Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?

Abstract Objective: Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk patients and modification of operative strategy may be crucial. Aim: The objective of this study was to estimate the desmoid tumor risk in relation to various surgical and clinical variables. Methods: Patients who had undergone polyposis since 1958 were included in the study. After exclusion criteria were met, those who had developed desmoid tumors were selected to undergo further evaluation. Results: The study revealed that the risk of developing desmoid tumors was associated with various factors such as sex ratio, colectomy, and reoperations. On the other hand, the type of surgery, family history, and surgical approach did not affect the risk of developing desmoid tumors. The data collected from 146 polyposis patients revealed that 16% had desmoid polyps. The sex ratio was 7:1, and the median age at colectomy was 28.6 years. Family history, multiple abdominal operations, and reoperations were some of the characteristics that were common in desmoid patients. Conclusion: Recognition of clinical (female sex) and surgical (timing of surgery and previous reoperations) data as unfavorable variables associated with greater risk may be useful during the decision-making process.

Functional outcomes in patients submitted to restorative proctocolectomy with ileal pouch anal anastomosis in a single tertiary center / Resultados funcionais em pacientes submetidos a proctocolectomia restauradora com anastomose íleoanal e bolsa ileal em um centro terciário

ABSTRACT Background: Functional results after restorative proctocolectomy for ulcerative colitis and familial adenomatous polyposis are variable. We assessed functional results in patients with ileal pouch anal anastomosis and evaluated potential factors associated with poor functional results. Methods: Retrospective cohort study of 38 patients who were submitted to a restorative proctocolectomy with ileal pouch anal anastomosis, in the context of ulcerative colitis and familial adenomatous polyposis, in at tertiary referral center, in the period between 1993 and 2013. Clinical records were analyzed and telephone interviews with protocoled questionnaire to 32 patients (12 ulcerative colitis, 20 familial adenomatous polyposis) were performed. Pouch functional results were also evaluated based in the Oresland score. The functional results were analyzed at four points of the patient outcome. Results: In 25 patients were performed restorative proctocolectomy with ileal pouch anal anastomosis and in 7 patients total colectomy preceded protectomy with ileal pouch anal anastomosis. Protective ileostomy was performed in all patients. There was no mortality and post-operative complications related with the pouch was 12.5% but treated conservatively. The mean follow-up was 13.2 years. Pouch failure occurs in 9.4% (2 in familial adenomatous polyposis and 1 in ulcerative colitis). Familial adenomatous polyposis patients achieved the best outcome but the outcome was acceptable in both groups. The median Oresland score was good with small variations over the years, although the best score being reached at 5 years after the surgery. Conclusions: The long-term results in patients undergoing restorative proctocolectomy with ileal pouch anal anastomosis were good in both groups, although better in familial adenomatous polyposis. In both, the best score of functional results seems to be reached at 5 years after surgery.

RESUMO Introdução: Os resultados funcionais após proctocolectomia restauradora em casos de colite ulcerativa e polipose adenomatosa familiar são variáveis. Avaliamos os resultados funcionais em pacientes com anastomose ileoanal e bolsa ileal bem como os fatores potenciais associados a resultados funcionais fracos. Métodos: Estudo retrospectivo de coorte com 38 pacientes submetidos a proctocolectomia restauradora com anastomose ileoanal e bolsa ileal, no contexto de colite ulcerativa e polipose adenomatosa familiar, em um centro de referência terciário, no período entre 1993 e 2013. Analisamos os registos clínicos e realizamos entrevistas telefónicas com um questionário protocolado a 32 pacientes (12 colite ulcerativa, 20 polipose adenomatosa familiar). Também foram avaliados os resultados funcionais da bolsa, com base no escore de Oresland. Os resultados funcionais foram analisados em quatro pontos do desfecho de cada paciente. Resultados: Em 25 pacientes foi realizada proctocolectomia restauradora com anastomose ileoanal e bolsa ileal, e em 7 pacientes uma colectomia total precedeu a protectomia com anastomose ileoanal e bolsa ileal. Todos os pacientes foram submetidos a uma ileostomia protetora. Não ocorreram óbitos e as complicações pós-operatórias relacionadas com a bolsa chegaram a 12,5%, mas foram tratadas conservadoramente. O seguimento médio foi de 13,2 anos. Ocorreu defeito na bolsa em 9,4% (2 em polipose adenomatosa familiar e 1 em colite ulcerativa). Os pacientes com polipose adenomatosa familiar obtiveram o melhor resultado; contudo, em ambos os grupos o resultado foi considerado aceitável. A mediana do score de Oresland foi boa, tendo sido observadas pequenas variações ao longo dos anos, embora o melhor score tenha sido verificado 5 anos após a cirurgia. Conclusões: A longo prazo, os resultados para os pacientes submetidos a proctocolectomia restauradora com anastomose ileoanal e bolsa ileal foram bons em ambos os grupos, embora tenham sido considerados melhores nos pacientes com polipose adenomatosa familiar. Nos dois grupos, o melhor escore de resultados funcionais parece ser alcançado por volta dos 5 anos após a cirurgia.

Clinical efficacy of laparoscopic and open total proctocolectomy with ileal pouch-anal anastomosis for ulcerative colitis / 中华消化外科杂志

Objective To compare the short-term and long-term outcomes between laparoscopic and open total proctocolectomy with ileal pouch-anal anastomosis (IPAA) for ulcerative colitis (UC).Methods The retrospective cohort study was conducted.The clinical data of 150 UC patients who underwent total proctocolectomy with IPAA in the Renji Hospital of Shanghai Jiaotong University between January 2003 and December 2016 were collected.Among 150 patients,87 undergoing laparoscopic total proctocolectomy with IPAA and 63 undergoing open total proctocolectomy with IPAA were respectively allocated into the laparoscopy group and open group.Observation indicators:(1) comparisons of intra-and post-operative situations;(2) postoperative complications;(3) follow-up situation.Follow-up using outpatient examination and telephone interview was performed to detect postoperative recovery and complications up to December 2017.Measurement data with normal distribution were represented as x-±s and comparison between groups was analyzed using the t test.Comparison between groups of count data was analyzed using the chi-square test.Results (1) Comparisons of intra-and post-operative situations:operation time,volume of intraoperative blood loss,time of initial intestinal stoma exsufflation and duration of hospital stay were respectively (306±3) minutes,(197± 12) mL,(62.1±1.8) hours,(8.2±0.4) days in the laparoscopy group and (224±4) minutes,(308±24) mL,(75.6±2.0) hours,(10.1±0.6) days in the open group,with statistically significant difference between groups (t =16.23,4.33,5.03,2.61,P＜ 0.05).All patients discharged successfully from hospital.All the 150 patients underwent stoma reversion of ileum at 3-12 months postoperatively,and the average time in the laparoscopy group and open group was respectively (6.0±5.6) months and (6.0±4.6)months,with no statistically significant difference between groups (t =0.01,P＞ 0.05).(2) Postoperative complications:cases with postoperative wound infection,retention of urine and frequency of defecation ＞ 4 times / day were respectively 2,8,21 in the laparoscopy group and 8,15,29 in the open group,with statistically significant differences between groups (x2 =5.25,4.37,0.96,P＜0.05).The cases with postoperative intestinal obstruction,anastomotic leakage,pelvic infection,pouch infection,pouch-related Crohn's disease and hyperplasia of ileal pouch were respectively 3,10,5,23,2,1 in the laparoscopy group and 8,7,4,24,1,0 in the open group,with no statistically significant differences between groups (x2=3.65,0.11,0.01,0.96,0.17,0.82,P＞0.05).Patients with postoperative complications were improved by acid suppression,fasting,anti-infection and fluid infusion.(3) Follow-up situation:150 patients were followed up for 12-60 months,with a median time of 48 months.There was no abnormality of postoperative anastomotic stoma and intestinal mucosa through comparison of colonoscopy results between pre-operation and 5 year postoperatively.During the follow-up,50 patients had shapeless stool and irregular defecation (times ＞ 4 times / day) at 3 years after stoma reversion of small intestine bypass,including 21 in the laparoscopy group and 29 in the open group,with a statistically significant differences between groups (x2 =4.72,P＜0.05).Eleven and 10 patients in the laparoscopy group and open group had shapeless stool and irregular defecation at 5 years postoperatively,but status were improved compared with the preoperative status,with no statistically significant difference between groups (x2=0.32,P ＞ 0.05).Conclusion The security of laparoscopic total proctocolectomy with IPAA for UC is equivalent to that of open total proctocolectomy,with the better short-term and long-term outcomes.

Surgical strategies of critical ulcerative colitis / 中华消化外科杂志

Surgical treatment of ulcerative colitis (UC) has received increasing attention.Total proctocolectomy with ileal pouch anal anastomosis (IPAA) has become a standard procedure.Acute severe UC (ASUC) is a difficult point of treatment.The commonly used strategy is to determine whether conversion therapy is performed after the observation of hormone therapy.Critical UC (CUC) is more urgent and complex due to the critical condition of the disease,which is often combined with uncontrollable massive hemorrhage,toxic megacolon or colonic perforation.Therefore,higher requirements for surgical and perioperative management are necessary.At present,when surgeons face CUC patients,what surgical strategy should be adopted is still lack of unified standard.The authors intended to sort out the timing,operation procedure and postoperative management of CUC so as to give full play to the value of surgical treatment and to improve the prognosis of CUC patients.

Surgery for ulcerative colitis in the era of biological agents therapy / 中华消化外科杂志

Ulcerative colitis is an inflammatory disease of colon and rectum whose etiology is still unclear.Infliximab is an anti-tumor necrosis factor antibody,which has been approved recently by the United States FDA for the treatment of ulcerative colitis to reduce signs and symptoms,to induce clinical remission and healing of the intestinal mucosa.Total proctocolectomy with pouch-anal anastomosis are the standard operation for ulcerative colitis now.The perioperative infliximab use,operation timing and procedures are the important factors affecting prognosis of patients in the era of infliximab therapy.

Analysis of complications of total proctocolectomy with ileal pouch-anal anastomosis in treatment of ulcerative colitis / 中华消化外科杂志

Objective To analyze the complications of total proctocolectomy with ileal pouch-anal anastomosis (TPC-IPAA) in treatment of severe or refractory ulcerative colitis (UC).Methods The retrospective cross-sectional study was conducted.The clinical data of 67 patients with severe or refractory UC who underwent TPC-IPAA in the Xinhua Hospital Affiliated to Shanghai Jiaotong University from February 2008 to October 2015 were collected.All the patients received open and laparoscopy-assisted TPC-IPAA,and two-stage or three-stage surgery was performed according to the patients' conditions.Observation indicators:(1) treatment and follow-up situations,(2) short-term complications after TPC-IPAA,(3) long-term complications after TPC-IPAA.The follow-up using telephone interview and outpatient examination was performed to detect the quality of postoperative life in patients and occurrence of complications up to July 2016.Measurement data with skewed distribution were described as M (range).The comparison of quality of pre-and post-operative life in patients was done using the paired t test.Results (1) Treatment and follow-up situations:all the 67 patients received successful surgery,including 45 with two-stage surgery and 22 with three-stage surgery.Thirty-seven patients underwent open surgery and 30 underwent laparoscopic surgery.The average frequency of postoperative daily defecation,average scores of quality of pre-and post-operative lifes were 5.6,0.47 and 0.67,respectively,with a statistically significant difference between preoperative indicators and postoperative indicators (t =-4.80,P ＜ 0.05).All the 67 patients were followed up for a median time of 4.6 years (range,1.0-8.4 years).(2) Short-term complications after TPC-IPAA:Of 67 patients,10 had short-term complications (some patients with multiple complications).One patient was complicated with presacral abscess secondary to sinus formation and then was cured by topical incision of sinus under colonoscopy.Five patients were complicated with anastomotic site-related complications of TPCIPAA,and 1 with pouch-anal anastomotic fistula combined with presacral abscess underwent drainage with ileostomy and didn't undergo stoma reversion of ileum.Two patients with fistula at the top of pouch were improved by surgery and repair.Of 2 patients with pouch-vagina fistula,1 underwent resection of pouch stump-vagina fistula and then were cured,and the space between pouch stump and posterior fornix was filled with a free greater omentum flap and temporary ileal bypass was simultaneously conducted.The other patient received twice transanal vaginal fistula repairs with advancement flap,and unclosed fistula was confirmed by angiography,with a clinical symptomatic relief.Eight patients with wound infection received actively dressing change after discharge,and no wound dehiscence was occurred.(3) Long-term complications after TPC-IPAA:28 of 67 patients had long-term complications.Twelve patients with intestinal obstruction underwent conservative treatments of gastrointestinal decompression,anti-inflammatory with corticosteroid and anti-infection with antibiotic,without the occurrence of severe intestinal perforation and prognosis needing surgical intervention,including 9 undergoing open TPC-IPAA and 3 undergoing laparoscopic TPC-IPAA.Thirteen patients with postoperative pouchitis received the conservative treatment of mesalazine.Three patients with failed pouch underwent small intestine permanent colostomy,including 2 with secondary pouch Crohn's disease and 1 with severe pouchitis.Conclusions TPC-IPAA is safe and effective in treatment of UC,with a lower incidence of complications.Anastomotic site-related complication of TPC-IPAA is the main short-term complication.And in the long-term complications,pouchitis is the most,followed it is intestine obstruction.

The postoperative complications and nursing experience of hand assisted laparoscopic J-type ileal pouch-anal anastomosis in familial adenomatous polyposis / 中国实用护理杂志

Objective To investigate the postoperative complication and nursing experience of hand-assisted laparoscopic J-type ileal pouch-anal anastomosis (IPAA) in familial adenomatous polyposis (FAP). Methods A total of 36 patients with FAP who had undergone hand-assisted laparoscopic J-type ileal pouch- anal anastomosis (IPAA) were analyzed retrospectively. Results The postoperative complication included ileal pouch bleeding in 2 cases, urinary retention in 3 female patients, external hemorrhoids swelling in 3 cases, and anastomotic fistula in 2 cases. Six cases had ileum pouch inflammation during the postoperative follow- up. All the complications were relieved after conservative medical therapy. Conclusions IPAA operation puts forward high technical requirements with more postoperative complications. Therefore, it is important to strengthen nursing of the patients, reduce postoperative complication and increase quality of life of FAP.

Closure of leak at the tip of the "J" after ileal pouch-anal anastomosis using a novel over-the-scope clipping system / Fechamento de vazamento na extremidade de bolsa ileal em J após anastomose bolsa ileal-anal usando um novo sistema "over-the-scope" clip (OTSC)

OBJECTIVE: leaks from the body or the tip of the J-pouch can occur after restorative proctocolecotmy. Although it may follow an indolent clinical course, it often requires surgical repair or pouch revision. Here we describe a novel endoscopic approach to close the leak at the tip of the J pouch. DESIGN: pouchoscopy was performed under sedation and endoscopic over-the-scope clipping system was used for the management of a leak from the tip of a J-pouch. RESULTS: under sedation, a pouchoscopy was performed and the deep tip of "J" leak was detected with a guidewire, along with water-contrasted pouchogram. The contained cavity from the leak was cleaned with hydrogen peroxide and 50% dextrose. Debridement of the epithelialized mouth of the leak was performed with an endoscopic cytology brush. Then endoscopic over-the-scope clipping system was used and the leak defect was completely closed. There was no bleeding or perforation. The entire procedure took 25 min. The patient continued to do well and reported resolution of the low back pain symptom. CONCLUSION: we reported the first case in the literature that a leak from the tip of a J-pouch was endoscopically occluded using a novel over-the-scope clipping system. (AU)

OBJETIVE: vazamentos do corpo da bolsa e da extremidade da bolsa ileal em J podem ocorrer após proctocolectomia restauradora. Embora possam apresentar um curso clínico indolente, muitas vezes requerem procedimentos abdominais. DESENHO: endoscopia da bolsa ileal foi realizada sob sedação e clipagem endoscópica usando o sistema "over-the-scope" foi utilizada para fechar um vazamento na extremidade da bolsa ileal em "J". RESULTADOS: sob sedação, uma endoscopia de bolsa ileal foi realizada e a extremidade profunda do vazamento da bolsa ileal em "J" foi detectada com um fio-guia. A cavidade que continha o vazamento foi limpa com peróxido de hidrogênio e 50% de dextrose. Desbridamento da borda epitelizada do vazamento foi realizada com uma escova de citologia endoscópica. Então o sistema endoscópico "over-the-scope" clip foi usado e o vazamento foi completamente fechado. Não houve sangramento ou perfuração. O processo todo levou 25 minutos. O paciente evoluiu bem e relatou a resolução do sintoma de dor lombar. CONCLUSÃO: relatamos o primeiro caso na literatura de vazamento na extremidade de bolsa ileal em J que foi fechado através de procedimento endoscópico usando um novo sistema "over-the-scope" clip (OTSC). (AU)

Clinical value of hand-sewn ileal pouch anal anastomosis after proctocolectomy for the treatment of ulcerative colitis / 中华消化外科杂志

Objective To investigate the clinical value of hand-sewn ileal pouch anal anastomosis (HIPAA) for the treatment of ulcerative colitis.Methods The clinical data of 191 patients with ulcerative colitis who were admitted to the General Hospital of Tianjin Medical University from January 1989 to December 2013 were retrospectively analyzed.All the patients received proctocolectomy and open or laparoscopic HIPAA.Patients were followed up by questionnaire at postoperative month 3 and 12.The function of pouch was evaluated according to the defecation frequency per 24 hours and per night,Bristol scale stool form and Kirwan classification.The quality of life was assessed according to the Cleveland global quality of life (CGQL).Patients were followed up till April 2014.The independent samples were compared by t test,and data from multiple groups were compared by analysis of variance,pairwise comparison was done by LSD-t test,and the count data were analyzed using the chi-square test.Results Of the 191 patients,180 received open surgery and 11 received laparoscopic surgery.Nine patients were treated by one-stage surgery,175 by two-stage surgery and 7 by three-stage surgery.The pouches of 181 patients were J shape,9 were H shape and 1 was W shape.No patient died perioperatively.Eight patients had abdominal infection and 7 had incisional infection at early period after the operation,and they were cured by antibiotics.Four patients had pouch anastomotic bleeding and 4 had anastomotic leakage at postoperative week 1-2,and they were cured by local hemostasis or titanium clipping.Six patients had high intestinal obstruction,and they were alleviated by symptomatic treatment.Eight patients had inflammation of pouch,and they were alleviated by diet adjustment or antibiotics treatment.Two patients had sexual dysfunction.All the patients were followed up for 1-25 years.The function of the pouch was improved gradually.At postoperative month 12,the defecation frequencies were (3.7 ± 1.4)/24 hours and (1.3 ±0.5)/night,and the number of patients with type Ⅳ (Bristol classification),type Ⅴ,type Ⅵ and in grade Ⅰ (Kirwan classification),grade Ⅱ,grade Ⅲ were 107,76,8 and 177,8,6,respectively,which were significantly improved when compared with (6.5 ±2.8)/24 hours,(2.9 ± 1.5)/night,86,89,16 and 160,19,12 at postoperative month 3 (t =12.36,3.98,x2=7.76,29.27,P＜ 0.05).The CGQL indexes before operation and at postoperative month 3 and 12 were 0.37 ±0.19,0.67 ±0.16 and 0.82 ±0.13,respectively,with significant differences (F =6.011,P ＜0.05).There were significant differences between the CGQL indexes before operation and at postoperative 12 and 3 months (t =16.69,10.06,P ＜ 0.05).A significant difference was also observed between the CGQL indexes before operation and at postoperative 12 months (t =27.01,P ＜ 0.05).Conclusions HIPAA is suitable for the surgical treatment of ulcerative colitis.The function of the pouch is good and the quality of life is improved.

Endoscopic management of anastomotic bleeding in the ileal pouch with staples removal and clipping / Abordagem endoscópica do sangramento anastomótico da bolsa ileal com a remoção dos grampos e clipagem

OBJECTIVE: Postoperative pouch bleeding is a rare but detrimental complication following ileal pouch surgery. It is usually self-limited, however continuous bleeding requires intervention. There is limited published data on its management. DESIGN: Ileoscopy via stoma for loop ileostomy and pouchoscopy via anus for ileal pouch were performed under sedation for the purpose of diagnosis and management of postoperative bleeding. RESULTS: Ileoscopy demonstrated a large, long blood clot in the lumen of efferent limb, but no sign of active bleeding was identified. Pouchoscopy showed that lumen of pouch body as well as afferent limb was filled with maroon-colored liquid stool. Pouch and neo-terminal ileum mucosa was normal. Two dislodged staples at the anastomotic line with sharp tips towards the lumen were found, with activating bleeding at one site. The staples were removed by biopsy forceps, and active bleeding was successfully controlled by the deployment of one endoclip. CONCLUSIONS: We reported the first case that postoperative pouch bleeding, which was caused by dislodged staples, was successfully managed by endoscopic removal of the staples combined with clipping. (AU)

OBJETIVO: O sangramento pós-operatório da bolsa ileal é uma complicação rara, mas prejudicial após abordagem cirúrgica da bolsa ileal. Esse sangramento é geralmente autolimitado, porém, requer intervenção quando contínuo. Não há dados publicados sobre o tratamento. MÉTODO: Ileoscopia através de estoma para ileostomia em alça e endoscopia via ânus para a bolsa ileal foram realizadas sob sedação para diagnóstico e tratamento do sangramento pós-operatório. RESULTADOS: A ileoscopia demonstrou um grande e longo coágulo sanguíneo no lúmen do ramo eferente, mas nenhum sinal de sangramento ativo foi identificado. A endoscopia da bolsa ileal mostrou que os lumens do corpo da bolsa e ramo aferente estavam cheios de fezes líquidas de cor marrom. A bolsa e a mucosa do íleo neoterminal estavam normais. Dois grampos deslocados na linha da anastomose e com pontas afiadas em direção ao lúmen foram encontrados, com sangramento ativo em um dos locais. Os grampos foram removidos com pinça de biópsia e o sangramento ativo controlado com sucesso pela implantação de um endoclipe. CONCLUSÃO: Relatamos o primeiro caso em que o sangramento pós-operatório da bolsa ileal causado por grampos deslocados foi controlado com sucesso pela remoção endoscópica dos grampos combinada com clipagem. (AU)

Management of ileal pouch prolapse with endoscopic hot snare / Tratamento de prolapso da bolsa ileal com alca diatermica endoscopica

Pouch prolapse is a complication following the creation of restorative proctocolectomy. There is a paucity of information in the literature pertaining to its management. An ileal J pouch patient with dyschezia presented to our Pouch Center. Under sedation, pouchoscopy was performed with a gastroscope. We detected an anterior distal pouch mucosal prolapse, 1.5 cm in diameter, blocking the anal canal. The prolapsed mucosa was excised with hot snare under a retroflex view. There was no bleeding or perforation. The entire procedure took 25 minutes. The patient tolerated the procedure well and was discharged home 30 minutes after post-procedural observation. The patient reported the resolution of the dyschezia symptom. The histopathological examination of excised specimen showed small bowel mucosa and sub-mucosa with changes compatible with mucosal prolapse. Endoscopic hot snare appears to be feasible in the management of pouch mucosal prolapse. (AU)

O prolapso da bolsa ileal é uma complicação que pode surgir após a criação da proctocolectomia restauradora. As informações na literatura são escassas quanto ao tratamento. Um paciente com bolsa ileal em "J" e apresentando disquezia deu entrada em nosso centro médico. Sob sedação, realizamos uma endoscopia da bolsa ileal. Detectamos uma bolsa distal anterior com prolapso da mucosa, com 1,5 cm de diâmetro, bloqueando o canal anal. O prolapso da mucosa foi retirado com alça diatérmica sob visão retroflexa. Não houve sangramento ou perfuração. A duração de todo o processo foi de 25 minutos. O paciente tolerou bem o procedimento e recebeu alta após 30 minutos de observação pós-procedimento. O paciente relatou a resolução do sintoma de disquezia. O exame histopatológico do espécime extirpado mostrou a mucosa e submucosa do intestino delgado com alterações compatíveis com o prolapso da mucosa. A alça diatérmica endoscópica parece ser viável no tratamento de prolapso da mucosa da bolsa ileal. (AU)

Early postoperative and long-term oncological outcomes of laparoscopic treatment for patients with familial adenomatous polyposis / 대한외과학회지

PURPOSE: We evaluated the short- and long-term outcomes of laparoscopic total proctocolectomy with ileal pouch-anal anastomosis (TPC/IPAA) for treatment of familial adenomatous polyposis (FAP). Also, we assessed the oncologic outcomes in FAP patients with coexisting malignancy. METHODS: From August 1999 to September 2010, 43 FAP patients with or without coexisting malignancy underwent TPC/IPAA by a laparoscopic-assisted or hand-assisted laparoscopic surgery. RESULTS: The median age was 33 years (range, 18 to 58 years) at the time of operation. IPAA was performed by a hand-sewn method in 21 patients (48.8%). The median operative time was 300 minutes (range, 135 to 610 minutes), which reached a plateau after 22 operations. Early postoperative complications within 30 days occurred in 7 patients (16.3%) and long-term morbidity occurred in 15 patients (34.9%) including 6 (14.0%) with desmoid tumors and 3 (7.0%) who required operative treatment. Twenty-two patients (51.2%) were diagnosed with coexisting colorectal malignancy. The median follow-up was 58.5 months (range, 7.9 to 97.8 months). There was only 1 case of local recurrence in the pelvic cavity. No cases of adenocarcinoma at the residual rectal mucosa developed. 5-year disease-free survival rate for 22 patients who had coexisting malignancy was 86.5% and 5-year overall survival rate was 92.6%. Three patients died from pulmonary or hepatic metastasis. CONCLUSION: Laparoscopic TPC/IPAA in patients with FAP is feasible and offers favorable postoperative outcomes. It also delivered acceptable oncological outcomes in patients with coexisting malignancy. Therefore, laparoscopic TPC/IPAA may be a favorable treatment option for FAP.

Study of anal function after ileal pouch anal anastomosis with modified double-stapled mucosectomy / 国际外科学杂志

Objective To evaluate the long-term functional outcome after ileal pouch anal anastomosis with modified double-stapled technique.Methods From January 2002 to March 2011,forty-five patients underwent ileal pouch anal anastomosis with modified double-stapled technique.The clinical data of these patients were reviewed.The postoperative anal function was assessed by Kirwan classification and Oresland pouch-specific function score.Results During the median follow-up of 65 months,2 patients with malignant adenomatous polyps died,2 patients were diagnosed dysplasia by biopsy,4 patients developed mild to moderate anastomotic narrowing,1 patient developed persistent anastomotic stricture needing surgical intervention,16 patients developed at least 1 episode of pouchitis.There was no incontinence in these patients,and the median functional Oresland score was 6,3 and 2 after 1 year,2.5 years and 5 years respectively.Conclusion The functional results of ileal pouch anal anastomosis with modified double-stapled technique are promising,with no incontinence in our patients.

Recovery from Recurrence of Post-operative Pouchitis Due to Ulcerative Colitis through Daitokato / 日本東洋医学雑誌

Pouchitis is the most common complication following ileal pouch-anal anastomosis in patients with ulcerative colitis and is strongly correlated with the risk of pouch failure and malignant mucosal transformation in the pouch. Here we report a case treated successfully with the Kampo decoction, daitokato. A 41-year-old male treated with ileal pouch-anal anastomosis due to severe ulcerative colitis suffered from repeated pouchitis and stenosis of the ileal pouch post-operatively. He complained of general fatigue, chills, continuous lower abdominal pain and discomfort, and severe pain with evacuation. Initial treatment with antibiotics and painkillers was ineffective, so the patient came to our hospital's Kampo clinic, where he was initially prescribed the decoction, shokenchuto. This proved ineffective at resolving his fatigue. Subsequent treatment with ogikenchuto improved physical strength but was ineffective for pouchitis. Antibiotic resistant pouchitis occurred afterwards and we attempted treatment with saikokeishito decoction and the extracts, hochuekkito and juzentaihoto, which similarly improved fatigue but not pouchitis. Following administration of daitokato, instances of pouchitis were reduced with no recurrence 6 months post-treatment. This case illustrates the therapeutic efficacy of daitokato in improving ileal pouch disorders resistant to treatment with Western medicine.

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.

The Correlation between Quality of Life and Functional Outcome after Restorative Proctocolectomy and Ileal Pouch-Anal Anastomosis

PURPOSE: The restorative proctocolectomy (RP) and ileal pouch-anal anastomosis (IPAA) has been accepted as the operation of choice for chronic ulcerative colitis (CUC) and familial adenomatous polyposis (FAP). However, much is still unknown about the functional outcome and the quality of life (QoL) in patients undergoing RP. The aims of this study were to evaluate the functional outcome and the QoL in patients undergoing RP and to assess the correlation between functional outcome and QoL. METHODS: The medical records of 20 patients who had undergone a RP for CUC (n=11) and FAP (n=9) from January 1993 to December 2003 were reviewed. The QoL was evaluated with the Korean version of Short Form 36 (SF-36) by telephone interview. The functional outcome was assessed by means of the Global Assessment of Function Scale (GAFS). The QoL of patients was compared with that of the general population matched for age and gender (n=107) with including the 7 men, the median age will be thought to be that of the 13 women. RESULTS: There were 7 men (35%) and 13 women (65%), and the median age was 46 years. The QoL in patients undergoing RP was comparable to that of the healthy general population on all scales. The Physical Component Summary (PCS) was correlated significantly with the daytime and the nighttime incontinence (P<0.01). The Mental Component Summary (MCS) was correlated significantly with the daytime and the nighttime incontinence and the daytime bowel movement (P<0.05). CONCLUSIONS: This study demonstrates that the QoL in patients undergoing RP is excellent and that the daytime incon tinence and the nighttime incontinence are significant factor influencing the QoL.

The Clinical Results of a Total Proctocolectomy with an Ileal Pouch-Anal Anastomosis: 12 Cases

PURPOSE: A restorative proctocolectomy has been accepted as the operation of choice for ulcerative colitis and familial adenomatous polyposis. The purpose of this study was to assess the postoperative complications and functional outcomes following a total proctocolectomy with a J ileal pouch-anal anastomosis. METHODS: The medical records of 12 patients who had undergone a total proctocolectomy, with a J ileal pouch-anal anastomosis, between January 1997 and June 2002, were retrospectively reviewed according to sex, age, underlying disease and postoperative complications. We evaluated the functional outcomes using medical record reviews and patients and telephone interviews. RESULTS: Total proctocolectomy, with a J ileal pouch-anal anastomosis, were done for ulcerative colitis (n=2) and familial adenomatous polyposis (n=10). A diverting ileostomy was performed in 8 patients. Postoperative complications occurred in 7 patients (58%), intestinal obstructions in 4 and complications related with anastomosis in 3, i.e. J ileal pouch leakage (n=2) and ileal pouch-vaginal fistula (n=1). Re-operations, due to postoperative complications, were performed in 4 patients, i.e. small bowel segmental resection (n=1), adhesiolysis (n=1), diverting ileostomy (n=1) and ileal pouch resection & reconstruction (n=1). The daily median defecation frequencies were 7.7 (range 4~20) a month after the operation, 5.4 (3~12) at 2~3 months, 4.5 (3~7) at 6 months and 4.1 (3~5) at 12 months, following the operation. Two patients had gas incontinence, 1 had fluid incontinence, 4 had night soiling and 3 needed pads, but these incontinences, the need for anti-diarrhea medication and the use of pads, all improved within 6 months of the operation. Fluid incontinence and the use of pads improved within 3 months of the operation, gas incontinence and night soiling improved within 6 months of the operation. The mean length of follow-up was 30.6 months. CONCLUSIONS: The postoperative complication rate was 58%. Thirty-three percent of patients had fecal incontinence, but all these improved within 6 months. The long- term functional outcomes, after a total proctocolectomy with J ileal pouch-anal anastomosis, were satisfactory, and the postoperative complications acceptable. The postoperative complication rates were no different between the protective diverting ileostomy and non-ileostomy .

A Case of Turcot's Syndrome Combined with Colon Cancer in a 15-year-old Girl

Turcot's syndrome is a rare hereditary disease marked by the association of central nervous system neuroepithelial tumor with colonic polyposis. Authors report herein a case of a 15-year-old girl diagnosed as having Turcot's syndrome, otherwise known as brain tumor-polyposis syndrome, combined with sigmoid colon cancer. The patient was carried out craniostomy and brain tumor removal. The tumor was confirmed histologically to be oligodendroglioma. The patient visited the department of internal medicine for bloody diarrhea during 6 months. Colonoscopy and biopsy was done. The patient was diagnosed as having Turcot's syndrome combined with sigmoid colon cancer, and was then transferred to the department of surgery for treatment of sigmoid colon cancer. Total proctocolectomy and IPAA (ileal pouch-anal anastomosis) was carried out. Multiple polyps were found in the colon, two large masses were confirmed histologically to be adenocarcinoma. The remaining polyps were adenomas. This case report describes the characteristic features of Turcot's syndrome presented by this patient.

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.

Manometric Assessment after Ileal Pouch- Anal Anastomosis

PURPOSE:Functional derangement in bowel movement after ileal pouch-anal anastomosis (IPAA) is not infrequent. It results from several mechanisms mainly decreased rectal reservoir capacity and rectal sensation. Anal sphincter or pelvic nerve damage during surgery contributes physiological changes, also. This study was performed to evaluate manometric changes after IPAA and compare them with functional outcomes regarding anastomotic technique. METHODS:Forty seven (M:F=23:24) patients who underwent IPAA and manometric assessment were enrolled. Pathological diagnoses of them were 32 ulcerative colitis, 12 familial adenomatous polyposis, and 3 hereditary non-polyposis colorectal cancer. Every pouch was constructed in J shape, 15cm length. Pouch-anal anastomosis was performed by 27 hand-sewn and 20 double stapling technique. Diverting ileostomy was performed in 30 cases (64%) and closed 2-3 months after IPAA. Manometry was performed preoperatively and 3 to 6 months interval, postoperatively. Twenty two patients underwent full manometic assessment pre- and post-operatively. The others did it either pre or postsoperatively. Functional outcome was investigated at the median follow-up period 25 (2-54) months. Statistical analysis was performed by using Chi- square and Fisher's exact test. Significance was assigned to a P value of 0.05). Postoperative stool frequency and incontinence rate were not different between two groups. Thirty one percent of patients revealed night time seepage. MRP of this seepage group was significantly lower than the other group (67.9 vs. 48.4 mmHg; P=0.038). CONCLUSIONS:Characteristic changes of manometric findings after IPAA were summarized as decrease of MRP and disappearance of RAIR. Rectal compliance was significantly improved since 6 months after IPAA or ileostomy closure. Decrease of MRP was more remarkable in hand-sewn group. However, we could not find any difference in functional outcomes between two anastomotic techniques. MRP was a crutial factor for postoperative seepage.