Open Versus Laparoscopic Total Proctocolectomy with Ileal Pouch Anal Anastomosis for FAP / 대한내시경복강경외과학회지

PURPOSE: Restorative proctocolectomy with an ileal pouch anal anastomosis (IPAA) is the main surgical treatment for patients with familial adenomatous polyposis (FAP). With the advancements of minimal-invasive surgery, proctocolectomy with an ileal pouch anal anastomosis is increasingly being done by laparoscopic methods. This report aims to compare the presumed benefits of the laparoscopic approach with that of open surgery. METHODS: We reviewed 25 patients who underwent total proctocolectomy with IPAA between 1994 and 2009. The data of 9 patients who underwent laparoscopic surgery was analyzed and compared with the data of 16 patients who underwent the conventional open surgery. RESULTS: Laparoscopic proctocolectomy with an ileal pouch anal anastomosis (IPAA) was performed successfully without severe complications in 9 patients. The mean operation time of the laparoscopic group was 352 min, and this was significantly longer than that of the conventional group (252 min). The mean intra-operative blood loss, time to first flatulance, the hospital stay and the time to starting an oral diet were not significantly different from that of the open group. CONCLUSION: Laparoscopic IPAA is a feasible and safe procedure due to the reduced trauma and pain and a more favorable cosmetic result. As the technique and instrumentation for laparoscopic colon surgery are developed, this procedure will likely become an appealing option for the management of patients with FAP.

Coexistence of the Renal Fibromuscular Dysplasia and Familial Adenomatous Polyposis / 대한신장학회지

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that most commonly affects the renal and internal carotid arteries. A Familial adenomatous polyposis (FAP) is characterized by hundreds of adenomatous colorectal polyps, with an almost inevitable progression to colorectal cancer. A 19-year-old woman with history of total colectomy as a result of familial adenomatous polyposis presented with high blood pressure incidentally. Abdominal CT scan showed "string of beads" appearance of right renal artery. Percutaneous transluminal renal angioplasty was performed and then hypertension was improved. To our knowledge, there has been no previous case report of secondary renovascular hypertension resulting from fibromuscular dysplasia in patients with FAP. Here, we report a patient with a review of the literature.

Laparoscopic Total Proctocolectomy with Ileal pouch-anal Anastomosis for Patients of Familial Adenomatous Polyposis with or without Coexisting Colorectal Cancer

PURPOSE: Familial adenomatous polyposis (FAP) normally appears in the early twenties and needs a restorative total proctocolectomy with ileal pouch-anal anastomosis (TPC/ IPAA). Thus, most patients with FAP are young, in socially active stage, and very concerned about their body image. Vast experience with laparoscopic colorectal surgery led us to perform laparoscopic-assissted TPC/IPAA for patients with FAP with or without cancer, and we evaluated the results from technical and oncologic aspects. METHODS: Seventeen of 20 FAP patients underwent laparoscopic- assisted surgery between July 1996 and June 2004. All procedures were done in a totally laparoscopic, a laparoscopic-assisted, or a hand-assisted laparoscopic fashion. RESULTS: Fifteen patients underwent laparoscopic-assisted TCP/IPAA; two others had a total colectomy with ileorectal anastomosis and a TCP with permanent ileostomy laparoscopically. Eight patients showed coexisting colorectal cancers. The mean operation time was 396.5 min. Patients passed flatus or liquid at the 2.2 post-operative day (POD), resumed meals at the 4th. POD, and were discharged at the 10th. POD. There were no intra-operative complications or open conversions. Post-operative complications occurred in 5 different patients. One patient with colon cancer had multiple hepatic metastases at 11 months after the operation and died at 24 months after the operation. CONCLUSIONS: Laparoscopic-assisted surgery for the patients with FAP was technically feasible and could be an alternative method. The systematized and experienced approach could reduce a operation time to be acceptable. In selected cases and with a vast of experience, coexisting colorectal cancer would not be contraindicated for laparoscopic approach for the treatment of FAP.

Comparison of the Result of Restorative Proctocolectomy and Ileal Pouch-anal Anastomosis in Familial Adenomatous Polyposis and Ulcerative Colitis

PURPOSE:The aim of this study was to compare the early postoperative results and the long-term outcome of restorative proctocolectomy and ileal pouch-anal anastomosis (IPAA) in familial adenomatous polyposis (FAP) and ulcerative colitis (UC). METHODS:Thirty patients that underwent IPAA for either FAP (14 patients) or UC (16 patients) at Kyung-Hee University Hospital between January 1987 and December 1999 were studied retrospectively. Either handsewn or stapled anastomosis technique was used in IPAA. Most patients (12 patients in FAP, 16 patients in UC) had a two-stage operation with temporary diverting loop ileostomy and two patients with FAP had a one-stage operation without temporary ileostomy. RESULTS:One patient in the UC group died from sepsis after operation (n=16, 6.25%), but no patients in the FAP group died. Overall operative complications appeared in two patients (14.3%) and four patients (25%) with FAP and UC, respectively. At follow-up (mean, 47.3 months), pouchitis was developed in four patients with UC, but no patients with FAP. The mean daytime stool frequency was 4.5 stools per day in FAP patients and 5.8 stools per day in UC patients (P=0.031), but night-time stool frequency was similar between two groups (1.2 and 1.4 in FAP and UC, respectively; P>0.05). Daytime fecal incontinence was noticed in two patients (14.3%) with FAP and four patients (26.7%) with UC. Night-time fecal incontinence was noticed in three patients (21.4%) with FAP and six patients (40.0%) with UC. CONCLUSIONS:FAP patients tolerated the operation better and had less long-term disability than did UC patients. This suggested that the long-term outcome of IPAA procedure may depend on the primary disease rather than the procedure itself.

Congenital Hypertrophy of the Retinal Pigment Epithelium in Familial Colonic Polyposis

Congenital hypertrophy of the retinal pigment epithlium(CHRPE)is a well-circumscribed, flat, pigmented fundus lesion that is presented solitary or grouped. It is the most common extracolonic manifestation of familial adenomatous polyposis(FAP). Unfortunately, there is no phenotypic biochemical abnormality or serological marker which indicates whether or not a family member will be affected FAP. However, most of patients with polyposis have CHRPE which is usually multiple and bilateral, The presence of CHRPE in a family member should therefore arouse suspicion of increased risk of polyposis. We experienced one case of familial adenomatous polyposis that showed no extracolonic manifestation except multiple, bilateral fundus lesions. A prophylactic total colectomy was performed. So we report it with the review of literature of clinical value of CHRPE as a predictive congenital marker of FAP.

Three Cases of Familial Adenomatous Polyposis

Familial polyposis is a rare genetic disorder which the large intestine is diffusely carpeted by numerous adenomatous polyps. According to the recent studies, familial polyposis is a disease which produces polypoid lesions not only in the large intestine but also in the stomach, duodenum, ileum and jejunum. The natural course of extracolonic lesions is variable, but the colonic polyps are associated with high incidence of cancer. The risk of malignant change is virtually 100% if untreated. Thus when adenomatosis is noted, familial members at risk for familial adenomatous polyposis must be screened and prophylactic surgery performed to prevent inevitable colon cancer. Controversy exists about the most appropriate prophylactic treatment. Because of many disadvantages of ileostomy, total colectomy with ileorectal anastomosis (ileoproctostomy) or total colectomy with rectal mucosa stripping and ileoanal anastomosis has become a more preferred approach. After ileorectal anastomosis, polyps in the retained rectum must be removed by endoscopic polypectomy and rigorously followed by sigmoidoscopy every few months for prevention of polyp reccurence or malignant transformation. We experienced three cases of familial adenomatous polyposis and 2 of them were treated successfully by preoperative colonoscopic polypectomy, total colectomy and ileoproctostomy.

Gardner's Syndrome A case report

Familial adenomatous polyposis(FAP) is a hereditary disorder characterized by the development in adolescence or early adult life of multiple adenomatous polyps throughout the colon and rectum. The risk of malignant transformation is so high as to virtually 100 per cent if the patient lives long enough. Gardner's syndrome, a variant of familial adenomatous polyposis, is characterized by colorectal adenomas, multiple osteomas and variety of soft tissue lesions, including sebaceous cysts, fibromas, lipomas, and desmoid tumors. The recent study show that this group of diseases are caused by different mutations of the same gene located on the long arm of chromosome 5. All family members at risk of inheriting this gene need frequent surveillance of the colon. This should start between the ages of 8 and 12 years and should be continued at regular intervals. Prompt colectomy is indicated at the time the diagnosis is made. We experienced a case of Gardner's syndrome, which had typical extracolonic manifestations. Herein, we report this case with a review of the literature.

Carcinoma in an Ileal Pouch after Proctocolectomy, with Ileal Pouch-Anal

Familial adenomatous polyposis(FAP) has a significant risk of developing colorectal cancer. As a consequence, their surgical treatment is currently widely performed by restorative proctocolectomy with ileal pouch-anal anastomosis(IPAA). Frequently, the potential for recurrence in FAP patients after above operation was overlooked. In fact, several reports were presented for warning of recurred cancer in ileal pouch. We report a case of a patient who developed adenocarcinoma in an ileal pouch after restorative proctocolectomy for familial adenomatous polyposis with a review of literature.

Familial Adenomatous Polyposis with Gallbladder Polyps

Familial adenomatous polyposis(FAP) is an inherited autosomal dominant disorder characterized by the appearance in early life of numerous adenomas of colon and rectum. According to the recent studies, familial polypolis is a disease which produces polypoid lesions not only in the large intestine but also in the stomach duodenum, ileum and jejunum. The natural course of extracolonic lesions is variable, but the colonic polyps are associated with high incidence of cancer. The risk of malignant change is virtually 100% if untreated. The authors report a case of FAP in a 36-year-old-female who presented colorectal polyposis with multiple adenomas of the gallbladder. A review of literature with emphasis on the association of adenoma of the gallbladder was made.

A Case of Familial Adenomatous Polyposis (FAP) with A Large Sentinel Polyp / 대한소화기내시경학회지

Familial adenomatous polyposis(FAP) is a rare hereditary disorder characterized by the development of hundreds to thounds polyps throughout the colon and rectum. Moreover, several extracolonic manifestations are seen. Recently, this disease is recognized as a adenomatous polyposis syndrome which can involve the entire astrointestinal tract. Several reports have demonstrated a high incidence of gastroduodenal polyps in patients with familial adenomatous polyposis. These colon polyps can be eventually developed as colon cancer, if not be treated. So early diagnosis is needed and prophylactic surgery should be erformed. We experienced a case of familial adenomatous polyposis with a large sentinel polyp on the sigmoid colon, presenting hematochezia and mucoid diarrhea. He was early treated before progression to carcinoma by total colectomy, rectal mucosectomy and J pouch ileoanal anastomosis.

Familial Polyposis Coli / 대한소화기내시경학회지

Familial adenomatous polyposis(FAP) is the most oommon of the polyposis syndromes. It is characterized by multiple colorectal adenoma and a high occurrence rate of associated colonic adenocarcinoma in all untreated cases. FPC is inherited as antosomal dominant trait. During 20 years after diagnosis, adenocarcinoma occurs in more than 50% of patients. Clinically, surgical resection should be advised at the time of diagnosis in almost all cases, and acquainted with the potential risks to their children and advised to seek genetic counselling. we report three cases with familial adenomatous polyposis in family.