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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.

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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.

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Ulcerative colitis (UC) is a chronic intestinal inflammatory disease and familial adenomatous polyposis (FAP) is an autosomal dominant inherited disease. Both diseases, despite being different, may require the same surgical procedure: proctocolectomy with ileal pouch-anal anastomosis (IPAA). The main complication after this procedure is pouch inflammation (pouchitis). This inflammatory complication can affect up to 60 percent of patients who receive IPAA for UC, and a very small percentage of the FAP patients. The purpose of this review was to determine the current molecular mechanisms in its pathogenesis and detail the risk factors involved in pouchitis, its diagnosis, and treatment.

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OBJECTIVE: To characterize a multi-institutional cohort of pediatric patients who underwent colectomy for familial adenomatous polyposis (FAP). STUDY DESIGN: In this retrospective cohort study, diagnosis and procedure codes were used to identify patients who underwent colectomy for FAP within the Pediatric Health Information System (PHIS). The inclusion criteria were validated at 3 children's hospitals and applied to PHIS to generate a cohort of patients with FAP between 2 and 21 years who had undergone colectomy between 2009 and 2019. Demographics, clinical and surgical characteristics, and endoscopic procedure trends as identified through PHIS are described. Descriptive and comparative statistics were used to analyze data. RESULTS: Within the PHIS, 428 pediatric patients with FAP who underwent colectomy were identified. Median age at colectomy was 14 years (range 2-21 years); 264 patients (62%) received an ileal pouch anal anastomosis and 13 (3%) underwent ileorectal anastomosis. Specific anastomotic surgical procedure codes were not reported for 151 patients (35%). Endoscopic assessment at the surgical institution occurred in 40% of the cohort before colectomy and in 22% of the cohort following colectomy. CONCLUSIONS: In this cohort, colectomy took place at an earlier age than suggested in published guidelines. Ileal pouch anal anastomosis is the predominant procedure for pediatric patients with FAP who underwent colectomy in US pediatric centers. Endoscopic assessment trends before and after surgery suggest that the surgical institution plays a limited role in the care of this population.

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PURPOSE: Ileal pouch-anal anastomosis (IPAA) has become the surgical procedure of choice for patients with ulcerative colitis (UC). IPAA was incorporated into our institution in 1984, and thereafter, more than 200 procedures have been performed. The functional results and morbidity of this surgery have been reported previously. However, long-term functional outcomes and quality of life have not been evaluated. METHODS: As a cohort study, we identified all consecutive patients who underwent IPAA for UC between 1984 and 2017 and selected those with more than 10-year follow-up. Demographic data, morbidity, and pouch survival information were obtained. Long-term functional results and quality of life were evaluated through an e-mail survey using the Öresland score and the Cleveland Global Quality of Life scales, respectively. RESULTS: Of 201 patients, 116 met the inclusion criteria. Median follow-up was 20 (10-34) years. Early post-operative complications (30 days) were observed in 19 (16.4%) patients and 66 (56.9%) presented adverse events. The IPAA preservation rate at 10 and 20 years was 96.5% and 93.1%, respectively. Long-term functional scores presented a median of 6 (1-15) points. IPAA function was satisfactory in 11 (20.0%) patients, acceptable in 18 (32.7%), and deficient in 26 (47.3%). The median score for global quality of life was 0.8 (0.23-1.0) points. CONCLUSION: IPAA as treatment for UC meets the expectations of cure of the disease, maintaining adequate long-term intestinal functionality associated with a good quality of life in most patients.

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INTRODUCTION: Patients with familial adenomatous polyposis (FAP) and ulcerative colitis (UC) commonly undergo restorative proctocolectomy with ileal-pouch anal anastomosis (RP-IPAA). We sought to describe patient characteristics and postoperative outcomes in this patient population. METHODS: Using the National Surgical Quality Improvement Program-Pediatric Participant Use Files from 2012 to 2015, children who were 6-18years old who underwent RP-IPAA for FAP or UC were identified. Postoperative morbidity, including reoperation and readmission were quantified. Associations between preoperative characteristics and postoperative outcomes were analyzed. RESULTS: A total of 260 children met the inclusion criteria, of which 56.2% had UC. Most cases were performed laparoscopically (58.1%), and the operative time was longer with a laparoscopic versus open approach (326 [257-408] versus 281 [216-391] minutes, p=0.02). The overall morbidity was 11.5%, and there were high reoperation and readmission rates (12.7% and 21.5%, respectively). On bivariate analysis, preoperative steroid use was associated with reoperation (22.5% versus 10.9%, p=0.04). On multivariable regression analysis, obesity was independently associated with reoperation (odds ratio: 3.34 [95% confidence intervals: 1.08-10.38], p=0.04). CONCLUSIONS: Children who undergo RP-IPAA have high rates of overall morbidity, reoperation, and readmission. Obesity was independently associated with reoperation. This data can be used by practitioners in the preoperative setting to better counsel families and establish expectations for the postoperative setting. TYPE OF STUDY: Retrospective Comparative Study. LEVEL OF EVIDENCE: Level III.

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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.

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PURPOSE: This study compared outcomes after laparoscopic (LAP) or conventional (open) total proctocolectomy with outcomes after ileal J-pouch anal anastomosis (IPAA) at a single institution. METHODS: Charts from 133 familial adenomatous polyposis patients (1997-2013) were reviewed. Demographic data (age, sex, color, American Society of Anesthesiologists [ASA] status, previous surgery, and body mass index) and surgical outcomes (length of stay, early and late morbidity, reoperation, and mortality rates) were compared among 63 patients undergoing IPAA. RESULTS: Demographic features were similar among patients (25 open and 38 LAP). Conversely, colorectal cancer at diagnosis prevailed in the open group (60% versus 31.6%; P = .02). Tumor stages (P = .65) and previous surgery index (20% versus 10.5%; P = .46) were similar. Surgical length was longer for LAP (374 versus 281 minutes, P = .003). Short-term complication rates (28% versus 28.9%), hospital stay (10.9 versus 8.9 days), and total long-term reoperations (28% versus 21%) were not statistically different. However, major late morbidity (16% versus 2.6%; P < .001) and late reoperation rates (16% versus 5.2%; P < .05) were greater among open patients. Both groups did not differ regarding pouch failure rates (8% versus 5.2%). There was no operative mortality in the present series. CONCLUSIONS: (1) LAP IPAA is a safe procedure associated with a low conversion rate, (2) short-term results showed no clear advantages for both approaches, and (3) a greater risk of major late complications and late reoperations should be expected after open procedures.

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Familial adenomatous polyposis (FAP) is an autosomal dominant inherited syndrome characterized by multiple adenomatous polyps (predisposing to colorectal cancer development) and numerous extracolonic manifestations. The underlying genetic burden generates variable clinical features that may influence operative management. As a precancerous hereditary condition, the rationale of performing a prophylactic surgery is a mainstay of FAP management. The purpose of the present paper is to bring up many controversial aspects regarding surgical treatment for FAP, and to discuss the results and perspectives of the operative choices and approaches. Preferably, the decision-making process should not be limited to the conventional confrontation of pros and cons of ileorectal anastomosis or restorative proctocolectomy. A wide discussion with the patient may evaluate issues such as age, genotype, family history, sphincter function, the presence or risk of desmoid disease, potential complications of each procedure and chances of postoperative surveillance. Therefore, the definition of the best moment and the choice of appropriate procedure constitute an individual decision that must take into consideration patient's preferences and full information about the complex nature of the disease. All these facts reinforce the idea that FAP patients should be managed by experienced surgeons working in specialized centers to achieve the best immediate and long-term results.

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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)

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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)

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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)

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