Journal of the Royal Society of Medicine Volume 85 February 1992
77
Rectal polyposis as a guide to duodenal polyposis in familial adenomatous polyposis
A D Spigelman FRACS1'2
C B Williams FRCP'
R K S Phillips MS",2
'St Mark's Hospital,
City Road, London EC1 and 2Professorial Surgical Unit, St Bartholomew's Hospital, West Smithfield, London EC1 Keywords: polyposis syndrome, familial; duodenal neoplasms; rectal neoplasms
Summary Almost all patients with familial adenomatous polyposis (FAP) develop duodenal polyps, the severity of which is graded stage 1 (minor) to stage V (cancer). Regular endoscopy is recommended for all patients with FAP. To test whether the development of severe duodenal polyposis could be predicted in another way, rectal and duodenal polyp severity were compared in 91 patients with FAP. The fulguration ratio (number ofrectal fulgurations divided by number of years since colectomy) supplied the rectal polyp severity index. Patients with stage V- duodenal polyposis had significantly higher fulguration ratios (median 0.38) than did patients with stage 1 disease (median 0; P=0.009). However, the wide scatter of results means that rectal polyp severity cannot be used as a guide to duodenal polyp severity in individual patients. The coexistence of populations with severe duodenal and rectal polyposis suggests that environmental factors are important in phenotypic expression in FAP. Introduction The realization that patients with polyposis are at high risk for developing periampullary duodenal cancer' and the finding that most patients with polyposis have dysplastic duodenal polyps (adenomas)2 led to the initiation of upper gastrointestinal screening endoscopy for all FAP patients. These examinations are conducted at intervals varying from 6 months to 3 years. If the severity of rectal polyposis paralleled that of duodenal polyposis, then regular examination of the rectum might allow foregut endoscopy to be done less frequently. One index of the severity of rectal polyps is the number of admissions for fulguration to polyps in the rectal stump (defined as the 10-12 cm of large bowel distal to the sacral promontory)3. Despite differences between the 15 consultant surgeons (1948-1989) at St Mark's Hospital in their management of rectal polyposis after ileorectal anastomosis, the performance of fulguration is a broad indicator of a troublesome rectal stump. The aim of this study was to see whether patients with a history of severe rectal polyposis (as judged by the fulguration ratio) were more likely to develop severe duodenal polyposis.
colectomy and ileorectal anastomosis, who were under regular follow-up at St Mark's Hospital and who had undergone screening upper gastrointestinal endoscopy or who had had duodenal cancer. Of 102 patients who underwent screening gastroduodenoscopy, 81 were evaluable (48 males, 33 females; mean age 42 years, range 20-62). Staging of duodenal polyposis was carried out as previously described (stages I to IV)3. The number of rectal stump fulgurations was divided by the length of time in years since colectomy and ileorectal anastomosis for each patient, to obtain the number of fulgurations per year (the fulguration ratio). These fulguration ratios were then allocated to the stage of duodenal polyposis found to be present for each individual and ranked in order of ascendancy. There were 10 other patients (seven males, three females) who had had duodenal cancer and the fulguration ratios of this group were compared to the others. If the adenoma-carcinoma sequence exists in the foregut, then cancer would occur after stage IV duodenal polyposis. Duodenal cancer is therefore referred to as stage V duodenal polyposis from now on. Because the length of follow-up was not the same for all patients, fulguration ratios were calculated for those patients who had been under follow-up for the period between 1978 and 1989. Ofthe 81 patients who had undergone foregut endoscopy as part of the screening programme, two (one each from the groups with stage III and stage IV duodenal polyposis) had undergone rectal excision prior to 1978, leaving 79 evaluable patients. Of the 10 patients with stage V duodenal polyposis, two had died and one was being followed-up elsewhere by 1978, leaving seven evaluable patients. 1.2 *
1
x
0.8 R a 0
4
0.6 0.4 -+ 0-4
Ot %°
0.2
e
80
X
*
*0*
xx
x
x
Iz
-
x
t
x
x xx
2
Patients and methods We measured the number of rectal fulgurations performed on patients with FAP who had undergone Correspondence to: R K S Phillips, St Mark's Hospital, City Road, London EC1
+
0
;QQQMQ
o
S ***-I** Duodenal Polyposis Stage x Iv X V + 11 * III *11111
Figure 1. Duodenal polyposis and number of fulgurations! year post ileorectal anastomosis. Median values for each polyposis stage is shown with a horizontal bar
0141-0768/92/ 020077-03/$02.00/0 © 1992 The Royal Society of Medicine
78
Journal of the Royal Society of Medicine Volume 85 February 1992
Table 1. Duodenal polyposis and fulgurations/year post ileorectal anastomosis
Stage of duodenal polyposis
Patients (n) Years post ileorectal anastomosis (range) Mean age (years) Sex: male female Fulguration ratio: median (interquartile range)
nII
III
IV
V*
17 179 (1-27) 39.1
28 315 (1-34) 37.9
27 423 (2-38) 44.1
9 193 (5-40) 49.6
10 256
(11-36) 53.7
13 4
14 14
17 10
4 5
7 3
0 (0-0.25)
0.13 (0-0.27)
0.23 (0.04-0.5)
0.38 (0.2-0.44)
0.38 (0.13-0.61)
*Foregut cancer patients Fulguration ratio/stage of duodenal polyposis: stage I compared with: stage IV, P
77
Rectal polyposis as a guide to duodenal polyposis in familial adenomatous polyposis
A D Spigelman FRACS1'2
C B Williams FRCP'
R K S Phillips MS",2
'St Mark's Hospital,
City Road, London EC1 and 2Professorial Surgical Unit, St Bartholomew's Hospital, West Smithfield, London EC1 Keywords: polyposis syndrome, familial; duodenal neoplasms; rectal neoplasms
Summary Almost all patients with familial adenomatous polyposis (FAP) develop duodenal polyps, the severity of which is graded stage 1 (minor) to stage V (cancer). Regular endoscopy is recommended for all patients with FAP. To test whether the development of severe duodenal polyposis could be predicted in another way, rectal and duodenal polyp severity were compared in 91 patients with FAP. The fulguration ratio (number ofrectal fulgurations divided by number of years since colectomy) supplied the rectal polyp severity index. Patients with stage V- duodenal polyposis had significantly higher fulguration ratios (median 0.38) than did patients with stage 1 disease (median 0; P=0.009). However, the wide scatter of results means that rectal polyp severity cannot be used as a guide to duodenal polyp severity in individual patients. The coexistence of populations with severe duodenal and rectal polyposis suggests that environmental factors are important in phenotypic expression in FAP. Introduction The realization that patients with polyposis are at high risk for developing periampullary duodenal cancer' and the finding that most patients with polyposis have dysplastic duodenal polyps (adenomas)2 led to the initiation of upper gastrointestinal screening endoscopy for all FAP patients. These examinations are conducted at intervals varying from 6 months to 3 years. If the severity of rectal polyposis paralleled that of duodenal polyposis, then regular examination of the rectum might allow foregut endoscopy to be done less frequently. One index of the severity of rectal polyps is the number of admissions for fulguration to polyps in the rectal stump (defined as the 10-12 cm of large bowel distal to the sacral promontory)3. Despite differences between the 15 consultant surgeons (1948-1989) at St Mark's Hospital in their management of rectal polyposis after ileorectal anastomosis, the performance of fulguration is a broad indicator of a troublesome rectal stump. The aim of this study was to see whether patients with a history of severe rectal polyposis (as judged by the fulguration ratio) were more likely to develop severe duodenal polyposis.
colectomy and ileorectal anastomosis, who were under regular follow-up at St Mark's Hospital and who had undergone screening upper gastrointestinal endoscopy or who had had duodenal cancer. Of 102 patients who underwent screening gastroduodenoscopy, 81 were evaluable (48 males, 33 females; mean age 42 years, range 20-62). Staging of duodenal polyposis was carried out as previously described (stages I to IV)3. The number of rectal stump fulgurations was divided by the length of time in years since colectomy and ileorectal anastomosis for each patient, to obtain the number of fulgurations per year (the fulguration ratio). These fulguration ratios were then allocated to the stage of duodenal polyposis found to be present for each individual and ranked in order of ascendancy. There were 10 other patients (seven males, three females) who had had duodenal cancer and the fulguration ratios of this group were compared to the others. If the adenoma-carcinoma sequence exists in the foregut, then cancer would occur after stage IV duodenal polyposis. Duodenal cancer is therefore referred to as stage V duodenal polyposis from now on. Because the length of follow-up was not the same for all patients, fulguration ratios were calculated for those patients who had been under follow-up for the period between 1978 and 1989. Ofthe 81 patients who had undergone foregut endoscopy as part of the screening programme, two (one each from the groups with stage III and stage IV duodenal polyposis) had undergone rectal excision prior to 1978, leaving 79 evaluable patients. Of the 10 patients with stage V duodenal polyposis, two had died and one was being followed-up elsewhere by 1978, leaving seven evaluable patients. 1.2 *
1
x
0.8 R a 0
4
0.6 0.4 -+ 0-4
Ot %°
0.2
e
80
X
*
*0*
xx
x
x
Iz
-
x
t
x
x xx
2
Patients and methods We measured the number of rectal fulgurations performed on patients with FAP who had undergone Correspondence to: R K S Phillips, St Mark's Hospital, City Road, London EC1
+
0
;QQQMQ
o
S ***-I** Duodenal Polyposis Stage x Iv X V + 11 * III *11111
Figure 1. Duodenal polyposis and number of fulgurations! year post ileorectal anastomosis. Median values for each polyposis stage is shown with a horizontal bar
0141-0768/92/ 020077-03/$02.00/0 © 1992 The Royal Society of Medicine
78
Journal of the Royal Society of Medicine Volume 85 February 1992
Table 1. Duodenal polyposis and fulgurations/year post ileorectal anastomosis
Stage of duodenal polyposis
Patients (n) Years post ileorectal anastomosis (range) Mean age (years) Sex: male female Fulguration ratio: median (interquartile range)
nII
III
IV
V*
17 179 (1-27) 39.1
28 315 (1-34) 37.9
27 423 (2-38) 44.1
9 193 (5-40) 49.6
10 256
(11-36) 53.7
13 4
14 14
17 10
4 5
7 3
0 (0-0.25)
0.13 (0-0.27)
0.23 (0.04-0.5)
0.38 (0.2-0.44)
0.38 (0.13-0.61)
*Foregut cancer patients Fulguration ratio/stage of duodenal polyposis: stage I compared with: stage IV, P
