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

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.

Ileal reservoir-associated complications in ulcerative colitis versus familial adenomatous polyposis: Impact on patient quality of life. / Complicaciones asociadas al reservorio ileal en colitis ulcerativa versus poliposis familiar adenomatosa: Impacto en la calidad de vida del paciente.

BACKGROUND: Proctocolectomy with ileal reservoir is the surgical procedure of choice for patients with ulcerative colitis and familial adenomatous polyposis. OBJECTIVES: To evaluate long-term postoperative complications (1994-2019) in patients operated for familial adenomatous polyposis (FAP) and ulcerative colitis (UC) and the degree of satisfaction with the procedure. METHODS: Observational study based on the analysis of a retrospective database with prospective follow-up in 115 consecutive patients: 79 with UC and 36 with FAP. A total of 88 patients were followed up, 60 with UC and 28 with PFA. RESULTS: 48 males (54.4%) with a mean age of 44.8 ± 10.6 years were evaluated. Indications for surgery were intractable disease in 54 patients (47%), dysplasia/cancer in 43 (37%), severe bleeding in 4 (4%) and perforation in 3 (3%). A proctectomy and mucosectomy of the rectal stump was performed in 67 (76.1%), and a double stapling technique in 21. A protective ileostomy was performed in all patients with UC and FAP. No differences were found in early complications between the two groups. Late complications showed a higher rate of reservoritis in UC patients compared to FAP (44.9 vs. 14.3%, p = 0.001), with more refractory reservoritis in the UC group (13.3 vs. 0%, p = 0.04) with no differences in bowel obstruction, strictures, or anastomotic fistulas. Overall satisfaction and adaptation were considered good in 87% of UC patients and only 57% in the FAP group (p < 0.01). CONCLUSIONS: Proctocolectomy with ileal reservoir has comparable morbidity and mortality, except for the higher rate of reservoritis in patients with a history of UC, despite this contingency there is a better quality of life and greater acceptance of surgery in UC patients than in FAP patients.

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.

Reservoritis secundaria a infección por citomegalovirus: reporte de caso y revisión de la literatura / Pouchitis secondary to cytomegalovirus infection: Case report and literature review

Resumen La proctocolectomía total con reservorio ileal es el procedimiento quirúrgico de elección para la colitis ulcerativa refractaria (CUR) al tratamiento médico y la reservoritis es la complicación más frecuente y puede afectar hasta al 50% de los pacientes en los primeros 5 años del procedimiento. Aunque la etiología no está bien establecida, su presentación podría estar relacionada con disbiosis como resultado de la estasis fecal en individuos genéticamente susceptibles y con una respuesta inmunitaria alterada. Los síntomas típicos de reservoritis como diarrea, dolor abdominal, tenesmo, urgencia, incontinencia fecal y, menos frecuentemente, sangrado rectal no son específicos y el diagnóstico debe confirmarse mediante una evaluación endoscópica e histológica. La infección por citomegalovirus es infrecuente como causa de reservoritis; sin embargo, debe considerarse en pacientes con reservoritis refractaria al manejo antibiótico inicial. Las pruebas diagnósticas incluyen pruebas serológicas como la medición de anticuerpos, antigenemia y proteína C-reactiva (PCR) en sangre. El tratamiento de elección es el ganciclovir, medicamento de administración endovenosa que puede inducir complicaciones graves como mielosupresión, neutropenia y trombocitopenia. Se recomienda el seguimiento endoscópico posterior al tratamiento para asegurar la cicatrización mucosa, especialmente cuando hay sospecha de enfermedad de Crohn o compromiso del asa aferente en la endoscopia inicial.

Abstract Total proctocolectomy with ileal pouch is the surgical procedure of choice for ulcerative colitis refractory to medical treatment, and pouchitis is the most frequent complication. It can affect up to 50% of patients in the first five years of the procedure. Although the etiology is not well established, its manifestation could be related to dysbiosis resulting from fecal stasis in genetically susceptible individuals with altered immune responses. Typical symptoms of pouchitis, such as diarrhea, abdominal pain, tenesmus, urgency, fecal incontinence, and, less commonly, rectal bleeding, are nonspecific, and the diagnosis must be confirmed by endoscopic and histologic examination. Cytomegalovirus infection is an infrequent cause of pouchitis; however, it should be considered in patients with pouchitis refractory to initial antibiotic management. Diagnostic tests include serological tests such as the measurement of antibodies, antigenemia, and C-reactive protein (CRP) in blood. The treatment of choice is ganciclovir, an intravenous drug that can induce severe complications such as myelosuppression, neutropenia, and thrombocytopenia. Post-treatment endoscopic follow-up is recommended to ensure mucosal healing, especially when there is suspicion of Crohn's disease or involvement of the afferent loop on initial endoscopy.

Pouchitis: insight into the pathogenesis and clinical aspects.

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.

Characterizing Pediatric Familial Adenomatous Polyposis in Patients Undergoing Colectomy in the United States.

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.

Ileal pouch-anal anastomosis in ulcerative colitis: outcomes, functional results, and quality of life in patients with more than 10-year follow-up.

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.

Short-term outcomes in children undergoing restorative proctocolectomy with ileal-pouch anal anastomosis.

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.

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.

Laparoscopic Versus Open Restorative Proctocolectomy for Familial Adenomatous Polyposis.

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.

Surgical treatment of familial adenomatous polyposis: dilemmas and current recommendations.

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.

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)

The epidemiological and clinical features of familial adenomatous polyposis in Ribeirão Preto / Caracteristicas clínicas e epidemiológicas da polipose adenomatosa familiar em Ribeirão Preto

PURPOSE: to study 75 familial adenomatous polyposis (FAP) patients treated in a single institution in Ribeirão Preto/SP, from January 1981 to December 2011. METHODS: this is a retrospective study and the following data were collected: gender, age, main symptoms, familial history, coexisting malignancies, surgical treatment, surgical morbidity and mortality, factors related to life quality. RESULTS: median age was 29 years. Male-to-female ratio was 1.2:1. Bleeding was the most common symptom (62.6%). Colorectal cancer incidence was 25.5% (n = 19). Extracolonic neoplasia incidence was 8%. Colectomy with ileorectal anastomosis (IRA) was performed in 72% of the patients. Eighteen patients (24%) were submitted to proctocolectomy with "J-pouch" ileoanal anastomosis. In three patients (4%) proctocolectomy with terminal ileostomy was performed. Early and late complication rate were similar (22.7% × 24%). Ileal pouch surgery exhibited tendency to a higher morbidity and mortality but no significance could be found. Overall mortality rate was 7.46%. Malignant neoplasia was the main cause of mortality, accounting for 60% of deaths. CONCLUSION: FAP is a rare pathology in our country. Genetic counseling and proper screening programs are essential tools to early diagnosis and follow-up. Surgery is the most effective treatment and the best option to prevent malignant neoplasia. (AU)

OBJETIVO: analisar 75 pacientes com polipose adenomatosa familiar (PAF) tratados no Hospital das clínicas da Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, entre janeiro de 1981 a dezembro de 2011. MATERIAIS E MÉTODOS: trata-se de estudo retrospectivo com coleta dos seguintes dados: sexo, idade, sintomas principais, história familiar, presença de malignidade, cirurgia realizada, morbidade e mortalidade cirúrgicas e fatores relacionados à qualidade de vida. RESULTADOS: a idade média encontrada foi de 29 anos. A razão entre os sexos foi de 1,2:1 com predomínio no sexo masculino. Sangramento intestinal foi o sintoma mais comum (62,7%). A incidência de câncer colorretal foi de 25,3% (n = 19). Neoplasias extracolônicas foram diagnosticadas em 8% dos pacientes. Colectomia total com íleo-reto anastomose (IRA) foi realizada em 72% (n = 54) dos pacientes. Proctocolectomia com anastomose ileoanal e bolsa ileal em "J" foi realizada em 24% (n = 18) dos casos e em 4% (n=3) dos pacientes optou-se pela proctocolectomia com ileostomia terminal (PCI). As taxas de complicações precoces e tardias foram semelhantes (22,7% × 24%). A cirurgia de bolsa ileal apresentou tendência a maior morbimortalidade, porém sem relevância estatística. A taxa geral de mortalidade foi de 7,46%. Neoplasias malignas foram responsáveis por 60% dos óbitos e complicações cirúrgicas por 40%. CONCLUSÕES: a PAF é uma patologia de baixa incidência no nosso país. O aconselhamento genético e o rastreamento familiar são instrumentos essenciais para o diagnóstico precoce e seguimento adequado. A cirurgia persiste como melhor opção para prevenção do câncer colorretal e tratamento da doença. (AU)

Quality of life in patients with ileal pouch for ulcerative colitis / Qualidade de vida em portadores de reservatório ileal por retocolite ulcerativa

INTRODUCTION: proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the standard surgical procedure for the treatment of ulcerative colitis (UC) and is associated with the prospect of cure. Experience gained over the years has demonstrated the occurrence of a high number of complications as well as bowel disorders that can compromise quality of life (QoL). OBJECTIVE: evaluate QoL in patients with IPAA for ulcerative colitis. PATIENTS AND METHODS: the Inflammatory Bowel Disease Questionnaire (IBDQ) was used to assess QoL in patients with IPAA after its validation in Portuguese. RESULTS: thirty-one patients submitted to IPAA by the same group of professionals were evaluated. QoL was classified as regular in all domains evaluated (intestinal and systemic symptoms and emotional and social aspects). There were no differences in relation to gender, type of pouch or postoperative time. However, elderly patients showed a tendency toward lower scores. Having a professional activity was associated with higher scores in systemic symptoms and social aspects (p < 0.05). Patients with ileostomy showed lower values in the domains of systemic symptoms, emotional and social aspects (p <0.05). CONCLUSION: in all domains assessed, patients with IPAA for UC had QoL classified as regular. Ileostomy and lack of professional activity negatively influenced QoL. (AU)

OBJETIVO: Avaliar a qualidade de vida em portadores de RI por RCUI. PACIENTES E MÉTODOS: Foi empregado IBDQ, validado em português na avaliação da QoL em portadores de RI. RESULTADOS: Foram avaliados 31 pacientes submetidos a RI pelo mesmo grupo. QoL foi classificada como regular em todos os domínios avaliados (sintomas intestinais e sistêmicos e aspectos emocionais e sociais). Não houve diferenças em relação ao sexo, tipo de reservatório ou tempo de pós-operatório. Entretanto, pacientes idosos apresentaram uma tendência a escores mais baixos. Atividade profissional relacionou-se com escores mais altos em sintomas sistêmicos e aspectos sociais (p < 0,05). Portadores de ileostomia apresentaram valores mais baixos nos domínios sintomas sistêmicos, aspectos emocionais e sociais (p < 0,05). CONCLUSÃO: Em todos os domínios avaliados, portadores de RI por RCUI apresentaram QoL classificada como regular. Ileostomia e inatividade profissional influenciaram negativamente a QoL. (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)

Current trends regarding protective ileostomy after restorative proctocolectomy / Tendências atuais sobre ileostomia protetora após proctocolectomia restauradora

The decision to perform a protective ileostomy after ileoanal-pouch anastomosis is controversial, and most of the discussion is based on its advantages and disadvantages. Although a temporary intestinal diversion has been routinely indicated in most patients, this choice is also associated with complications. The present work aims to review the outcomes after restorative proctocolectomy with or without a protective ileostomy in the treatment of ulcerative colitis and polyposis syndromes. Most papers emphasize that diversion protects against anastomosis leaks; consequently, it may prevent pelvic sepsis and pouch failure. Otherwise, a defunctioning ileostomy may cause morbidity such as dehydration, electrolyte imbalance, psychological problems, skin irritation, anastomosis strictures and intestinal obstruction, among others. There are those who believe that the omission of an ileostomy after the confection of ileal pouches should be reserved for selected patients, with quite acceptable results. The selection criteria should include surgeon, patient and procedure features to ensure a good outcome. (AU)

A decisão de realizar ileostomia de proteção após anastomose da bolsa ileal ao canal anal é controversa, sendo a discussão baseada em suas vantagens e desvantagens. Embora a derivação intestinal temporária tenha sido indicada rotineiramente na maioria dos pacientes, essa escolha também está associada a complicações. O presente trabalho teve como objetivo rever os resultados após proctocolectomia restauradora com ou sem ileostomia de proteção no tratamento da colite ulcerativa e síndromes polipoides. Muitos trabalhos enfatizam que a derivação protege contra fístulas anastomóticas; consequentemente, ela pode prevenir sepse pélvica e perda da bolsa. Por outro lado, a derivação por ileostomia pode ser causa de morbidade como desidratação, distúrbios eletrolíticos, problemas psicológicos, lesões dérmicas, estenose de anastomose e obstrução intestinal, entre outras. Há aqueles que acreditam que a omissão de ileostomia após a confecção de bolsa ileal deve ser reservada a pacientes selecionados, obtendo-se resultados aceitáveis. Os critérios de seleção devem incluir características do cirurgião, do paciente e do procedimento na tentativa de se obter bons resultados. (AU)

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)

Histopathological evaluation and risk factors related to the development of pouchitis in patients with ileal pouches for ulcerative colitis

OBJECTIVE: Many changes in mucosal morphology are observed following ileal pouch construction, including colonic metaplasia and dysplasia. Additionally, one rare but potential complication is the development of adenocarcinoma of the reservoir. The aim of this study was to evaluate the most frequently observed histopathological changes in ileal pouches and to correlate these changes with potential risk factors for complications. METHODS: A total of 41 patients were enrolled in the study and divided into the following three groups: a non-pouchitis group (group 1) (n = 20; 8 males; mean age: 47.5 years) demonstrating optimal outcome; a pouchitis without antibiotics group (group 2) (n = 14; 4 males; mean age: 47 years), containing individuals with pouchitis who did not receive treatment with antibiotics; and a pouchitis plus antibiotics group (group 3) (n = 7; 3 males; mean age: 41 years), containing those patients with pouchitis who were administered antibiotics. Ileal pouch endoscopy was performed, and tissue biopsy samples were collected for histopathological analysis. RESULTS: Colonic metaplasia was found in 15 (36.6%) of the 41 patients evaluated; of these, five (25%) were from group 1, eight (57.1%) were from group 2, and two (28.6%) were from group 3. However, no correlation was established between the presence of metaplasia and pouchitis (p = 0.17). and no differences in mucosal atrophy or the degree of chronic or acute inflammation were observed between groups 1, 2, and 3 (p>0.45). Moreover, no dysplasia or neoplastic changes were detected. However, the degree of mucosal atrophy correlated well with the time of postoperative follow-up (p = 0.05). CONCLUSIONS: The degree of mucosal atrophy, the presence of colonic metaplasia, and the degree of acute or chronic inflammation do not appear to constitute risk factors for the development of pouchitis. Moreover, we observed that longer postoperative follow-up times were associated with greater degrees of mucosal atrophy.

Resultados funcionales del reservorio ileal / Functional results of ileal pouch

Background: Proctocolectomy with ileal pouch and posterior pouch anal anastomosis is the treatment of choice of severe ulcerative colitis and polyposis. Aim: To evaluate the functional results of ileal pouch anal anastomosis. Material and Methods: Retrospective assessment of all patients subjected to an ileal pouch anal anastomosis. Pouch function was evaluated using Oresland score that goes from 0 or perfect to 15 or bad. Results: During a 10 years period, 35 patients were operated. Ten patients aged 19 to 67 years were treated for an ulcerative colitis and nine, aged 18 to 54 years for a familial adenomatous polyposis. Global complication rate of the procedure was 20 percent. Late complication rate was 17 percent. Specific complication rate of the rectal- ileal pouch anastomosis was 6 percent. Specific late complications of the technique were only observed in six of 20 patients with ulcerative colitis. Pouch failure was observed in two patients (6 percent). Global Oresland score was 3.2. Seventeen of 22 patients with ulcerative colitis and seven of eight patients with polyposis had a satisfactory or acceptable score. Patients had a mean of 4.4 bowel movements per day (range 1 to 15) without differences according to the underlying disease. Conclusions: Eighty percent of patients subjected to an ileal pouch anal anastomosis reported an Oresland score of less than seven, that is satisfactory in terms of quality of life.

Introducción: La proctocolectomía con reservorio ileal y anastomosis reservorio-anal (RIARA) es el tratamiento quirúrgico de elección en la colitis ulcerosa grave y las poliposis. Objetivo: Evaluar resultados funcionales de la RIARA. Pacientes y Método: Evaluación retrospectiva de todos los pacientes intervenidos por una RIARA en forma consecutiva. Los resultados funcionales se evaluaron mediante entrevista según el escore de Õresland. Resultados: En un período de 10 años se intervinieron 35 pacientes, 26 por colitis ulcerosa (CU) (edad promedio 36,8; extremos 19-67) y 9 por poliposis adenomatosa familiar (PAF) (edad promedio 38,8; extremos 18-54). La morbilidad global de la RIARA alcanza al 20 por ciento y las complicaciones tardías se elevan al 17 por ciento. La morbilidad específica de la RIARA fue 5,6 por ciento. Las complicaciones tardías específicas de la técnica se presentaron exclusivamente en el grupo CU (6/30=20 por ciento) y la falla del reservorio alcanza al 5,7 por ciento (2 casos). El puntaje global de Õresland fue 3,2, sin diferencias entre ambos grupos. Hubo 17 de 22 (77 por ciento) pacientes con un puntaje satisfactorio o aceptable en el grupo CU y 7 de 8 (88 por ciento) en el grupo PAF. El promedio de evacuaciones en 24 horas fue 4,4 (extremos 1-15) en la serie global, sin diferencias entre ambos grupos. Conclusión: El 80 por ciento de los pacientes sometidos a una RIARA tienen un escore menor de 7 en la escala de Õresland, lo que equivale a una calidad de vida similar a la población general. Aunque los resultados funcionales de la RIARA no son perfectos, la calidad de vida y la satisfacción del paciente son satisfactorias.

Processo inflamatório em mucosa de reservatórios ileais de doentes assintomáticos / Inflammatory activity of pelvic ileal pouches mucosa in asymptomatic patients

A inflamação inespecífica do reservatório ileal (RI) após retocolectomia total é a complicação pós-operatória mais comum nos doentes operados por retocolite ulcerativa inespecífica (RCUI). Os mecanismos imunológicos envolvidos na etiologia não são totalmente conhecidos. OBJETIVO: Avaliar a ativação de STAT-1 e a expressão da citocina INF-gama em mucosa de RI endoscópica e histologicamente normal, de doentes operados por RCUI e PAF, assintomáticos. CASUISTICA E MÉTODOS: Estudou-se 18 doentes submetidos à retocolectomia total com RI em (J), sendo nove com RCUI e nove com polipose adenomatosa familiar (PAF). Realizou-se biópsias da mucosa dos RI e de íleo terminal. As expressões de INF-gama e a ativação de STAT-1 foram avaliadas por meio de imunoblot de extrato protéico total. RESULTADOS: A ativação de STAT-1 foi maior em mucosa de RI de doentes operados por RCUI, quando comparada aos grupos PAF e Controle (p menor que 0.05). A expressão de INF-gama foi maior no grupo RCUI quando comparada ao grupo Controle (p menor que 0.05), mas não em relação ao grupo PAF (p maior que 0.05). CONCLUSÃO: Estes achados podem justificar a maior susceptibilidade dos doentes com RCUI à inflamação inespecífica do RI quando comparados aos portadores de PAF, sendo a principal via inflamatória similar à verificada na RCUI.

Pouchitis after total retocolectomy is the most common complication in ulcerative colitis (UC). The immunological mechanisms involved in the genesis of pouchitis are unclear. PURPOSE: To evaluate STAT-1 activation and IFN-gama expression in normal ileal pouch mucosa. METHODS: Eighteen asymptomatic patients submitted to total retocolectomy and J pouch, were evaluated, being nine with UC and nine with FAP. The activation of STAT-1 and expressions of the cytokine were determined by immunoblot of total protein extracts from pouch mucosal biopsies. RESULTS: STAT-1 activation was increased in UC, when compared to FAP and controls. Higher levels of IFN-gama expression were observed in UC when compared to control group, but was similar to FAP. CONCLUSIONS: These findings could explain a higher susceptibility to this inflammatory complication in UC when compared to FAP, which may be similar to UC disease.