World J. Surg. 15, 47-49, 1991
0
World Journal of Surgery 9 1991 by the Soci6t6 lnternationale de Chirurgie
Choice of Operation in Familial Adenomatous Polyposis David G. Jagelman, M.S.(Lon.), F.R.C.S.(Eng.), F.A.C.S. Cleveland Clinic Florida, Fort Lauderdale, Florida, U.S.A. Three surgical options are discussed for patients with familial adenomatous polyposis: proctocolectomy with Ueostomy, colectomy with ileorectai anastomosis, and colectomy with rectal mucosectomy and ileoanal pouch with a temporary ileostomy.
There has long been a debate as to the most appropriate operative procedure for patients with familial adenomatous polyposis (FAP). The clinical spectrum of this disease has been better defined and it is now known to be a generalized growth disorder as opposed to a specific colonic disease. Benign and extra colonic manifestations may occur, and often do, but the major concern is the development of and death from colorectal cancer which, if left untreated, is 100%. Removing the whole of the large intestine, however, will not guarantee a cure since it is anticipated that about 8-10% of patients with FAP will succumb to periampullary carcinoma and a further number of patients will die from desmoid, adrenal, brain, and thyroid tumors. The choice of prophylactic colonic operation must always be prefaced by the concept that the patient is not always certain of being cured; however, the major cause of death is colorectal cancer, and prophylactic colectomy soon after diagnosis is the most appropriate management for these patients. Over the years, the debate has centered around the discussion as to whether proctocolectomy with ileostomy is a more appropriate choice than colectomy with ileorectal anastomosis. In recent years, with the availability of colectomy, rectal mucosectomy and ileoanal pouch procedure, a further dimension in this discussion has arisen [1]. I would like to enumerate the advantages and disadvantages of the latter 2 surgical alternatives. It would seem unlikely that, in 1989, a patient should ever require proctocolectomy with ileostomy unless the diagnosis was made very late and a cancer had already arisen in the lower rectum. In the patient with FAP without malignant degeneration, there is no indication for proctocolectomy with ileostomy at this time. Proctocolectomy with Ileostomy
A few years ago, when the only other surgical alternative was ileorectal anastomosis, many surgeons recommended proctoReprint requests: David G. Jagelman, Cleveland Clinic Florida, 3000 West Cypress Creek Road, Fort Lauderdale, Florida 33309, U.S.A.
colectomy with ileostomy. This was based on the fear of the subsequent development of cancer of the rectum and it was felt unjustified to allow the rectum to remain because of that risk. It was, of course, somewhat difficult to persuade young asymptomatic patients to undergo ileostomy as compared to those patients who have ulcerative colitis who are grossly symptomatic. It has also been obvious in reviewing our records that family members who have been subjected to proctocolectomy with ileostomy have, in fact, caused other family members to have a negative response to the procedure, making it more difficult for those at risk to comply with surveillance examinations since they fear that they may end up with an ileostomy as well. Many of these patients have failed to return to follow-up and, in fact, the procedure has been a discouragement to the other family members from actually seeking help. Of course, if they fail to undergo a surgical prophylactic colectomy and are affected by the disease, they are in a 100% bracket for the development of colorectal cancer. Nowadays, with the availability of other surgical options, proctocolectomy with ileostomy as a primary procedure is no longer a necessary choice for patients with FAP. The only situation that might dictate the use of proctocolectomy with ileostomy would be a patient who is diagnosed late in the course of the disease who has already developed a rectal carcinoma low in the rectum. In the patient who has already had a colectomy with ileorectal anastomosis, who is older, and whose sphincters may be less satisfactory and who may subsequently develop a cancer in the rectum, it may be necessary to convert to an ileostomy. These are, however, somewhat unusual occurrences. Regular surveillance programs will diagnose the disease at an early age in children. Patients who have had an ileorectal anastomosis, if they maintain regular proctoscopic surveillance, have a comparatively low risk of developing cancer in the rectum. Colectomy with Ueorectal Anastomosis
The risk of cancer in the colon or rectum is, of course, 100% if left untreated; however, when diagnosed early, many patients have comparatively few polyps in the rectal area. Colectomy with ileorectal anastomosis, leaving approximately 12-15 cm of the rectum with primary anastomosis, has been the main surgical procedure for this condition in many centers, including our own. The operation is comparatively simple, it is a 1-stage
48
procedure, is compatible with a good functional result [2], and the complications are minimal. There should be no risk to sexual nerve function since the pelvis is not dissected and there is a limited amount of bowel left remaining for ongoing surveillance. Those performing this procedure are well aware that the rectal mucosa may undergo subsequent adenoma formation and there is a risk of malignant degeneration, even if one has surveillance proctoscopies with fulguration of detected polyps. This risk is, however, comparatively low. The negativity toward this procedure has been based on the data presented from the Mayo Clinic where it was found that the cumulative risk of developing rectal cancer was 59% at 23 years following colectomy [3]. Bess and associates [4] updated this experience and confirmed a high risk of rectal cancer of 32% in patients with a median follow-up of 20 years. In spite of this apparent dismal experience, 86% of their patients were free of rectal cancer 15 years after colectomy. This poor experience has not been duplicated by other centers which have investigated a large number of patients in follow-up over a good number of years. Bussey [5] reported the St. Mark's Hospital experience and found a cumulative risk of rectal cancer in patients having ileorectal anastomosis of 3.6% at 25-year follow-up and, at that time, no patients had died of rectal cancer. Their experience was subsequently updated [6] and, although more cancers arose, the mortality for rectal cancer was still low. During the same follow-up period, more patients had died with cancer of the duodenum than with cancer of the rectum. In our series [7] evaluating 133 patients, 10 developed rectal cancer but only 1 patient died of this complication. There was an 88% actuarial survivorsfiip free of rectal cancer at 20 years. This was very similar to the St. Mark's Hospital experience and, again, in our series, more patients have died of periampullary cancer than have died of rectal cancer following ileorectal anastomosis during the same 20~year follow-up period. It is obvious that the risk of rectal cancer is real, however, it seems to be controllable. Even though cancers can occur in comparatively small numbers over the years, most of them are detected early and are cured by proctectomy at that time. It is, thus, mandatory that annual proctoscopic surveillance be performed in these patients for life. It should also be noted that a patient undergoing such surveillance who seems to be developing inappropriately large numbers of rectal polyps can always be converted to an ileoanal pouch procedure or a protocolectomy with ileostomy at a later date to preempt the development of cancer of the rectum. Even patients who develop cancer in the rectum can have an ileostomy with proctectomy and those that develop cancer in the upper rectum would still be potential candidates for the ileoanal pouch procedure. The determining factor, therefore, in the choice of this procedure is that cancer can occur, but it seems to be controllable in most series with accurate follow-up, and that comparatively few patients would die of rectal cancer. One is trading off this apparently small risk of rectal cancer for the simplicity of ileorectal anastomosis, the lower rate of complications, and the quicker recovery with, in general, better bowel function than achieved by the ileoanal pouch procedure. Colectomy with Ileoanal Pouch
Colectomy with rectal mucosectomy and ileoanal pouch procedure with temporary loop ileostomy has advanced in recent years to be a very desirable option in patients who have FAP or
World J. Surg. Vol. 15, No, 1, Jan./Feh. 1991
ulcerative colitis. It offers removal of the large bowel mucosa and, therefore, eliminates the risk of colon or colorectal cancer and, at the same time, obviates the need for a permanent ileostomy. At face value, this is a very attractive alternative and is particularly satisfying as a more complex operative procedure surgically; however, it is not without its drawbacks. There is, certainly, a greater complication rate with this procedure, including pelvic sepsis and leakage from the pouch; it is a 2-stage procedure demanding the use of a temporary loop ileostomy and its subsequent closure some weeks or months later. The functional result is still questionable and, clinically, at least, would seem to be less satisfactory than the functional result of bowel activity following ileorectal anastomosis [8]. There have been some patients who have had an ileoanal pouch procedure performed without the concomitant temporary loop ileostomy. Some of these patients have fared successfully; others, however, have had complications of leakage and peritonitis and most surgeons, I think, would feel that the loop ileostomy at this stage in the development of the procedure is indicated in the vast majority of patients as a safety feature. We have reserved the procedure for patients who present late with large numbers of polyps in the rectum or for those who had already had an ileorectal anastomosis who subsequently develop large numbers of polyps and in whom one is particularly concerned of the risk of rectal cancer.
Summary
There are 3 surgical options for patients who have familial adenomatous polyposis. None of them guarantees a 100% cure of the disease, although they all minimize or eliminate the risk of colorectal cancer. Proctocolectomy with ileostomy is rarely necessary as a procedure of choice. Its only indication would be in patients who have already developed a rectal cancer in the lower third of the rectum. It tends not to be well accepted in asymptomatic patients and acts as a deterrent to family members to even seek help through surveillance. Colectomy with ileorectal anastomosis is a comparatively safe, simple, and uncomplicated procedure with a rapid recovery and a good functional result. It does, however, leave the patient liable for a small risk of rectal cancer, although not a large risk of dying of rectal cancer. Patients who do not do well with ileoreetal anastomosis or who subsequently develop large numbers of polyps or even cancer in the upper parts of the rectum at an early stage can still be converted to the third option, which is an ileoanal pouch procedure. Colectomy with rectal mucosectomy and ileoanal pouch procedure with a temporary ileostomy is not, in our view, necessary for all patients with FAP. It is reserved in our practice for patients who present late with large numbers of rectal polyps or for those who desire not to have any risk of rectal cancer and is also used for patients who have had ileorectal anastomosis who develop large numbers of polyps in follow-up surveillance and screening. We do not think that the complexity and higher complication rate and occasionally less-than-adequate functional result warrants using this procedure in all patients with FAP--especially in our patient population where the registry will often detect this disease at or
D.G. Jagelman: Familial Polyposis: Treatment
around the age of puberty when these patients may only have a handful of polyps in the rectum. Whichever operative procedure is chosen, these patients still require ongoing follow-up for life for fear of developing extracolonic benign and malignant manifestations in the duodenum, small intestine, adrenal gland, and thyroid gland as well as desmoid tumors which, in themselves, can cause death. R~sum~
I1 existe 3 possibilit6s th6rapeutiques pour le patient ayant une polypose ad6nomateuse familiale: coloproctectomie totale avec ileostomie, colectomie avec anastomose il6orectale, et colectomie avec mucosectomie rectale et cr6ation d'un resevoir il6oanal avec il6ostomie temporaire. Resumen
Se discuten 3 opciones quirtirgicas para pacientes con poliposis adenomatosa familiar: proctocolectomia con ileostomia, colectomia con anastomosis ileorrectal, y colectomia con mucosectom/a rectal y bolsa ileoanal con ileostomfa temporal.
49
References
1. Dozois, R.R., Berk, T., Billow, S., Cohen, Z., DeCosse, J.J., Hawley, P.R., Jagelman, D.G., J~irvinen, H.J., Macrae, F.A.: Symposium. Surgical aspects of familial adenomatous polyposis. Int. J. Colorect. Dis. 3:1, 1988 2. Newton, C.R., Baker, W.N.W.: Comparison of bowel function after ileorectal anastomosis for ulcerative colitis and colonic polyposis. Gut 16:785, 1975 3. Moertel, C.G., Hill, J.R., Adson, M.A.: Management of multiple polyposis of the large bowel. Cancer 28:160, 1971 4. Bess, M.A., Adson, M.A., Elveback, L.R., Moertel, C.G.: Rectal cancer following colectomy for polyposis. Arch. Surg. 115:460, 1980 5. Bussey, H.J.R.: Familial Polyposis Coli: Family Studies, Histopathology, Differential Diagnosis, and Results of Treatment, Baltimore, Johns Hopkins University Press, 1975, pp. 65-66 6. Bussey, H.J.R., Eyers, A.A., Ritchie, S.M., Thomson, J.P.S.: The rectum in adenomatous polyposis: The St. Mark's policy. Br. J. Surg. 72[Suppl.]:529, 1985 7. Sarre, R.G., Jagelman, D.G., Beck, G.J., McGannon, E., Fazio, V.W., Weakley, F.L., Lavery, I.C.: Colectomy with ileorectal anastomosis for familial adenomatous polyposis: The risk of rectal cancer. Surgery 101:20, 1987 8. Dozois, R.R., Kelly, K.A., Welling, D.R., Gordon, H., Beart, R.W., Wolff, B.G., Pemberton, J.H., Ilstrup, D.M.: Ileal pouch-anal anastomosis: Comparison of results in familial adenomatous polyposis and chronic ulcerative colitis. Ann. Surg. 210:268, 1989
0
World Journal of Surgery 9 1991 by the Soci6t6 lnternationale de Chirurgie
Choice of Operation in Familial Adenomatous Polyposis David G. Jagelman, M.S.(Lon.), F.R.C.S.(Eng.), F.A.C.S. Cleveland Clinic Florida, Fort Lauderdale, Florida, U.S.A. Three surgical options are discussed for patients with familial adenomatous polyposis: proctocolectomy with Ueostomy, colectomy with ileorectai anastomosis, and colectomy with rectal mucosectomy and ileoanal pouch with a temporary ileostomy.
There has long been a debate as to the most appropriate operative procedure for patients with familial adenomatous polyposis (FAP). The clinical spectrum of this disease has been better defined and it is now known to be a generalized growth disorder as opposed to a specific colonic disease. Benign and extra colonic manifestations may occur, and often do, but the major concern is the development of and death from colorectal cancer which, if left untreated, is 100%. Removing the whole of the large intestine, however, will not guarantee a cure since it is anticipated that about 8-10% of patients with FAP will succumb to periampullary carcinoma and a further number of patients will die from desmoid, adrenal, brain, and thyroid tumors. The choice of prophylactic colonic operation must always be prefaced by the concept that the patient is not always certain of being cured; however, the major cause of death is colorectal cancer, and prophylactic colectomy soon after diagnosis is the most appropriate management for these patients. Over the years, the debate has centered around the discussion as to whether proctocolectomy with ileostomy is a more appropriate choice than colectomy with ileorectal anastomosis. In recent years, with the availability of colectomy, rectal mucosectomy and ileoanal pouch procedure, a further dimension in this discussion has arisen [1]. I would like to enumerate the advantages and disadvantages of the latter 2 surgical alternatives. It would seem unlikely that, in 1989, a patient should ever require proctocolectomy with ileostomy unless the diagnosis was made very late and a cancer had already arisen in the lower rectum. In the patient with FAP without malignant degeneration, there is no indication for proctocolectomy with ileostomy at this time. Proctocolectomy with Ileostomy
A few years ago, when the only other surgical alternative was ileorectal anastomosis, many surgeons recommended proctoReprint requests: David G. Jagelman, Cleveland Clinic Florida, 3000 West Cypress Creek Road, Fort Lauderdale, Florida 33309, U.S.A.
colectomy with ileostomy. This was based on the fear of the subsequent development of cancer of the rectum and it was felt unjustified to allow the rectum to remain because of that risk. It was, of course, somewhat difficult to persuade young asymptomatic patients to undergo ileostomy as compared to those patients who have ulcerative colitis who are grossly symptomatic. It has also been obvious in reviewing our records that family members who have been subjected to proctocolectomy with ileostomy have, in fact, caused other family members to have a negative response to the procedure, making it more difficult for those at risk to comply with surveillance examinations since they fear that they may end up with an ileostomy as well. Many of these patients have failed to return to follow-up and, in fact, the procedure has been a discouragement to the other family members from actually seeking help. Of course, if they fail to undergo a surgical prophylactic colectomy and are affected by the disease, they are in a 100% bracket for the development of colorectal cancer. Nowadays, with the availability of other surgical options, proctocolectomy with ileostomy as a primary procedure is no longer a necessary choice for patients with FAP. The only situation that might dictate the use of proctocolectomy with ileostomy would be a patient who is diagnosed late in the course of the disease who has already developed a rectal carcinoma low in the rectum. In the patient who has already had a colectomy with ileorectal anastomosis, who is older, and whose sphincters may be less satisfactory and who may subsequently develop a cancer in the rectum, it may be necessary to convert to an ileostomy. These are, however, somewhat unusual occurrences. Regular surveillance programs will diagnose the disease at an early age in children. Patients who have had an ileorectal anastomosis, if they maintain regular proctoscopic surveillance, have a comparatively low risk of developing cancer in the rectum. Colectomy with Ueorectal Anastomosis
The risk of cancer in the colon or rectum is, of course, 100% if left untreated; however, when diagnosed early, many patients have comparatively few polyps in the rectal area. Colectomy with ileorectal anastomosis, leaving approximately 12-15 cm of the rectum with primary anastomosis, has been the main surgical procedure for this condition in many centers, including our own. The operation is comparatively simple, it is a 1-stage
48
procedure, is compatible with a good functional result [2], and the complications are minimal. There should be no risk to sexual nerve function since the pelvis is not dissected and there is a limited amount of bowel left remaining for ongoing surveillance. Those performing this procedure are well aware that the rectal mucosa may undergo subsequent adenoma formation and there is a risk of malignant degeneration, even if one has surveillance proctoscopies with fulguration of detected polyps. This risk is, however, comparatively low. The negativity toward this procedure has been based on the data presented from the Mayo Clinic where it was found that the cumulative risk of developing rectal cancer was 59% at 23 years following colectomy [3]. Bess and associates [4] updated this experience and confirmed a high risk of rectal cancer of 32% in patients with a median follow-up of 20 years. In spite of this apparent dismal experience, 86% of their patients were free of rectal cancer 15 years after colectomy. This poor experience has not been duplicated by other centers which have investigated a large number of patients in follow-up over a good number of years. Bussey [5] reported the St. Mark's Hospital experience and found a cumulative risk of rectal cancer in patients having ileorectal anastomosis of 3.6% at 25-year follow-up and, at that time, no patients had died of rectal cancer. Their experience was subsequently updated [6] and, although more cancers arose, the mortality for rectal cancer was still low. During the same follow-up period, more patients had died with cancer of the duodenum than with cancer of the rectum. In our series [7] evaluating 133 patients, 10 developed rectal cancer but only 1 patient died of this complication. There was an 88% actuarial survivorsfiip free of rectal cancer at 20 years. This was very similar to the St. Mark's Hospital experience and, again, in our series, more patients have died of periampullary cancer than have died of rectal cancer following ileorectal anastomosis during the same 20~year follow-up period. It is obvious that the risk of rectal cancer is real, however, it seems to be controllable. Even though cancers can occur in comparatively small numbers over the years, most of them are detected early and are cured by proctectomy at that time. It is, thus, mandatory that annual proctoscopic surveillance be performed in these patients for life. It should also be noted that a patient undergoing such surveillance who seems to be developing inappropriately large numbers of rectal polyps can always be converted to an ileoanal pouch procedure or a protocolectomy with ileostomy at a later date to preempt the development of cancer of the rectum. Even patients who develop cancer in the rectum can have an ileostomy with proctectomy and those that develop cancer in the upper rectum would still be potential candidates for the ileoanal pouch procedure. The determining factor, therefore, in the choice of this procedure is that cancer can occur, but it seems to be controllable in most series with accurate follow-up, and that comparatively few patients would die of rectal cancer. One is trading off this apparently small risk of rectal cancer for the simplicity of ileorectal anastomosis, the lower rate of complications, and the quicker recovery with, in general, better bowel function than achieved by the ileoanal pouch procedure. Colectomy with Ileoanal Pouch
Colectomy with rectal mucosectomy and ileoanal pouch procedure with temporary loop ileostomy has advanced in recent years to be a very desirable option in patients who have FAP or
World J. Surg. Vol. 15, No, 1, Jan./Feh. 1991
ulcerative colitis. It offers removal of the large bowel mucosa and, therefore, eliminates the risk of colon or colorectal cancer and, at the same time, obviates the need for a permanent ileostomy. At face value, this is a very attractive alternative and is particularly satisfying as a more complex operative procedure surgically; however, it is not without its drawbacks. There is, certainly, a greater complication rate with this procedure, including pelvic sepsis and leakage from the pouch; it is a 2-stage procedure demanding the use of a temporary loop ileostomy and its subsequent closure some weeks or months later. The functional result is still questionable and, clinically, at least, would seem to be less satisfactory than the functional result of bowel activity following ileorectal anastomosis [8]. There have been some patients who have had an ileoanal pouch procedure performed without the concomitant temporary loop ileostomy. Some of these patients have fared successfully; others, however, have had complications of leakage and peritonitis and most surgeons, I think, would feel that the loop ileostomy at this stage in the development of the procedure is indicated in the vast majority of patients as a safety feature. We have reserved the procedure for patients who present late with large numbers of polyps in the rectum or for those who had already had an ileorectal anastomosis who subsequently develop large numbers of polyps and in whom one is particularly concerned of the risk of rectal cancer.
Summary
There are 3 surgical options for patients who have familial adenomatous polyposis. None of them guarantees a 100% cure of the disease, although they all minimize or eliminate the risk of colorectal cancer. Proctocolectomy with ileostomy is rarely necessary as a procedure of choice. Its only indication would be in patients who have already developed a rectal cancer in the lower third of the rectum. It tends not to be well accepted in asymptomatic patients and acts as a deterrent to family members to even seek help through surveillance. Colectomy with ileorectal anastomosis is a comparatively safe, simple, and uncomplicated procedure with a rapid recovery and a good functional result. It does, however, leave the patient liable for a small risk of rectal cancer, although not a large risk of dying of rectal cancer. Patients who do not do well with ileoreetal anastomosis or who subsequently develop large numbers of polyps or even cancer in the upper parts of the rectum at an early stage can still be converted to the third option, which is an ileoanal pouch procedure. Colectomy with rectal mucosectomy and ileoanal pouch procedure with a temporary ileostomy is not, in our view, necessary for all patients with FAP. It is reserved in our practice for patients who present late with large numbers of rectal polyps or for those who desire not to have any risk of rectal cancer and is also used for patients who have had ileorectal anastomosis who develop large numbers of polyps in follow-up surveillance and screening. We do not think that the complexity and higher complication rate and occasionally less-than-adequate functional result warrants using this procedure in all patients with FAP--especially in our patient population where the registry will often detect this disease at or
D.G. Jagelman: Familial Polyposis: Treatment
around the age of puberty when these patients may only have a handful of polyps in the rectum. Whichever operative procedure is chosen, these patients still require ongoing follow-up for life for fear of developing extracolonic benign and malignant manifestations in the duodenum, small intestine, adrenal gland, and thyroid gland as well as desmoid tumors which, in themselves, can cause death. R~sum~
I1 existe 3 possibilit6s th6rapeutiques pour le patient ayant une polypose ad6nomateuse familiale: coloproctectomie totale avec ileostomie, colectomie avec anastomose il6orectale, et colectomie avec mucosectomie rectale et cr6ation d'un resevoir il6oanal avec il6ostomie temporaire. Resumen
Se discuten 3 opciones quirtirgicas para pacientes con poliposis adenomatosa familiar: proctocolectomia con ileostomia, colectomia con anastomosis ileorrectal, y colectomia con mucosectom/a rectal y bolsa ileoanal con ileostomfa temporal.
49
References
1. Dozois, R.R., Berk, T., Billow, S., Cohen, Z., DeCosse, J.J., Hawley, P.R., Jagelman, D.G., J~irvinen, H.J., Macrae, F.A.: Symposium. Surgical aspects of familial adenomatous polyposis. Int. J. Colorect. Dis. 3:1, 1988 2. Newton, C.R., Baker, W.N.W.: Comparison of bowel function after ileorectal anastomosis for ulcerative colitis and colonic polyposis. Gut 16:785, 1975 3. Moertel, C.G., Hill, J.R., Adson, M.A.: Management of multiple polyposis of the large bowel. Cancer 28:160, 1971 4. Bess, M.A., Adson, M.A., Elveback, L.R., Moertel, C.G.: Rectal cancer following colectomy for polyposis. Arch. Surg. 115:460, 1980 5. Bussey, H.J.R.: Familial Polyposis Coli: Family Studies, Histopathology, Differential Diagnosis, and Results of Treatment, Baltimore, Johns Hopkins University Press, 1975, pp. 65-66 6. Bussey, H.J.R., Eyers, A.A., Ritchie, S.M., Thomson, J.P.S.: The rectum in adenomatous polyposis: The St. Mark's policy. Br. J. Surg. 72[Suppl.]:529, 1985 7. Sarre, R.G., Jagelman, D.G., Beck, G.J., McGannon, E., Fazio, V.W., Weakley, F.L., Lavery, I.C.: Colectomy with ileorectal anastomosis for familial adenomatous polyposis: The risk of rectal cancer. Surgery 101:20, 1987 8. Dozois, R.R., Kelly, K.A., Welling, D.R., Gordon, H., Beart, R.W., Wolff, B.G., Pemberton, J.H., Ilstrup, D.M.: Ileal pouch-anal anastomosis: Comparison of results in familial adenomatous polyposis and chronic ulcerative colitis. Ann. Surg. 210:268, 1989
