotype, phenotype & age at surgery Sinha et al, Br J Surg 2010
18 AMC: Proctocolectomy with ileoanal pouchreconstruction, stapeled Final operation Can be performed laparoscopically Functional outcome is good
19 Risks of pouch: 212 pats in Netherlands, 7.9 yrs follow-up Risk of adenomas 45% Risk of advanced pathology 11.8% Risk of cancer 1.9%, 10-year cumulative risk 75.5% However, due to retrospective nature no exact location of lesions possible.. Friederich et al, Clin Gastro Hep 2008
20 Patient 52 yr
21 And: Risk of adenoma formation Pouch In (small) anorectal segment Risk is larger if anastomosis is stapeled and not handsewn (St Marks Polyposis registry, 206 pats with pouch, 10.3 yrs follow-up) Von Roon et al, Ann Surg 2011
22 Patient 64 yr
23 Patient 36 yr
24 Patient 31 yr
25 Patient 20 years
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29 Patient 40 years
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41 Now in AMC: All patients undergo colonoscopy before colonic surgery If polyps close to anorectal verge: first polypectomies If in doubt: surgical mucosectomy and inlay with ileal mucosa
42 Surveillance after surgical resection: Ileorectal anastomosis: 6 monthly sigmoidoscopy (yearly??) Ileoanal pouch-reconstruction: Yearly pouch-scopy with gastroscope (NB minute inspection of anorectal segment, also in retroflex!) Ileostomy: No data on endoscopy of stoma
43 Duodenum
44 Questions for duodenum in FAP: Who do we surveil? Method of surveillance Frequency of surveillance When to intervene Which treatment-options
45 Duodenal polyposis in FAP In past: patients died from CRC Genetic testing & awareness of risk surveillance and colectomy better survival Most important causes of death in FAP: desmoïds and duodenal carcinoma
46 Duodenal polyposis Prevalence 60% Life-time cumulative risk 100% Björk et al Gastro 01 Groves et al Gut 02 Bülow et al Gut 04
47 Duodenal polyposis Prevalence 60% Life-time cumulative risk 100% Estimated cumulative risk duodenal carcinoma 4-10% Björk et al Gastro 01 Groves et al Gut 02 Bülow et al Gut 04
48 Duodenal polyposis in FAP How to assess who is at risk for duodenal carcinoma??
49 Spigelman classification # points # Polyps >20 Size (mm) >10 Type adenoma Degree of dysplasia tubulair tubulovillous villous low-grade high-grade Spigelman et al, Lancet 1989
50 Spigelman classification Stage 0: 0 points Stage I: 1-4 points Stage II: 5-6 points Stage III: 7-8 points Stadium IV: 9-12 points
51 Who is most at risk? Correlation between Spigelman stage and chance of getting duodenal cancer highest risk in patients with stage IV Groves Gut 02: 36% vs 2.4% Sp III Bülow Gut 04: 7% vs 0.7% Sp 0-III
52 Duodenum: method, age & frequency Gastroduodenoscopy with assessment of Spigelman stage Start at yrs Interval depending on Spigelman-stage (5 yearly 6 monthly)
53 Duodenum: method Use of advanced imaging techniques? To detect polyps To assess margins To differentiate to target biopsies
54 Duodenum: chromo
55 Duodenum: chromo
56 Study on chromo in duodenum in 45 pats: Mean Before indigo After indigo p # polyps Size mm Dekker at al, Endoscopy 2009
57 Chromoendoscopy per patient: More duodenal polyps detected in 13 pats Maximum size of the polyps increased in 7 pats Total number of points for the Spigelmanclassification was increased in 8 pats However, this resulted in an increased Spigelman-classification in only 5 pats (11%) Dekker at al, Endoscopy 2009
58 Spigelman classifications: chromoendoscopy Spigelman Before indigo After indigo O 3 (7%) 2 (4%) I 2 (4%) 2 (4%) II 11 (24%) 10 (22%) III 16 (36%) 17 (35%) IV 13 (29%) 14 (31%)
59 Chromoendoscopy duodenal FAP: Extra work No proven additonal benefit Not routinely Dekker at al, Endoscopy 2009
60 NBI in duodenal FAP? No studies published, 1 case-report Prospective study ongoing in AMC (with Spanish help) Yamao et al, Med Sci Monit 2009
61 TVA with LGD
62 TVA with LGD
63 TVA with HGD
64 Duodenum: When to intervene?? Correlation between Spigelman stage and chance of getting duodenal cancer highest risk in patients with stage IV However many patients are nowadays in stage IV
65 Netherlands 45 FAP patients (22, median age 47 yrs) Forward and sideward viewing gastroscopy
66 Spigelman stages Spigelman This study Historical Bülow Groves 0 3 (7%) 34% - 2% I 2 (4%) 15% - 13% II 11 (24%) 27% - 39% III 16 (36%) 17% - 36% IV 13 (29%) 7% - 10% Dekker at al, Endoscopy 2009
67 Reasons for many stage IV? Age of patients? Better quality of endoscopy?
68 Also: 44% had papillary adenoma
69 In whom to intervene? Assess during gastroscopy Spigelman-stage (so count, measure and take biopsies) PLUS papilla separately
70 In whom to intervene? Growing papillary adenoma: papillectomy Multiple adenomas with HGD or lage polyp-burden and one with HGD: surgical resection
71 Surgical options: Sparing: Transduodenal polypexcision Partial duodenectomy Definitive, high morbidity & mortality Pancreaticoduodenectomy ( Whipple ) Pancreassparing duodenectomy
72 In whom to intervene? Growing papillary adenoma: papillectomy Multiple adenomas with HGD or lage polyp-burden and one with HGD: surgical resection Relatively low polyp-burden and one adenoma with HGD: EMR??
73 Endoscopic ampullectomie Retrospective analysis 59 FAP-pats: Multipele sessions only 34% resulted in normal histology (NB is that required??) Norton et al 2002
74 St Marks All FAP-pats with duodenal polyps >1 cm NO papillary adenoma Endoscopic polypectomy +/- APC Latchford et al, InSight 2007
75 Interim analysis 46 pats 85 therapeutic procedures Median follow-up 24 mo (3-111) 14 downstaged, 3 upstaged, 13 stable, 16 await staging 10 bleedings (4 transfusions), no perforations or deaths Latchford et al, InSight 2007
76 AMC Patients with FAP en duodenal polyps with HGD òr papillary adenoma increasing in size Endoscopic treatment of only those polyps: EMR, APC, ampullectomy etc (propofol, 1 night cinical observation) Re-endoscopy after 3 months If necessary re-treatment
77 Endoscopic ampullectomy Growing TVA with LGD
78 Duodenal TVA met HGD
79 Duodenal TVA with HGD
80 After surgical resection: Also surveillance of jejunum, especially area of neopapilla..
81 Stomach: fundic gland polyps
82 Stomach: fundic gland polyps Benign Literature: one doubtful cancer.. Practice: inspect and biopsy if abnormality
83 Stomach: antral adenomas
84 Stomach: antral adenomas Incidence increased?? Chance of malignant transformation? Practice: inspect antrum and biopsy any abnormalities If proven by histopathology: polypectomy??
85 Patient 40 years
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88 Jejenum & ileum
89 Surveillance jejunum & ileum in FAP? Almost all patients have polyps Several case reports.. However, what is true incidence of cancer?? Ruys et al, Clin Gastro Hep 2010
90 Surveillance jejunum & ileum in FAP? Capsule endoscopy SBE DBE
91 Surveillance jejunum & ileum in FAP? No proven benefit Exceptions: Before duodenal resection After duodenal resection Spigelman IV??
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99 Conclusions FAP-patients deserve close endoscopic surveillance and timely surgical (medical??) intervention No evidence-based guidelines for both diagnosis and treatment Uniformly collected, prospective data are difficult to gather but needed!
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