Surgical results and quality of life of patients submitted to restorative proctocolectomy and ileal pouch-anal anastomosis / Resultados cirúrgicos e qualidade de vida de pacientes submetidos à proctocolectomia total e reservatório ileoanal

ABSTRACT Purpose: restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the surgical procedure of choice in some cases of familial adenomatous polyposis (FAP) and ulcerative colitis (UC). IPAA allows complete removal of the diseased colon and rectum, however, it is associated with substantial morbidity and potential consequences to patients' quality of life (QoL). Aims: to evaluate the surgical results, functional outcomes and QoL after IPAA; and to examine the impact of surgical complications upon QoL. Methods: we reviewed the records of 55 patients after IPAA, with emphasis on surgical outcomes. Forty patients answered the questionnaires. The Cleveland Global Quality of Life (CGQL), Inflammatory Bowel Disease Questionnaire (IBDQ), and Short Form 36 Health Survey Questionnaire (SF36). Results: the average age was 42.1±14.1 years. 63.6% of the patients were male, and 69.1% had FAP. Operative mortality was 1.8% and overall morbidity was 76.4%. Anastomotic leakage was the most frequent early complication (34.5%). Pouchitis (10.8%) and small bowel obstruction (9.1%) were the most common late complications. Patients with UC had the most severe complications (p=0.014). Pelvic complications did not have a negative effect on functional outcomes or QoL scores. Female patients had decreased pouch evacuation frequency, fewer nocturnal bowel movements, decreased bowel symptom impact on QoL (p=0.012), and better CGQL (p=0.04). Patients with better education had better QoL scores, and patients who had their pouches for more than five years scored lower. Conclusion: the high morbidity has no impact on function or QoL. Bowel function is generally acceptable. QoL is good and affected by sex, education and time interval since IPAA.

RESUMO Objetivo: a Proctocolectomia com reservatório ileoanal (PCT-RIA) é método de escolha em alguns casos de Polipose Adenomatosa Familiar (PAF) e Retocolite Ulcerativa (RCU). Embora tenha potencial curativo, apresenta morbidade considerável e pode afetar a qualidade de vida (QV) dos pacientes. Objetivos: avaliar resultados cirúrgicos e impacto das complicações pélvicas na função intestinal e QV. Métodos: foram avaliados retrospectivamente 55 pacientes submetidos a PCT-RIA, de janeiro de 2003 até abril de 2017, com ênfase na técnica operatória e morbidade. Quarenta pacientes responderam aos questionários Cleveland Global Quality of Life (CGQL), Inflammatory Bowel Disease Questionnaire (IBDQ) e Short Form 36 Health Survey Questionnaire (SF36). Resultados: A média de idade foi 42,1±14,1 anos, sendo 63,6% do sexo masculino e 69,1% com diagnóstico de PAF. A mortalidade cirúrgica foi 1,8% e morbidade 76,4%. Fístula anastomótica foi a complicação precoce mais frequente (34,5%) e, as tardias foram bolsite (10,8%) e obstrução intestinal (9,1%). As complicações precoces mais graves foram mais frequentes em pacientes com RCU (p=0,014). Não houve impacto das complicações na função intestinal nem na QV. As mulheres apresentaram menor frequência evacuatória e noturna, menor interferência dos sintomas intestinais na QV (p=0,012) e CGQL mais elevado (p=0,04). Melhor QV foi referida pelos pacientes com maior escolaridade e, foi observada piora em pacientes com mais de cinco anos de confecção do RIA. Conclusões: não se evidenciou impacto das complicações na função intestinal nem na QV. A função intestinal é satisfatória e a QV é boa na maioria dos pacientes, sendo influenciada pelo sexo, escolaridade e tempo de confecção do RIA.

Abordagem diagnóstica e de manuseio da pouchite em doença inflamatória intestinal / Diagnostic and management approach to pouchitis in inflammatory bowel disease

ABSTRACT In patients with ulcerative colitis refractory to medical therapy, total proctocolectomy and posterior ileal-anal pouch anastomosis is the standard surgical therapy. One of the possible complications is pouchitis. Depending on the duration of the symptoms, it can be classified as acute, recurrent, or chronic. The latter, according to the response to therapy, can be defined as antibiotic-dependent or refractory. The treatment of pouchitis is based on the use of antibiotics and probiotics. Thiopurine and biological therapy have been suggested in patients with refractory pouchitis. Special care should be taken in the endoscopic surveillance of these patients, especially if they present risk factors such as dysplasia or previous colorectal cancer, primary sclerosing cholangitis or ulcerative colitis for more than 10 years.

RESUMO Em pacientes com colite ulcerativa refratária à terapia médica, a proctocolectomia total e anastomose de bolsa ileal-anal posterior é a terapia cirúrgica padrão. Uma das possíveis complicações é a pouchite. Dependendo da duração dos sintomas, pode ser classificado como aguda, recorrente ou crônica. Esta última, de acordo com a resposta à terapia, pode ser definida como dependente de antibióticos ou refratária a eles. O tratamento da pouchite baseia-se no uso de antibióticos e probióticos. A thiopurina e a terapia biológica têm sido sugeridas em pacientes com pouchite refratária. Um cuidado especial deve ser tomado na vigilância endoscópica desses pacientes, especialmente se apresentarem fatores de risco, como displasia ou câncer colorretal anterior, colangite esclerosante primária ou colite ulcerativa por mais de 10 anos.

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.

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 complications and metachronous rectal cancer risk in patients with classic familial adenomatous polyposis / Complicações cirúrgicas e risco de câncer retal metacrônico em pacientes com polipose adenomatosa familiar clássica

INTRODUCTION: Familial adenomatous polyposis (FAP), an autosomal dominant disease characterized by development of numerous adenomatous polyps in the colon and rectum, is caused by germline mutations in the Adenomatous Polyposis Coli (APC) gene. METHODS: To determine the surgical morbidity in patients with classical familial adenomatous polyposis and determine the incidence of metachronous colorectal cancer (CRC) in those undergoing total colectomy (TC) with ileorectal anastomosis or restorative total proctocolectomy (TPC) and ileal pouch anal anastomosis. We analyzed patients with familial adenomatous polyposis who received treatment and regular follow-up at the A.C. Camargo Cancer Center from 1994 to 2013. RESULTS: Operative complications occurred in 22 patients (34.3%), 16 (25%) being early complications and 8 (12.5%) late complications. No mortality occurred as a result of postoperative complications. The incidence of metachronous rectal cancer after total proctocolectomy was 2.3% and after total colectomy 18.18% (p = 0.044). CONCLUSIONS: In order to provide better quality of life for individuals with familial adenomatous polyposis, total colectomy is commonly offered, as this simple technique is traditionally associated with lower rates of postoperative complications and better functional outcomes. However, it has become a less attractive technique in patients with familial adenomatous polyposis in its classical or diffuse form, since it has a significantly higher probability of metachronous rectal cancer. (AU)

INTRODUÇÃO: Polipose adenomatosa familiar (PAF), uma doença autossômica dominante caracterizada pela formação de numerosos pólipos adenomatosos no cólon e reto, é causada por mutações da linha germinativa no gene da polipose adenomatosa do cólon (PAC). MÉTODOS: Para determinar a morbidade cirúrgica em pacientes com PAF clássica e determinar a incidência de câncer colorretal (CCR) metacrônico naqueles pacientes submetidos à colectomia total (CT) com anastomose íleo-retal ou submetidos à proctocolectomia restaurativa (PCT) e anastomose bolsa ileal-anal, foram analisados pacientes com PAF que foram tratados e tiveram acompanhamento periódico no A. C. Camargo Cancer Center de 1994 até 2013. RESULTADOS: Ocorreram complicações cirúrgicas em 22 pacientes (34,3%); 16 (25%) tiveram complicações precoces e 8 (12,5%) complicações tardias. Não houve mortes como resultado de complicações pós-operatórias. A incidência de câncer de reto metacrônico após PCT foi de 2,3% e após CT foi de 18,18% (p = 0,044). CONCLUSÕES: A fim de proporcionar melhor qualidade de vida para os pacientes com PAF, CT é comumente oferecida, pois esta técnica simples está tradicionalmente associada com menores percentuais de complicações pós-operatórias e melhores resultados funcionais. No entanto, CT se tornou uma técnica menos atraente em pacientes com PAF em sua forma clássica ou difusa, uma vez que traz consigo uma probabilidade significativamente maior de câncer retal metacrônico. (AU)

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.

Quality of life in the late follow-up of ulcerative colitis patients submitted to restorative proctocolectomy with sphincter preservation over ten years ago.

OBJECTIVE: To evaluate, by means of the Inflammatory Bowel Disease Questionnaire (IBDQ), the quality of life of ulcerative colitis patients submitted to proctocolectomy with sphincter preservation using J-pouch reconstruction over ten years ago. METHODS: The study consisted of 36 patients interviewed using the Inflammatory Bowel Disease Questionnaire. The score scale, resulting from the addition of each answer, ranged from 32 to 224, where the highest score indicates the best quality of life. The chi square test was used to verify the existence of meaningful differences between the results of the questionnaire and age, and gender proportion. For each section, as well as for all of them combined, the Kruskal-Wallis test was used to verify if there were differences in the Inflammatory Bowel Disease Questionnaire scores among the groups in relation to the proportions. RESULTS: After applying the Inflammatory Bowel Disease Questionnaire, it was determined that quality of life was considered excellent for 9 (25%), good for 11 (30.6%), regular for 13 (36.1%), and bad for 3 (8.3%) patients. In our study, we determined that 85% of the patients were pleased with and thankful for the surgery that they underwent. CONCLUSION: We can conclude that the possibility of sphincter preservation should always be taken into account, since patients remain clinically stable and have a high quality of life even after long periods.

Quality of life in the late follow-up of ulcerative colitis patients submitted to restorative proctocolectomy with sphincter preservation over ten years ago

OBJECTIVE: To evaluate, by means of the Inflammatory Bowel Disease Questionnaire (IBDQ), the quality of life of ulcerative colitis patients submitted to proctocolectomy with sphincter preservation using J-pouch reconstruction over ten years ago. METHODS: The study consisted of 36 patients interviewed using the Inflammatory Bowel Disease Questionnaire. The score scale, resulting from the addition of each answer, ranged from 32 to 224, where the highest score indicates the best quality of life. The chi square test was used to verify the existence of meaningful differences between the results of the questionnaire and age, and gender proportion. For each section, as well as for all of them combined, the Kruskal-Wallis test was used to verify if there were differences in the Inflammatory Bowel Disease Questionnaire scores among the groups in relation to the proportions. RESULTS: After applying the Inflammatory Bowel Disease Questionnaire, it was determined that quality of life was considered excellent for 9 (25 percent), good for 11 (30.6 percent), regular for 13 (36.1 percent), and bad for 3 (8.3 percent) patients. In our study, we determined that 85 percent of the patients were pleased with and thankful for the surgery that they underwent. CONCLUSION: We can conclude that the possibility of sphincter preservation should always be taken into account, since patients remain clinically stable and have a high quality of life even after long periods.

Tratamiento quirúrgico de la rectocolitis ulcerativa: ¿Qué podemos ofrecerle a nuestros pacientes?

Introducción: La Rectocolitis Ulcerativa es una enfermedad cuyo tratamiento primordial es médico, pero la cirugía es una opción terapéutica eventualmente considerada. El objetivo de esta revisión es exponer la evolución histórica, indicaciones y resultados alcanzados con las intervenciones quirúrgicas propuestas para pacientes con Rectocolitis Ulcerativa. Métodos: Análisis de artículos pertinentes al tratamiento quirúrgico de la RCU identificados electrónicamente a través de búsqueda en PubMed, Lilacs y revisión manual de las referencias bibliograficas de dichos artículos. Resultados: Las indicaciones quirúrgicas de pacientes con Rectocolitis Ulcerativa incluyen: situaciones de emergencia, displasia, cáncer colorrectal y efectos secundarios inmanejables o falta de respuesta al tratamiento médico. En las situaciones de emergencia la opción más adecuada es la colectomía subtotal o total con ileostomía terminal. La cirugía electiva cuenta con diferentes opciones. La operación más funcional es la Proctocolectomía con Reservorio Ileoanal. Esta intervención ha sido ampliamente practicada, estudiada y aceptada mundialmente y brinda al paciente resultados satisfactorios. Conclusiones: La evolución del tratamiento quirúrgico para la Rectocolitis Ulcerativa ha sido contínua. Actualmente, las intervenciones quirúrgicas propuestas ofrecen una calidad de vida adecuada. Sin embargo, ya que las opciones quirúrgicas modifican la fisiología intestinal, debe discutirse ampliamente con el paciente las expectativas postoperatorias. Dado que en nuestro país es baja la frecuencia de enfermedades inflamatorias intestinales, parece razonable establecer grupos de trabajo multidisciplinarios, que conjuguen atención medico-quirúrgica, nutricional, de enfermería y psicológica, para ofrecer a estos pacientes la mejor y más actualizada atención posible.

Background: treatment for Ulcerative Colitis is mainly medical; however, surgery is a suitable option for some patients. The aim of this review article is to delineate the historical development of surgery for ulcerative colitis, and to analyze current surgical indications and outcomes of surgical options for patients with Ulcerative Colitis. Methods: Review of suitable articles identified electronically using PubMed and Lilacs search and manual checking of the references of the cited articles. Results: Surgical indications are: emergency conditions, dysplasia-colorectal cancer and unacceptable secondary effects or lack of response to medical treatment. Either subtotal or total colectomies with terminal ileostomy are the most accepted options for those patients in an emergency situation. There are several proposed elective interventions, and, currently, restorative proctocolectomy with the creation of an ileoanal reservoir is the best option. Such intervention has been widely performed through different centers with satisfactory results worldwide. Conclusions: Surgical treatment for Ulcerative Colitis has evolved continuously. Currently, surgical options can bring to the patients an adequate quality of life. Since surgical interventions modify intestinal physiology, physicians, surgeons and patients should have a comprehensive preoperative discussion, so the patients may have reasonable postoperative expectations. On the other hand, in areas with low prevalence of Inflammatory Bowel Disease, such as Venezuela, seems reasonable to establish multidisciplinary teams to offer the best care for patients with such diseases.