familial adenomatous polyposis ( fap ) is an autosomal dominant disease characterized by the presence of numerous adenomatous polyps in the colon . it affects between 1 in 8,300 to 1 in 14,025 live births , affecting both the sexes equally . the polyps inevitably develop into cancer in 10 years to15 years after their appearance , and the only treatment available at present to prevent the cancer is total proctocolectomy . the characteristic of this disease is the young age at presentation with the average age at diagnosis of colorectal cancer being 39 years . in patients diagnosed with fap , prophylactic colectomy the young age of the patient makes cosmesis a significant consideration when choosing the mode of therapy . the aim of this study was to demonstrate the feasibility of laparoscopic restorative proctocolectomy with ileal pouch anal anastomosis ( rpc - ipaa ) for fap and to verify its outcome . a single operating surgeon ( cp ) assisted by a surgical team performed all the surgeries . before embarking on this study , we had developed sufficient experience in laparoscopic colorectal surgery , which has been published elsewhere . all patients are administered intravenous antibiotics in the form of ciprofloxacin 500 mg iv , ornidazole 500 mg iv one hour before surgery , and antithrombotic prophylaxis is administered with low - molecular - weight heparin and elastic stockings . a nasogastric tube and a urinary catheter are inserted before the start of the procedure . basically , the surgery consists of a combination of various phases of mobilization and ligation of pedicles involved in the performance of left , right , and transverse colectomy . the thighs are flexed minimally so as to not to impede the movement of laparoscopic instruments . the importance of a remote - controlled operating table that can be tilted on any axis can not be overemphasized . it is gravity that is most often used as a retractor to keep the bowels away from the operating area . the procedure starts with mobilization of the sigmoid and rectosigmoid , which is continued cranially towards the descending colon . . the surgeon moves between the legs of the patients for mobilization of the splenic flexure and transverse colon . the ascending colon and hepatic flexure are mobilized from the left side of the patient , and the distal ileum is transected using laparoscopic staplers . the surgeon returns to the right side for rectal mobilization and ultra - low resection using staplers . during all the phases of the surgery , the dissection has been medial to lateral . we follow the planes of oncological clearance as these planes are avascular , and secondly , to provide adequate clearance even if the final histopathological examination of the specimen reveals malignancy that was not detected by preoperative sampling of the polyps . thereafter , we deal with the left colic and middle colic pedicles in a similar fashion during the phases of dissection of the left and transverse colon , respectively . during the phase of transverse colon mobilization , the greater omentum is released from the transverse colon and placed in the left upper quadrant . flush ligating the pedicles and use of ultracision for dissection enables a relatively bloodless field and minimal perioperative blood loss . through a minilaparotomy pfannenstiel incision the stapled end of the terminal ileum is delivered into the wound , and a j pouch is fashioned by using the linear stapler . an enterotomy is made at the apex of the pouch , and the blades of the linear stapler ( 7.5 cm ) are introduced into each of the limbs of the pouch and sequentially fired twice to obtain the pouch . the anvil of the circular stapler is inserted into the apex of the pouch through the enterotomy and secured with pursestring sutures . the pouch is delivered into the abdomen , the pfannenstiel incision is closed , and pneumoperitoneum reestablished . after confirming the correct orientation of the pouch , it is anastomosed to the anal canal via a transanally introduced circular stapler ( figure 2 ) . the magnification afforded by the laparoscope allows ultra - low dissection so that the rectal stump is divided at the dentate line itself . in case a proximal division occurs , rectal mucosectomy is carried out transanally prior to pouch anal anastomosis . we perform a protective ileostomy in all patients , which is closed after 6 weeks . we prefer a brooke 's type of everting ileostomy placed in the right iliac fossa . a. 10 mm  camera port for mobilization of rectum / sigmoid / hepatic flexure and right half of trans - verse colon ; right - hand working port for mobilization of descending colon , caecum , and ascending colon ; left - hand working port for mobilization of splenic flexure and left half of the transverse colon . b. 10 mm or 5 mm  right - hand working port for mobilization of transverse colon ; retraction for mobilization of rectum , sigmoid colon , and descending colon . c. 10 mm camera port for mobilization of descending colon , splenic flexure , left half of transverse colon , caecum , and ascending colon . d. 5 mm  left - hand working port for mobilization of rectum , sigmoid colon , descending colon , caecum , ascending colon , hepatic flexure , and right half of transverse colon ; retraction for mobilization of splenic flexure and left half of transverse colon . fifteen fap patients underwent restorative total proctocolectomy with ileal pouch anal anastomosis ( rpc - ipaa ) . in these 4 patients , preoperative colonoscopy and biopsy had revealed adenocarcinoma in rectal polyps . the american joint committee on cancer ( ajcc ) staging of 2 patients was t2n0m0 , and the other 2 patients had t3n0m0 stage malignancy . the tumors were located 8 cm , 12 cm , 13 cm , and 16 cm from the anal verge in these patients . the operating time ranged for 190 minutes to 365 minutes with a median of 225 minutes . the mean blood loss was 60 ml with a range of 30 ml to 180 ml . the average incision length of the mini - pfannenstiel incision was 5 cm ( range , 4 to 6.5 ) . twelve patients resumed a liquid diet on the second postoperative day , while 3 patients did so on the third postoperative day . the malignancy and staging was confirmed in the 4 patients with proven rectal adenocarcinoma . in one patient with pre - operative staging of t3n0m0 , in all the patients , the margins were free from tumors , and the minimum distal margin was 4 cm . the pouch frequency varied between 3 and 6 , with the median being 4 times . though initially , patients complained of liquid stools , by one month postoperatively , they were passing semisolid stools and did not suffer from urgency . one patient developed wound infection that was treated by a change in antibiotic , according to the culture sensitivity report . this patient was an elderly male of 52 years and had an enlarged prostate ( 42 g ) on ultrasonography preoperatively . he was started on tablet tamsulosin 0.4 mg once a day at night , and the catheter was removed on the seventh day . she was kept nil orally with ryle 's tube decompression of the stomach and repeated enemas to clear the pouch . the patient recovered and was started on oral feedings by the fifth day of treatment and was discharged on the seventh day . the patient was taught self - administration of enemas and pouch irrigation . with this , the patient has not experienced any problems after a mean follow - up of 3 years and 10 months . none of the male patients had problems with potency , orgasm sensation , ejaculation , or micturition . in response to a questionnaire , all the patients declared themselves to be highly satisfied with the outcome of the surgery and the cosmesis and would recommend the procedure to other patients . all the patients were called for follow - up after 7 days , 1 month , 6 months , and yearly thereafter , except for the patients with malignancy who were called monthly after the first month and up to 1 year and then yearly thereafter . at every visit , the patient is examined . at the 3-month visit , endoscopic pouch examination with a flexible sigmoidoscope and upper gi endoscopy all patients have maintained follow - up , and the average follow - up has been 3 years and 10 months . there has been no recurrence of malignancy or polyps in any of the patients . moreover , the upper gi endoscopy has revealed the absence of polyps in the duodenum . the patient with t3n1mo malignancy was referred to an oncologist and received adjuvant chemotherapy consisting of irinotecan , 5-fu , and folinic acid . he has tolerated the chemotherapy well and is disease - free after 2 years and 4 months of follow - up . though all the patients ' first - degree relatives have been counseled to undergo screening for familial adenomatous polyposis , only 13 such relatives belonging to 5 patients have come for a colonoscopy . of these familial adenomatous polyposis ( fap ) is a rare autosomal dominant disease caused by a defect in the apc gene of the 5q21 chromosome . all affected untreated patients will die of colorectal adenocarcinomas in the fourth or fifth decades , which was the fate of most patients in the first half of the 20th century . however , thereafter , several national registries were established , and screening programs were started . early detection and treatment of this disease has resulted in a reduced incidence of death from colorectal cancer . it has been demonstrated that establishment of a national registry improves the colectomy rate , reduces the prevalence of colorectal cancer , and improves survival . today , the most frequent causes of death in screening detected patients are duodenal cancer and desmoid tumor . similarly , in a study based on the hong kong registry , the median age of diagnosis was significantly lower in patients detected by screening than those diagnosed by symptomatic presentation ( 29 years vs. 34 years , respectively ) . at the time of diagnosis , 9.7% of the screened patients had malignancy compared with 61% of the unscreened patients . however , india does not have a national registry for fap , nor does it have a screening program . as a result , most of our patients presented late ( mean age being 44.4 years ) , and 4 of the 9 patients had already developed malignancy . of the 9 patients , 5 were diagnosed with fap while being investigated by us for abdominal symptoms , including the 4 patients with malignancy . the remaining patients had siblings or parents suffering from the disease and were referred to us for management of their disease . thus , establishment of national or regional registries is the need of the hour to help early detection and treatment of these patients . though primary chemoprevention of fap has been attempted with sulindac and cyclo - oxygenase inhibitors , it has not proven completely successful . the surgical options available include total proctocolectomy with ileostomy ( tpc ) and restorative proctocolectomy with ileal pouch anal anastomosis ( rpc - ipaa ) , which ensures elimination of all rectal and colonic mucosa . however , tpc mandates a permanent ileostomy , which is not a desirable option in these predominantly young patients . the laparoscopic approach would seem a logical choice in these patients due to the improved cosmetic benefit . however , laparoscopic total colectomy is a most complex and demanding procedure in laparoscopic colorectal surgery , requiring significant experience in advanced laparoscopic surgery . surgeons have to operate in all the quadrants of the abdomen and should have a thorough knowledge of anatomy of these regions before performing this procedure . though the initial reports indicated that laparoscopic colectomy took a significantly prolonged time to accomplish compared with open colectomy , with increasing experience , this difference has been reduced . various studies have shown that laparoscopic surgery is associated with decreased analgesic requirement , earlier return of bowel function , reduced length of hospital stay , earlier return to routine work and improved cosmesis compared with open surgery . restorative proctocolectomy with ileal pouch anal anastomosis ( rpc  ipaa ) was first described in 1978 . since then , this procedure has become the procedure of choice in the surgical management of patients with ulcerative colitis ( uc ) and familial adenomatous polyposis ( fap ) . this is the preferred approach , especially in this subset of patients who are usually young and hence appreciate the cosmetic advantage and have a better body image . experience gained in performing partial and segmental colectomies helps in mastering the techniques of total colectomy . it is difficult to judge the impact of laparoscopic surgery in treatment of fap compared with conventional techniques due to a lack of adequate data in the literature . a few controlled studies have compared laparoscopic rpc - ipaa with open procedures for patients with ulcerative colitis . some of these series have also included patients with fap . most of the series have shown prolonged operative times with the laparoscopic approach compared with the open approach , except for a series by araki et al . the latter showed similar operative times in both the arms probably because only the colonic mobilization was being done laparoscopically , while the vessel transection and rectal mobilization was being done by a mini - laparotomy incision . however , with the use of advanced instrumentation like the ligasure ( valleylab , bolder , co , usa ) for vessel tran - section and harmonic scalpel ( ethicon endo - surgery , cincinnati , oh , usa ) for dissection and tissue transection , the speed of surgery increases , which is borne out by the fact that our median operating time of 225 minutes was comparable to open resection times reported in the former series . while the earlier study by schmitt et al reported a higher morbidity of 68% by the laparoscopic approach compared with 35% by the open approach , all of the later series showed lower morbidity by the laparoscopic approach . in our series , we did not encounter any significant complication except for one patient who presented with subacute intestinal obstruction 6 months after the surgery and was managed conservatively . length of hospitalization in our series averaged 8 days , which is similar to that reported by others and slightly less than for the open counterparts . however , this is not a true reflection of the need for hospitalization of these patients because our patients preferred to remain in the hospital until sutures were removed from the mini - pfannenstiel incision , and the final histopathological examination report of the specimen was ready . due to these peculiar geographical and socioeconomic factors , in spite of most ( 80% ) of our patients having started an oral diet by the second postoperative day and all of them being on oral diet by the third day , the length of hospitalization was inordinately long . in fact , cosmetic benefit alone has been shown to be of paramount importance to patients , especially as the young age group of patients is more concerned about body image . denker et al have shown that satisfaction with the cosmetic result of the scar was significantly higher with the laparoscopic rpc - ipaa compared with the open group . we had a similar experience as the cosmetic benefit of a small pfannenstiel incision ( average length of 5 cm ) was appreciated by all our patients in the postoperative surveillance questionnaire , and all of them would recommend this procedure to other patients . all the patients with malignancy underwent total mesorectal excision and after a mean follow - up of 2.1 years are free from recurrence . one of the controversies surrounding rpc - ipaa has been the development of pouch adenomas , the incidence of which has been reported to be between 8% and 60% on long - term follow - up . one of the risk factors for the development of pouch polyps is the retained rectal mucosa as a result of the stapled anastomosis . van duijvendijk et al found that the cumulative risk of developing polyps after rpc and ipaa at the anastomotic site was 31% for the stapled group and 10% for the sutured group . in contrast , polese et al did not find any increase in risk of pouch polyp formation after stapled anastomosis . though our follow - up period has not been long ( range , 1 year to 8 years , median 3.4 ) , we have not encountered pouch polyposis in any of our patients . however , we suggest a regular follow - up regime for our patients for routine screening , which consists of yearly endoscopy of the pouch with biopsy of any suspicious lesions . finally , the contentious issue is the cost factor . though no studies to date have objectively dealt with this issue , we feel that the initial higher instrument cost for laparoscopic surgery can be offset by having a dedicated laparoscopic surgical unit , use of reusable instruments , faster operative times due to availability of advanced instrumentation , and the shorter hospitalization . moreover , the cosmetic benefit and patient satisfaction afforded by the laparoscopic approach have also to be factored in . based on this , we are of the opinion that laparoscopic rpc - ipaa should be the approach of choice in the predominantly young patients afflicted by fap . we have shown that laparoscopic total proctocolectomy with ileal pouch anal anastomosis is a feasible surgery with minimal morbidity . while the laparoscopic approach offers definite cosmetic benefit as well as early return of bowel function , its real worth can only be established by comparative studies with the open approach , which , given the rarity of the condition , may be a difficult proposition . we feel that in the absence of the peculiar geographical and socioeconomic constraints that we face in our country , the length of hospitalization can also be reduced in these patients , further adding to its benefits
background : familial adenomatous polyposis is a hereditary disease characterized by the presence of thousands of colonic adenomas , which , if untreated , invariably undergo malignant transformation . because this disease manifests at a young age , the laparoscopic approach to perform surgery would be desirable due to its cosmetic benefits . 
 we describe our experience with this procedure and review the literature on the topic.methods:this is a case series of 15 patients who underwent restorative proctocolectomy with ileo - anal pouch anastomosis for familial adenomatous polyposis between 2000 and 2007 . 
 the salient operative steps are described.results:there were 9 males and 6 females , 32 to 52 years of age , with an average age of 44.8 years . 
 the median body mass index was 21.5 ( range , 17 to 28 ) . 
 rectal cancer was already present in 4 patients at the time of diagnosis . 
 the median operating time was 225 minutes . 
 mean blood loss was 60ml , with none of the patients requiring perioperative blood transfusion . 
 none of the surgeries required conversion to the open approach . 
 bowel function resumed on the second postoperative day in 12 patients and on the third postoperative day in 3 patients . 
 the median hospital stay was 8 days . 
 postoperatively , there was no mortality and no serious morbidity.conclusion:laparoscopic restorative proctocolectomy with ileal pouch anal anastomosis is a feasible surgery for familial adenomatous polyposis , and considering its cosmetic benefit , is a desirable option for this group of predominantly young patients .