Current management status of hereditary colorectal cancer / 中华胃肠外科杂志

Hereditary colorectal cancer accounts for approximately 5% of all colorectal cancer cases, mainly including familial adenomatous polyposis and Lynch syndrome. Total proctocolectomy plus ileal pouch-anal anastomosis and total colectomy plus ileorectal anastomosis are two major procedures for familial adenomatous polyposis, however, the exact impact of these two procedures on surgical efficacy, oncologic efficacy as well as functional results still remains uncertain. Segmental colectomy and total colectomy are two major procedures for Lynch syndrome, each of them both has advantages and disadvantages, and there still lacks a consensus about the optimal strategy because of the nature of retrospective study with a relatively insufficient evidence support. As a result, we would make a review about the current surgical treatment status and future perspectives of hereditary colorectal cancer.

Mesenteric lengthening in pouch surgery: review of surgical techniques

Described for the first time in themedical literature in 1978 by Parks and Nicholls, total proctocolectomy with ileal-pouch anal anastomosis (IPAA) is nowadays the procedure of choice for patients with ulcerative colitis inwhom themedical therapy has, a selected group of patients with Crohń s disease, and for patients with familial adenomatous polyposis. Despite the advances in medical treatment regarding inflammatory bowel disease, up to 30% of patients still require surgery, and restorative proctocolectomy and IPAA are the mainstay of the surgical treatment. It is considered a demanding and technically-challenging procedure, with the main challenge being the performance of a tension-free IPAA; the main reason for failure of the tension-free anastomosis is a shortened mesentery. With particular attention to detail, sufficient length can be achieved to enable a safe anastomosis in most patients. Herein, we describe the available techniques to lengthen the mesentery of the ileal pouch to perform an easyto- reach tension-free anastomosis. (AU)

Resecciones colorrectales segmentarias en pacientes con colitis ulcerosa y displasia o adenocarcinoma / Segmental colectomy in patients with colitis-associated neoplasia or dysplasia

Resumen Introducción: El tratamiento estándar para los pacientes con colitis ulcerosa y displasia o adenocarcinoma de colon ha sido la proctocolectomía total, lo que conlleva una morbilidad y una reducción en la calidad de vida significativa. Materiales y Método: Se hace un análisis retrospectivo de 5 pacientes con colitis ulcerosa a los que se realiza una resección segmentaria por displasia o adenocarcinoma. Resultados: La mediana de edad al diagnóstico de colitis ulcerosa y de la neoplasia fue de 56 y 62 años respectivamente. El tiempo de evolución de la enfermedad fue de 1 a 13 años. La mediana de seguimiento postoperatorio fue de 57 meses apareciendo en uno de los pacientes un nuevo foco de displasia. Conclusiones: En determinados pacientes seleccionados, las resecciones segmentarias podrían ser una opción segura si tienen buen control de la enfermedad, escasa actividad inflamatoria, pocos años de evolución y que puedan realizar un adecuado seguimiento posterior.

Introduction: The historical management for patients with ulcerative colitis and displasia or adenocarcinoma associated was to perform a total proctocolectomy, what cause important morbidity and affect patient's quality of life. Materials and Method: A retrospective review about 5 patients with a segmental colectomy due to dysplasia or adenocarcinoma is done. Results: The median age at diagnosis of ulcerative colitis and cancer was 56 and 62 years respectively. Disease time evolution was between 1 and 13 years. The patients were followed up for a median of 57 months. During the follow-up evaluation, 1 patient was found to have dysplasia. Conclusion: Segmental colectomy could be a safe option in clinically stable patients, few years of diagnosis and with and a suitable follow up.

Oblique vs. circular anastomosis in the children underwent soave's pull-through surgery for the treatment of hirschsprung's disease: which is the best? / Anastomose oblíqua vs. circular em crianças submetidas à soave pull-through para o tratamento da doença de hirschsprung: qual é a melhor?

ABSTRACT Background: Several types of complications including constipation, fecal soiling, perianal excoriation, were reported among different types of surgery for Hirschsprung's disease. Aim: To compare circular and oblique anastomoses following Soave's procedure for the treatment of Hirschsprung's disease. Methods: Children who underwent Saove's pull through procedure with oblique and circular anastomoses were included. Duration of the follow up was two years after surgery. Postoperative complications, such as wound infection, wound dehiscence, peritonitis, fecal soiling, perianal excoriation, were recorded for each patient. Results: Thirty-eight children underwent oblique anastomoses. Circular ones were done for 32 children. Perianal excoriation was seen in 57.89% and 46.87% of children in oblique and circular group, respectively. Enterocolitis was more frequent in circular (40.62%) than oblique (28.94%) group. Anastomotic stricture was more frequent in circular (15.62%) than oblique (7.89%). Conclusion: Perianal excoriation was the most common complication among patient in both groups. Oblique anastomoses had fewer complications than circular, and may be appropriate option for patient who underwent Soave's procedure.

RESUMO Racional: Vários tipos de complicações, incluindo constipação, secreção fecal, escoriação perianal foram relatadas entre diferentes tipos de operações para a doença de Hirschsprung. Objetivo: Comparar as anastomoses circulares e oblíquas realizadas no procedimento de Soave para o tratamento da doença de Hirschsprung. Métodos: Neste estudo, foram incluídas crianças submetidas ao procedimento pull-through de Saove com anastomoses oblíquas e circulares. A duração do acompanhamento foi de dois anos no pós-operatório. Complicações, como infecção da ferida, deiscência da ferida, peritonite, secreção fecal, escoriação perianal foram registradas para cada paciente. Resultados: Trinta e oito crianças foram submetidas à anastomoses oblíquas. As circulares foram realizadas em 32. Escoriação perianal foi observada em 57,89% e 46,87% das crianças nos grupos oblíquo e circular, respectivamente. Enterocolite foi mais frequente no grupo circular (40,62%) do que oblíquo (28,94%). A estenose anastomótica foi mais frequente na circular (15,62%) do que na oblíqua (7,89%). Conclusão: A escoriação perianal foi a complicação mais comum entre os pacientes nos dois grupos. A anastomose oblíqua teve menos complicações do que a anastomose circular e pode ser a opção adequada para o paciente submetido ao procedimento de Soave.

Resultados inmediatos y alejados del reservorio ileal con anastomosis reservorio-anal practicado en forma simultánea o diferida con la proctocolectomía en pacientes portadores de colitis ulcerosa / Ileal pouch-anal anastomosis performed simultaneously or delayed with the proctocolectomy: short and long-term results in patients with ulcerative colitis

Objetivo: Analizar las diferencias en los resultados quirúrgicos entre 2 grupos en colitis ulcerosa: proctocolectomía, reservorio ileal y anastomosis reservorio-anal (RIARA), simultáneo con la proctocolectomía (grupo 1) o diferido (grupo 2). Material y método: Estudio retrospectivo en 126 pacientes sometidos a RIARA. En todos los pacientes se confeccionó un RIARA en «J¼, excepto en 4 que se hizo en «S¼. Todos fueron protegidos con ileostomía. Complicaciones Clavien-Dindo II-V fueron registradas. Resultados: Pacientes con una mediana de edad de 37 años (12-61), 72 eran de género femenino (57%). Se practicó proctocolectomía y RIARA en 24 pacientes (19%) y proctectomía y RIARA en 102 (81%). Se observaron complicaciones postoperatorias en 19 pacientes (13%). Infección de la herida y sepsis pelviana, en 4% respectivamente, fueron las más frecuentes. Tres pacientes fueron reintervenidos: 2 por hemoperitoneo y uno por necrosis isquémica del reservorio. No hubo mortalidad postoperatoria. No se observó diferencia significativa en la morbilidad postoperatoria entre los grupos. Se observaron complicaciones a largo plazo en 48 pacientes (38%): obstrucción intestinal en 18 pacientes (14%), fístula reservorio-vaginal (FRV) en 9 (12,5%), y reservoritis crónica en 8 (6,9%) fueron las más frecuentes. Al comparar los 2 grupos, se observó mayor frecuencia de FRV en el grupo 1 (p = 0,02). Conclusión: En este estudio no se demostró diferencia en la morbilidad postoperatoria entre el grupo 1 y 2. En los resultados alejados hubo mayor frecuencia significativa de FRV en el grupo 1.

Aim: To compare the surgical results of both groups: Simultaneous with the proctocolectomy (SRP) (group 1) or delayed after colectomy (DRP) (group 2). Material and methods: Retrospective study on 126 patients submitted to RP. All patients had a J-pouch, except 4 S. All of them protected with a loop ileostomy. The median time between colectomy and IPAA was 5 months (4-6 range). Clavien-Dindo II-V complications were registered. Results: 126 patients had IPAA. Age median 37 years (12-61 range); 72 (57%) women. SRP in 24 (19%) and DRP was performed in 102 (81%). Postoperative complications were recorded in 19 patients (13%). Wound infection and pelvic sepsis were observed in 4% each. Three patients needed early reoperation: two for hemoperitoneum and one for ischemic necrosis of the pouch. There was no post-operative mortality. No significant difference in 30 days postoperative complication rate was found between SRP and DRP. On long-term follow-up: Intestinal obstruction in 18 patients (14%), pouch-vaginal fistula (PVF) in 9 (12.5%) and chronic pouchitis in 8 (6.9%) were the most common complications. PVF was significantly more frequent on group 1. Conclusion: In this series, no significant difference was found in the early surgical results between group 1 and 2. In the long term, PVF was significantly more common in group 1.

Reconstrucción anorrectal total luego de una coloproctectomía: resultados funcionales / [Total anorectal reconstruction after proctocolectomy: surgical results and functional outcome].

The aim of this report is to describe a novel technical approach to total anorectal reconstruction and show surgical results and functional outcome. The technique is an innovative surgery to restore gastrointestinal perineal continuity after coloproctectomy in patients with familial adenomatous polyposis. We made the internal anal sphincter replacement with demucosated small bowel plication, the external anal sphincter replacement with an artificial bowel sphincter (ABS) and the restitution of intestinal transit with and ileal "S" pouch. After 12 months follow-up the control of gas is irregular, normal continence to solid stool was achieved with only occasional minimal soiling after defecation. The Jorge-Wexner incontinence score is 6 (moderate incontinence). The fecal incontinence quality of life (FIQL) comparing stoma vs. non-stomas, shows a relevant clinical difference, with improvement in all scales. This study has limitations because it is preliminary, observational and with no control group. We conclude that this recent surgical technique requires expertise in pelvic floor surgery and management of fecal incontinence. The surgeon should be able not only to introduce an artificial anal sphincter, but also to make the plication of intestinal muscle layer to create a zone of high pressure in anal canal and the ileal pouch.

Resección mesorrectal total y anastomosis coloanal con reservorio colónico en J para el tratamiento de los cánceres de recto medio y bajo / Total mesorectum resection and coloanal anastomosis with J colonic reservoir for treatment of medium and low rectum cancer

INTRODUCCIÓN. La proctosigmoidectomía con resección mesorrectal total, reservorio en J y anastomosis coloanal es útil en los pacientes con cánceres de recto medio y bajo, para evitar la colostomía terminal definitiva. El objetivo de este trabajo fue analizar la factibilidad de dicha técnica quirúrgica, el tratamiento integral multidisciplinario y los resultados obtenidos. MÉTODOS. Se estudiaron 15 pacientes que padecían adenocarcinomas de recto medio y bajo, tratados entre enero de 1996 y diciembre de 2002 en el servicio de Cirugía Esplácnica del Instituto Nacional de Oncología y Radiobiología de La Habana. El tratamiento consistió en una combinación de radioterapia más quimioterapia concurrentes neoadyuvantes, seguidas de cirugía y quimioterapia adyuvante. RESULTADOS. La edad promedio de los pacientes fue de 56 años. El adenocarcinoma fue el tipo histológico diagnosticado en todos los pacientes. La estadificación de los tumores fue la siguiente: T1 y T2, 4 pacientes (27 por ciento, respectivamente); T3, 7 pacientes (46 por ciento). Cuatro pacientes (20 por ciento) se complicaron como consecuencia del tratamiento radiante y 5 (33,3 por ciento), a causa del tratamiento quirúrgico. La mortalidad quirúrgica fue de 1 paciente (6,6 por ciento) y 11 pacientes (73,3 por ciento) sobrevivieron más de 5 años. Ningún paciente presentó recidiva tumoral pélvica ni de la anastomosis coloanal. Se logró buena continencia esfinteriana. CONCLUSIONES. La resección mesorrectal total y anastomosis coloanal con reservorio colónico en J evita la colostomía terminal definitiva, cura a un alto porcentaje de pacientes con cánceres de recto medio y bajo, no transgrede los principios de la cirugía oncológica, es bien aceptada por los pacientes y es factible en nuestro medio(AU)

INTRODUCTION: Proctosigmoidectomy with total mesorectum resection, reservoir in J and colorectal is useful in patients with low and middle rectum cancer, to avoid the definite terminal colostomy. The aim of present paper was to analyze the feasibility of such surgical technique, the multidisciplinary integral treatment and the results obtained. METHODS: Fifteen patients were studied suffering of low and middle adenocarcinoma treated between January, 1996 and December, 2002 in Splanchnic Surgery Service of National Institute of Oncology and Radiobiology of La Habana City. Treatment included a combination of radiotherapy plus neocoadjuvant concurrent chemotherapy, followed by adjuvant chemotherapy and surgery. RESULTS: Mean age of patients was of 56 years. The adenocarcinoma was the histological type diagnosed in all patients. Tumor staging the following: T1 and T2, in four patients (27 percent, respectively); T3 in seven patients (46 percent). Four patients (20 percent) had complications due to radiation treatment and five (33,3 percent), by surgical treatment. Surgical mortality occurred in one patient (6,6 percent) and eleven patients (73,3 percent) survived over 5 years. Neither patient had pelvic tumor relapse or by colorectal anastomosis. There was good sphincter continence. CONCLUSIONS: Total mesorectum resection and colorectal anastomosis with a colonic reservoir in J prevent the definite terminal colostomy, to cure a high percentage of patients with low and middle rectum cancer without respecting the oncology surgery principles, is well accepted by patients and it is feasible in our practice(AU)

Inflamación del remanente rectal (Cuffitis) posterior a una proctocolectomía total con reservorio ileal: reporte de caso y enfoque actual de tratamiento / Rectal cuff inflammation (Cuffitis) after total proctoclectomy with ileal pouch anal anastomosis: case report and current approach to treatment

La alternativa quirúrgica de elección para los pacientes con colitis ulcerosa (CU) refractaria al tratamiento médico es la proctocolectomía total con una reconstrucción a través de la confección de un reservorio ileal y una anastomosis ileo-anal. Esta cirugía permite la curación de la enfermedad con buen resultado funcional. A pesar de las demostradas ventajas, no está exenta de complicaciones. Una de las complicaciones es la inflamación sintomática del remanente rectal o cuffitis. Esta genera controversias importantes acerca de la técnica que debe utilizarse para la anastomosis ileo-anal debido a la asociación que presenta con la preservación de mucosa rectal en técnicas de sutura mecánica. El presente trabajo propone la revisión del tema y el análisis del enfoque actual de tratamiento, basada en un caso clínico.

Restorative proctocolectomy with ileal pouch is the treatment of choice in the majority of patients with ulcerative colitis (UC) refractory to medical treatment. This surgery can cure the disease with good functional outcome. Despite the proven benefits, is not without complications. One of the complications is symptomatic inflammation of the rectal remnant or cuffitis. This generates significant controversy about the technique to be used for the Ileo-anal anastomosis because of the association presented to the preservation of rectal mucosa in mechanical suture techniques. This paper proposes the review of the topic and analysis of the current approach to treatment based on one case.

Proctocolectomía restauradora con reservorio íleoanal laparoscópica sin ileostomía de protección / Restorative proctocolectomy with ileal pouch anal anastomosis without loop ileostomy

El objetivo de este trabajo es presentar el resultado de la primera proctocolectomía laparoscópica con reservorio íleo-anal, sin ileostomía de protección, practicada en el Hospital Clínico de la Pontificia Universidad Católica de Chile. La operación se realizó en un paciente de 34 años con poliposis adenomatosa familiar. El paciente tuvo un cuadro clínico de un mes y medio de evolución, caracterizado por dolor abdominal y cambio del hábito intestinal. El estudio colonoscópico reveló la presencia de múltiples pólipos en colon (>100 pólipos), así como también, compromiso rectal. Dado los hallazgos intraoperatorios se decidió efectuar una proctocolectomía laparoscópica más un reservorio ileal en J, sin ileostomía de protección; la pieza operatoria se extrajo mediante una incisión de Pfannenstiel. La cirugía se realizó sin dificultades en un tiempo de 340 minutos. El paciente se realimentó con líquidos al 5º día y se dio de alta al 9º día postoperatorio, sin complicaciones. No se registraron complicaciones en el seguimiento temprano a 30 días del alta hospitalaria. Luego de 6 meses desde su operación, el paciente presenta 4-6 deposiciones diarias, sin urgencia y utiliza loperamida® en forma esporádica.

The aim of this paper is to present the result of the first laparoscopic proctocolectomy with Neo anal pouch, without loop ileostomy, made in the Clinical Hospital of the Pontificia Universidad Católica de Chile. The operation was made in a patient of 34 years old, with adenomatous familial polyposis. The patient had a clinical presentation of a month and a half with abdominal pain and change of abdominal bowel evacuation. The colonoscopic study reveal the presence of multiples polyps in the colon (>100 polyps), and rectal compromise. In order to the intraoperative findings we decided made a laparoscopic proctocolectomy with an ileal J pouch, without protection ileostomy, and surgical specimen was extracted through Pfannensteil's incision. The surgery was made without difficulties, with an operative time of 340 minutes. The patient was feed with liquid diet in the 5° postoperative day, and was discharge the 9° postoperative day, without complications. There were not complications in the early follow up (<30 days since the operation). After 6 months, the patient presents 4-6 bowel movements per day, without urgency and use loperamida sporadically.

Resultados funcionales a largo plazo luego de la coloproctectomía con reservorio ileal en pacientes con y sin complicaciones postoperatorias / Long term functional outcomes after ileoanal pouch in patients with and without postoperative complications

Introducción: La coloproctectomía con reservorio ileal es la opción quirúrgica de elección en pacientes con colitis ulcerosa (CU). Las complicaciones postoperatorias on frecuentes pero los resultados definitivos son considerados, en general, buenos. Objetivo: nuestro objetivo fue evaluar los resultados funcionales a largo plazo de una serie consecutiva de pacientes con pouch ileal (PI) y establecer su relación con las complicaciones postoperatorias. Pacientes y métodos: incluimos 144 pacientes categorizados en 2 grupos: I- pacientes sin complicaciones postoperatorias (n: 71); y II- pacientes con complicaciones postoperatorias (n: 73). Los pacientes fueron seguidos por un tiempo medio de 3,8 años (rango 1-15 años). Evaluamos: número de deposiciones, capacidad de discriminación entre gases y materiafecal, inhibición voluntaria de la defecación, requerimiento de medicación antidiarreica y grado de incontinencia. Resultados: los resultados funcionales fueron similares entre los dos grupos con respecto al número de deposiciones, a la necesidad de tomar antidiarreicos, a la discriminación anal y a la inhibición voluntaria de la defecación por más de 5 hs. Se observó una mayor tendencia a presentar escapes en los pacientes con complicaciones (34%) respecto de aquellos sin ellas (20%; p<0.08 NS). Comparado con el grupo II, los escapes en el grupo I estaban significativamente relacionados con el incumplimiento de la dieta (p<0.01). La infección pélvica fue la complicación más frecuente en los pacientes con incontinencia (56%). Conclusiones: nuestro estudio demostró que la mayoría de los parámetros funcionales a largo plazo no son influenciados por la ocurrencia de complicaciones postoperatorias, con excepción de una tendencia a la incontinencia, más frecuentemente asociada con la infección pélvica.

Background: the proctocolectomy with ileal pouch (IP) has become the gold standard for surgical treatment for ulcerative colitis (UC). The postoperative complications are frequent but the definitive outcome is acceptable. Aim: our aim was to evaluate the long term functional outcome in a series of consecutive patients with IP and o establish its relation with postoperative complications. Patients and methods: we included 144 patients categorized in two groups: I- patients without postoperative complications (n: 71); II- patients with postoperative complications (n: 73). Patients were followed for a median time of 3.8 years (range 1-15). We evaluated: stool frequency, anal discrimination between gas and stool, voluntary evacuation inhibition, requirement of antidiarrheal medications and presence of incontinence. Results: the functional outcome was similar between the two groups in terms of stool frequency, medication use, anal discrimination and the voluntary inhibition of evacuation for more than 5 hours. A trend to present seepage was seen in patients with complications (34%) more than in those without them (20%; p<0.08 NS). Compared with group II, seepage in group I was significantly related with dietary lapses (p<0.01). The most frequent complication in incontinent patients was pelvic sepsis (56%). Conclusions: our study showed that most of long-term functional parameters are not influenced by the presence of postoperative complications. However, a trend for incontinence seems to be related to postoperative complications, more frequently with pelvic sepsis.

Use of a colonic pouch as a rectal substitute after rectal excision

Mid or distal rectal resection with straight coloanal anastomosis effectively treats distal rectal cancer and avoids a permanent stoma. However, the straight colonic segment is a poor reservoir for stools, and patients usually experience varying degrees of impaired rectal function after operation, including frequent bowel movements, incontinence, tenesmus, and soiling. In contrast a J-shaped colonic pouch provides an adequate neorectal reservoir after operation. Patients with a colonic pouch-anal canal anastomosis have bowell movements per day than patients with straight colorectal or coloanal anastomosis. Furthermore, the morbility of the colonic pouch is not greater than that of the straight coloanal anastomosis. An important technical aspect of the colonic pouch procedure is that the limbs used to form the pouch must be no longer than 5 to 6 cm. Patients with larger pouches experience emptying difficulties. Also, the level of the anastomosis between the pouch and the anal canal must be no more than 4 cm from the anal verge, again to avoid problems with defecation. With these caveats, the operation should be considered in patients who require excision of the mid and distal rectum for cancer.

Surgical treatment of ulcerative colitis

This is a preliminary report of 28 patients with ulcerative colitis who were surgically treated with "J" pouch endorectal pull-through operation from 1978 to 1996. All patients are continent with a median stool frequency of 4 times per day, no night soilage, low postoperative complications and one mortality due to pulmonary embolism

Familiar Juvenile adenomatous polyposis: a rare entity-case report with emphasis on the mode of treatment

Juvenile and adenomatous polyps occurring simultaneously in the large bowel is a very rare entity and its mode of treatment is similar to familial adenomatous polyposis coli. In our case we performed a total proctocolectomy with an ileostomy, a procedure which is not a first choice in familial adenomatous polyposis coli

Anastomosis íleo-anal con o sin reservorio en niños y adolescentes con rectocolitis ulcerosa y poliposis familiar / Ileoanal anastomosis with and without reservoir in children and adolescents with ulcerative colitis and familial poliposis coli

Evaluamos el curso post-operatorio en niños anastomosis ileo-anal (Grupo 1, N-9), comparado con los resultados después de la anastomosis ileo-anal con reservorio (Grupo 2, N=7). Tres de nueve niños del Grupo 1 desarrollaron diarrea intratable, lo que hizo necesario una revisión de la anastomosis, para incluir una bolsa ileal. No hubo necesidad de revisión quirúrgica de la anastomosis para los pacientes del Grupo 2, aunque dos pacients tienen todavía la ileostomía, debido a la formación de absceso perianastomótico. Estos dos pacientes fueron excluídos del análisis subsiguiente. Dos meses después que la cirugía fue completada, niños en el Grupo 1 tuvieron 16 ñ 2 evacuaciones diarias en los cinco niños del Grupo 2, P < 0,05. Niños en el Grupo 1 tuvieron una evolución más pobre comparado con pacientes en el Grupo 2, determinado esto por: Incontinencia fecal (7/9 vs 0/5), dermatitis perianal (6/9 vs 1/5), tratamiento con agentes antidiarréicos (5/9 vs 0/5) ausentismo escolar (4/9 vs 0/5). Concluímos que para niños quienes requieren colectomía, quizás se obtengan mejores resultdos funcionales después de realizar una anastomosis ileo-anal con reservorio (creación de bolsa ileal) comparado con la anastomosis ileo-anal simple sin reservorio