Correlation Between the Severity of Adhesions and Desmoid Disease in Patients With Familial Adenomatous Polyposis: A Prospective Cohort Study.

BACKGROUND: Clinical experience teaches that intraperitoneal adhesions are more severe in patients with familial adenomatous polyposis than in patients without it. This impression may come from the common association of familial adenomatous polyposis with desmoid disease. OBJECTIVES: This study aimed to determine whether patients with familial adenomatous polyposis and desmoid disease develop more severe adhesions than those without desmoid disease. DESIGN: Prospectively collected data study. SETTINGS: Hereditary colorectal cancer center in a tertiary referral hospital. PATIENTS: Patients undergoing first reoperative intra-abdominal surgery for familial adenomatous polyposis; controls were those having their initial abdominal surgery. INTERVENTIONS: Surgery and adhesiolysis. MAIN OUTCOME MEASURES: Presence and type of desmoid disease; presence and severity of nondesmoid intraperitoneal adhesions. Where patients had multiple operations, only the first reoperative surgery was chosen. Desmoid disease was noted as reaction (sheet) or mass. Adhesions were graded as none, mild (<10 min for mobilization), average (10-30 min), and severe (>30 min or significant bowel damage). Patients having their first abdominal surgery for familial adenomatous polyposis were used as a control group. RESULTS: A total of 211 patients had no prior surgery; 5% had desmoids and 1% had adhesions. One hundred thirty-seven patients underwent reoperative surgery: 39% had desmoid disease ( p < 0.05 vs no prior surgery), the highest rate being in patients after IPAA (57%), and 45% had severe adhesions ( p < 0.01 vs no prior surgery), worst after Koch pouch (89%), and total proctocolectomy with ileostomy (82%). Thirty-six percent of patients without desmoid disease had severe adhesions. Desmoid reaction was associated with severe adhesions in 47% of cases and desmoid tumors in 66% of cases. LIMITATIONS: Possible limitations include the potential overlap between desmoid adhesions and nondesmoid adhesions and the potential for inaccuracy in defining the time of adhesiolyses. CONCLUSIONS: Familial adenomatous polyposis is associated with severe postoperative adhesions after reoperative abdominal surgery, especially in patients who develop desmoid disease. See Video Abstract . CORRELACIN ENTRE LA GRAVEDAD DE LAS ADHERENCIAS Y LA ENFERMEDAD DESMOIDEA EN PACIENTES CON POLIPOSIS ADENOMATOSA FAMILIAR ESTUDIO PROSPECTIVO DE COHORTES: ANTECEDENTES:La experiencia clínica demuestra que las adherencias intraperitoneales son más graves en pacientes con poliposis adenomatosa familiar que en pacientes sin enfermedad desmoidea. Esta impresión puede provenir de la asociación común de poliposis adenomatosa familiar con enfermedad desmoidea.OBJETIVOS:Ver si los pacientes con poliposis adenomatosa familiar y enfermedad desmoidea desarrollan adherencias más graves que aquellos sin enfermedad desmoidea.DISEÑO:Estudio de datos recolectados prospectivamente.AJUSTES:Centro de cáncer colorrectal hereditario en un hospital de referencia terciario.PACIENTES:Pacientes sometidos a una primera cirugía intraabdominal de caracter reoperatorio por poliposis adenomatosa familiar: los controles fueron los que se sometieron a su cirugía abdominal inicial.INTERVENCIONES:Cirugía y adhesiolisis.PRINCIPALES MEDIDAS DE RESULTADO:Presencia y tipo de enfermedad desmoidea; presencia y severidad de adherencias intraperitoneales no desmoideas. Cuando los pacientes tenían múltiples operaciones, solo se eligió la primera cirugía reoperatoria. La enfermedad desmoidea se anotó como reacción (hoja filamentosa) o masa. Las adherencias se calificaron como ninguna, leve (<10 minutos para la movilización), promedio (10 a 30 minutos) y severa (>30 minutos o daño intestinal significativo). Los pacientes sometidos a una primera cirugía abdominal por poliposis adenomatosa familiar se utilizaron como grupo de control.RESULTADOS:211 pacientes no tenían cirugía previa: 5% desmoideos y 1% adherencias. 137 pacientes se sometieron a cirugía reoperatoria: 39% tenía enfermedad desmoidea ( p < 0,05 frente aquellos sin cirugía previa), la tasa más alta se presentó en aquellos pacientes después de una anastomosis ileoanal con reservorio (57%) donde el 45% tenía adherencias graves ( p < 0,01 frente aquellos sin cirugía previa), peores resultados se observaron después de la confección de un reservorio de Koch (89%) y luego de proctocolectomía total con ileostomía (82%). El 36% de los pacientes sin enfermedad desmoidea tenían adherencias graves. La reacción desmoidea se asoció con adherencias graves en el 47% de los casos, y los tumores desmoides se asociaron con adherencias graves en el 66% de los casos.LIMITACIONES:Superposición potencial entre adherencias desmoideas y adherencias no desmoideas. Posible inexactitud en la definición del tiempo de adhesiolisis.CONCLUSIONES:La poliposis adenomatosa familiar se asocia con adherencias postoperatorias graves después de una cirugía abdominal reoperatoria, especialmente en pacientes que desarrollan enfermedad desmoidea. (Traducción-Dr. Xavier Delgadillo ).

Does one size fit all? Risks and benefits of neoadjuvant chemoradiation in patients with clinical stage IIA rectal cancer requiring abdominoperineal resection.

BACKGROUND: Neoadjuvant chemoradiotherapy (nCRT) has become the standard of care for locally advanced rectal cancer, decreasing locoregional recurrence, yet with an unclear survival advantage. We aimed to assess the benefit of nCRT on oncologic and perioperative outcomes of patients with clinical stage IIA rectal adenocarcinoma treated with abdominoperineal resection (APR). METHODS: Patients with clinical T3N0 rectal adenocarcinoma that underwent APR between 1995 and 2014 were included. Patients who received nCRT were compared with patients who did not. Multivariate analysis was conducted to compare oncological and perioperative outcomes between the groups. RESULTS: 127 patients were included, of which 94 received nCRT. Median follow-up was 11.9 years. There was no difference in circumferential margins, postoperative morbidity, and complication rates between the groups. There was no difference in 5-year oncological outcomes between the groups. CONCLUSIONS: No difference was found in 5-year oncological outcomes between patients with clinical T3N0 rectal adenocarcinoma necessitating an APR who received nCRT and those not receiving nCRT, with similar overall complication rates.

Superior Mesenteric Artery Pseudoaneurysms in Patients With Familial Adenomatous Polyposis-Associated Intra-abdominal Desmoids: Case Series.

BACKGROUND: Rupture of a superior mesenteric artery pseudoaneurysm is a rare but potentially lethal complication in patients with familial adenomatous polyposis and desmoid disease. OBJECTIVE: We report our experience in the management of such patients with a rare but significant and life-threatening condition. DESIGN: This is a descriptive study of a small series of patients. SETTINGS: Data were obtained from their medical charts and from the Cologene Database of the David G. Jagelman Polyposis Registry in the Sanford R. Weiss, M.D., Center for Hereditary Colorectal Cancer at the Cleveland Clinic Foundation. PATIENTS: Of 227 patients with mesenteric desmoid disease, there were 4 cases of superior mesenteric artery pseudoaneurysm rupture. MAIN OUTCOMES MEASURES: We reviewed the patients with mesenteric desmoid tumors in our desmoid registry. The registry is approved by the institutional review board of the Cleveland Clinic. RESULTS: The patients were young (aged from 22 to 28 y at presentation), with otherwise minimal comorbidities. Two patients had a previous proctocolectomy and J-pouch, and 2 had a total colectomy and ileorectal anastomosis. Two patients had preemptive endoluminal stenting and fared better than the 2 who had damage control embolization. One patient died and, in the others, recovery was prolonged and complicated. Two of the 4 patients have ended up with a reasonable quality of life. LIMITATIONS: We acknowledge that this is a rare complication of an uncommon disease and, as such, any case series will be limited by small numbers; therefore, a tailored approach is warranted when managing such complex patients. CONCLUSIONS: We advocate an increased awareness of the possibility of visceral pseudoaneurysms in patients with familial adenomatous polyposis who have desmoid disease encasing the superior mesenteric artery. See Video Abstract at http://links.lww.com/DCR/A914.

Factors associated with noncomplete mesorectal excision following surgery for rectal adenocarcinoma.

BACKGROUND: The completeness of the resected mesorectum is a quality metric in rectal cancer surgery and has been related to oncological outcomes. Our aim was to identify variables associated with non-complete mesorectal excision and determine any effect on overall survival. METHODS: Consecutive patients who underwent curative intent surgery for rectal adenocarcinoma (2009-2016) were identified from a prospectively-maintained institutional database. Patients were grouped according to their mesorectal grade: complete, near-complete and incomplete. Multivariate analysis was performed to identify the association between various patient, disease and surgeon-related characteristics and mesorectal grading. Log-rank tests were used to evaluate any difference in overall survival between the groups. RESULTS: 689 patients met inclusion criteria. Demographics and perioperative variables were comparable between the groups. On multivariate analysis, abdominoperineal resection, and involved circumferential resection margin were significantly associated with non-complete mesorectum. Finally, patients with non-complete mesorectal grading have approximately twice the hazard of death compared to those with complete mesorectal grading. CONCLUSIONS: Several factors are associated with a non-complete mesorectal excision. Non-complete mesorectal grade is associated with decreased survival.

Desmoids and genotype in familial adenomatous polyposis.

BACKGROUND: Desmoid disease can be a serious, life-threatening complication of familial adenomatous polyposis. The ability to predict patients at increased desmoid risk is important, but a convincing genotype-phenotype correlation for desmoid formation has not yet been described. PURPOSE: The aim of this study is to assess the relationship between desmoid disease and genotype in patients with familial adenomatous polyposis. DESIGN: This is a cohort study. PATIENTS: All patients with familial adenomatous polyposis and a documented pathogenic APC mutation in themselves or a first-degree relative were selected. MAIN OUTCOMES MEASURES: The comparison of genotype and the presence, stage, and site of desmoid disease are the primary end points of this study. RESULTS: Three hundred twenty-three patients from 219 families were identified. Mutations spanned the length of the gene, from codon 213 to codon 2051. Desmoid disease was diagnosed in 77 patients from 68 families. Desmoid disease was found in 14.9% of patients with a mutation 5' of codon 400, 23.2% of patients with a mutation from codon 401 to 1400, and in 37.1% of those with a mutation 3' of 1400. All patients with 5' mutations had stage I or II abdominal desmoid disease, and all tumors were stable or shrinking. Twelve percent of patients who had desmoid disease with mutations between codons 400 and 1400 had stage III or IV desmoid disease, and 5 of 42 (12%) tumors were growing at the time of the study. There had been 2 desmoid-related deaths. Almost half (44%) of patients who had desmoid disease with mutations 3' of codon 1400 had stage III or IV disease. Three of 14 tumors were growing (21%), and there were 4 desmoid-related deaths. LIMITATIONS: This study was conducted at a tertiary referral center, and there was no systematic surveillance for desmoids. CONCLUSION: Desmoid disease occurs in patients who have familial adenomatous polyposis with almost any APC mutation, although there is an increased propensity in those with a 3' mutation. The incidence and severity of the desmoid disease are related to the site of the mutation.

The use of ancillary techniques to aid colonoscope insertion.

BACKGROUND: There is more to a high-quality colonoscopy than reaching the cecum or finding adenomas. Gentle insertion involves ancillary techniques, some of which are impossible in patients receiving deep sedation. The aim of this study was to assess the importance of ancillary techniques in achieving a comfortable, complete colonoscopy. METHODS: This was a prospective study of 500 consecutive outpatient colonoscopies performed by a single endoscopist. During the procedure, note was taken of various ancillary techniques used to facilitate insertion: turning the patient, patient holding their breath, abdominal pressure. The success of these techniques in helping scope advancement was also noted. After the procedure the patient was asked to rate the severity of their pain and their degree of satisfaction with the examination. RESULTS: A total of 238 women and 262 men participated in the study, and colonoscopy was complete in 96.2 and 98.5%, respectively. Overall, 97% of patients received versed alone (average dose 2 mg), and 3% received a narcotic (average dose 46 U). Average pain score was 3.9 on a scale of 1 (least pain) to 10 (worst pain), and average satisfaction with the procedure as a whole was 9.5 on a similar scale. Ancillary techniques of holding breath, directed abdominal pressure, and turning to the left, right, and prone were used in 46, 56, 17, 23, and 4% of examinations, respectively. Each technique was effective in helping insertion in over 70% of cases. Turning the patient was also used to clear stool, open the colon, or facilitate polypectomy in another 33 cases. Multiple techniques were used in 49% of patients. CONCLUSION: The use of ancillary techniques to facilitate colonoscope insertion helps avoid the expense and potential side effects of deep sedation.

Desmoid tumors do not prevent proctectomy following abdominal colectomy and ileorectal anastomosis in patients with familial adenomatous polyposis.

BACKGROUND: Elective proctocolectomy has been recommended for patients at high risk of desmoids based on the possibility that cancer in a retained rectum may be unresectable because of desmoid disease. There are no data to support the reality of this concern. OBJECTIVE: The aim of this study was to see how often proctectomy was prevented by desmoids. DESIGN: This retrospective, descriptive, database study was augmented by chart review. SETTING: This study was conducted at a hereditary colorectal cancer clinic in a tertiary referral center. PATIENTS: Those presenting for proctectomy after colectomy and ileorectal anastomosis for familial adenomatous polyposis were selected. INTERVENTIONS: Patients underwent a proctectomy. MAIN OUTCOME MEASURES: The primary outcomes measured were the rate of proctectomy, rate of IPAA, and the incidence of desmoid disease. RESULTS: Sixty- seven patients, 34 men and 33 women, underwent an operation with the intent of performing proctectomy. Mean age at surgery was 39.7 years, an average of 175 months from ileorectal anastomosis. Indications for proctectomy were uncontrollable adenomas in 56, cancer in 8, and high-grade dysplasia in 3. Proctectomy was always possible. Ileal pouch-anal anastomosis was planned in 62 patients; 54 had this operation. Desmoid disease was found in 26 patients (38.8%) and influenced surgery in 13 cases, stopping pouch-anal anastomosis in 8. One patient had no resection, 2 had a pouch-low rectal anastomosis, and 5 had proctectomy and ileostomy. Proctectomy and ileostomy was planned in 5 patients and performed in all. LIMITATIONS: This is a retrospective review from a single institution. CONCLUSION: The fear of an unresectable rectum or an impossible pouch-anal anastomosis should not be an indication for proctectomy in patients with low rectal polyp counts but a high risk for desmoid disease.

Keeping the cecum clean: a randomized, prospective, placebo-controlled trial of loperamide as part of preparation for colonoscopy.

BACKGROUND: Coating of the cecum with sticky bile causes a problem with inspection of the mucosa during colonoscopy. PURPOSE: This study aimed to see whether 4 mg of loperamide taken after colonoscopy preparation would delay the passage of bile and improve the quality of cecal preparation. PATIENTS: Patients undergoing office colonoscopy were included. Patients with incomplete colonoscopies or right colectomy were excluded. STUDY DESIGN: This study was a prospective, randomized, double-blinded, and placebo-controlled trial. INTERVENTION: Either 2 placebo capsules or 2 loperamide capsules were taken after gut lavage, as soon as the passage of liquid stool ceased. Cecal photographs were scored in a blinded fashion. MAIN OUTCOME MEASURES: The primary outcomes measured were the quality of cecal preparation on a scale of 1 to 5 and overall preparation on a scale of 1 to 4. RESULTS: Ninety-eight patients took loperamide (50 men, 48 women) and 102 took placebo (57 men, 45 women). Mean ages of the loperamide group were: men, 61.9 ± 11.9 years, and women, 61.8 ± 10.0 years; and mean ages of the placebo group were: men, 62.5 ± 12.1 years, and women, 58.6 ± 9.8 years. Over 90% of patients used a polyethylene glycol-based preparation. Thirteen of 102 (12.7%) placebo cases had a dirty or coated cecum (score 4 or 5). In the loperamide group, this number was 2 of 98 (2.0%; p = 0.0041). Nineteen placebo cases (18.6%) had an overall fair/poor preparation in comparison with 9 of the loperamide group (9.2%; p = 0.0543). Days to first bowel movement were 2.4 ± 1.1 for loperamide and 2.5 ± 1.7 for placebo (p = 0.7224). Fifty-eight percent of loperamide patients had polyps, and 74% of these patients had multiple polyps; 67% of placebo patients had polyps, but only 54% of these patients had multiple polyps (vs loperamide, p = 0.0183). CONCLUSION: Judicious use of loperamide can significantly improve the quality of cecal preparation and may increase polyp yield.

Mortality of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis: a single center review of 154 patients.

INTRODUCTION: Intra-abdominal desmoid tumors are one of the leading causes of death in patients with familial adenomatous polyposis. Their behavior is unpredictable and their biology is poorly understood, accounting for the lack of a standardized medical and surgical approach. The aim of this study was to evaluate the mortality rate of patients with intra-abdominal desmoid tumors and to identify prognostic factors for the evolution of the disease. MATERIALS AND METHODS: A total of 154 patients with intra-abdominal desmoid tumors were included in the study. Each tumor was staged and each patient was categorized according to the stage of their most advanced tumor. Mortality was analyzed and the univariate risk factors associated with survival were included in a multivariable Cox regression model. A scoring system was derived from the multivariate analysis to refine outcomes within stages. RESULTS: Five-year survival of patients with stage I, II, III, and IV intra-abdominal desmoid tumor were 95%, 100%, 89%, and 76% respectively (P < 0.001). Severe pain/narcotic dependency, tumor size larger than 10 cm, and need for total parenteral nutrition were shown to further define survival within stages. Five-year survival rate of stage IV patient with all of the above-mentioned risk factors was only 53%. CONCLUSIONS: Our study confirmed the validity of the staging system to predict mortality in patients with intra-abdominal desmoid tumors and identified additional risk factors able to better define the risk of death within each stage. Risk stratification is crucial in directing patients with advanced disease and poor prognosis to the most appropriate medical and surgical options.

Permanent ostomy after ileoanal pouch failure: pouch in situ or pouch excision?

BACKGROUND: The risks and benefits of pouch excision and end ileostomy creation when compared to the alternative option of a permanent diversion with the pouch left in situ when restoration of intestinal continuity is not pursued for patients who develop pouch failure after IPAA have not been well characterized. OBJECTIVE: This study aimed to compare the early and long-term outcomes after permanent diversion with the pouch left in situ vs pouch excision with end ileostomy creation for pouch failure. DESIGN: This study is a retrospective review of prospectively gathered data. SETTINGS: This investigation was conducted at a tertiary center. PATIENTS: Patients with pouch failure who underwent a permanent ileostomy with the pouch left in situ and those who underwent pouch excision were included in the study. MAIN OUTCOME MEASURES: The primary outcomes measured were the perioperative outcomes and quality of life using the pouch and Short Form 12 questionnaires. RESULTS: One hundred thirty-six patients with pouch failure underwent either pouch left in situ (n = 31) or pouch excision (n = 105). Age (p = 0.72), sex (p = 0.72), ASA score (p = 0.22), BMI (p = 0.83), disease duration (p = 0.74), time to surgery for pouch failure (p = 0.053), diagnosis at pouch failure (p = 0.18), and follow-up (p = 0.76) were similar. The predominant reason for pouch failure was septic complications in 15 (48.4%) patients in the pouch left in situ group and 39 (37.1%) patients in the pouch excision group (p = 0.3). Thirty-day complications, including prolonged ileus (p = 0.59), pelvic abscess (p = 1.0), wound infection (p = 1.0), and bowel obstruction (p = 1.0), were similar. At the most recent follow-up (median, 9.9 y), quality of life (p = 0.005) and health (p = 0.008), current energy level (p = 0.026), Cleveland Global Quality of Life score (p = 0.005), and Short Form 12 mental (p = 0.004) and physical (p = 0.014) component scales were significantly higher after pouch excision than after pouch left in situ. Urinary and sexual function was similar between the groups. Anal pain (n = 4) and seepage with pad use (n = 8) were the predominant concerns of the pouch left in situ group on long-term follow-up. None of the 18 patients with pouch in situ, for whom information relating to long-term pouch surveillance was available, developed dysplasia or cancer. LIMITATIONS: This study was limited by its retrospective nature. CONCLUSIONS: Although technically more challenging, pouch excision, rather than pouch left in situ, is the preferable option for patients who develop pouch failure and are not candidates for restoration of intestinal continuity. Because pouch left in situ was not associated with neoplasia, this option is a reasonable intermediate or long-term alternative when pouch excision is not feasible or advisable.