Management of colonic complications of type IV Ehlers-Danlos syndrome: a systematic review and evidence-based management strategy.

AIM: Type IV Ehlers Danlos Syndrome (EDS) is a connective tissue disorder affecting approximately 1 per 100,000-200,000 people. Life expectancy is reduced secondary to spontaneous vascular rupture or colonic perforation. Surgery carries significant morbidity and mortality. While strategies to manage colonic perforation include primary repair with or without a defunctioning stoma, Hartmann's procedure, total abdominal colectomy with end ileostomy and ileorectal anastomosis, evidence is contradictory and has not previously been evaluated in order to form a treatment strategy. We aim to review the published literature and identify outcome data relating to operative management of colonic perforation in type IV EDS. METHODS: Pubmed, EM-BASE, Cochrane library and Google Scholar were searched with the following details: Ehlers Danlos Syndrome AND colonic surgery. The main outcome measure was re-perforation rates following colonic surgery on patients with type IV EDS. If the nature of surgery and follow up were reported, data were recorded in a SPSS database according to PRISMA guidelines. RESULTS: One hundred and nine operations have been described in 51 patients in 44 case series. There were 26 visceral re-perforations, 2 affecting the small intestine and 24 colonic. Survival analysis favoured total abdominal colectomy compared with operations where the colon was left in situ. CONCLUSIONS: Total abdominal colectomy with end ileostomy or ileorectal anastomosis are the safest strategies after colonic perforation in type IV EDS. Anastomotic leak rates are high. End colostomy is high risk for colonic re-perforation and anastomotic leak rates are extremely high. Restoration of colonic continuity should be avoided.

Meta-analysis of the effect of perioperative intravenous lidocaine on return of gastrointestinal function after colorectal surgery.

BACKGROUND: Return of normal gastrointestinal (GI) function is a critical determinant of recovery after colorectal surgery. The aim of this meta-analysis was to evaluate whether perioperative intravenous (IV) lidocaine benefits return of gastrointestinal function after colorectal resection. METHODS: A comprehensive search of Ovid Medline, PubMed, Embase, Cochrane library, and clinicaltrials.org was performed on 1st July 2018. A manual search of reference lists was also performed. Inclusion criteria were as follows: randomized controlled trials (RCTs) of intravenous (IV) lidocaine administered perioperatively compared to placebo (0.9% saline infusion) as part of a multimodal perioperative analgesic regimen, human adults (> 16 years), and open or laparoscopic colorectal resectional surgery. EXCLUSION CRITERIA: non-colorectal surgery, non-placebo comparator, children, non-general anaesthetic, and pharmacokinetic studies. The primary endpoint was time to first bowel movement. Secondary endpoints were time to first passage of flatus, time to toleration of diet, nausea and vomiting, ileus, pain scores, opioid analgesia consumption, and length of stay. RESULTS: One hundred and ninety one studies were screened, with 9 RCTs meeting inclusion criteria (405 patients, four laparoscopic and five open surgery studies). IV lidocaine reduced time to first bowel movement compared to placebo [seven studies, 325 patients, mean weighted difference - 9.54 h, 95% CI 18.72-0.36, p = 0.04]. Ileus, pain scores, and length of stay were reduced with IV lidocaine compared with placebo. CONCLUSIONS: Perioperative IV lidocaine may improve recovery of gastrointestinal function after colorectal surgery. Large-scale effectiveness studies to measure effect size and evaluate optimum dose/duration are warranted.

An evidence-based treatment algorithm for colorectal polyp cancers: results from the Scottish Screen-detected Polyp Cancer Study (SSPoCS).

OBJECTIVES: Colorectal polyp cancers present clinicians with a treatment dilemma. Decisions regarding whether to offer segmental resection or endoscopic surveillance are often taken without reference to good quality evidence. The aim of this study was to develop a treatment algorithm for patients with screen-detected polyp cancers. DESIGN: This national cohort study included all patients with a polyp cancer identified through the Scottish Bowel Screening Programme between 2000 and 2012. Multivariate regression analysis was used to assess the impact of clinical, endoscopic and pathological variables on the rate of adverse events (residual tumour in patients undergoing segmental resection or cancer-related death or disease recurrence in any patient). These data were used to develop a clinically relevant treatment algorithm. RESULTS: 485 patients with polyp cancers were included. 186/485 (38%) underwent segmental resection and residual tumour was identified in 41/186 (22%). The only factor associated with an increased risk of residual tumour in the bowel wall was incomplete excision of the original polyp (OR 5.61, p=0.001), while only lymphovascular invasion was associated with an increased risk of lymph node metastases (OR 5.95, p=0.002). When patients undergoing segmental resection or endoscopic surveillance were considered together, the risk of adverse events was significantly higher in patients with incomplete excision (OR 10.23, p<0.001) or lymphovascular invasion (OR 2.65, p=0.023). CONCLUSION: A policy of surveillance is adequate for the majority of patients with screen-detected colorectal polyp cancers. Consideration of segmental resection should be reserved for those with incomplete excision or evidence of lymphovascular invasion.

Multicentre study of short-course radiotherapy and transanal endoscopic microsurgery for early rectal cancer.

BACKGROUND: Organ-preserving treatment for early-stage rectal cancer may avoid the substantial perioperative morbidity and functional sequelae associated with total mesorectal excision (TME). The initial results of an organ-preserving approach using preoperative short-course radiotherapy (SCRT) and transanal endoscopic microsurgery (TEMS) are presented. METHODS: Patients with cT1-2N0 rectal cancers staged using high-quality MRI and endorectal ultrasonography received SCRT, with TEMS 8-10 weeks later, at four regional referral centres between 2007 and 2013. Patients were generally considered high risk for TME surgery (a small number refused TME). RESULTS: Following SCRT and TEMS, 60 (97 per cent) of 62 patients had an R0 resection. Histopathological staging identified 20 ypT0 tumours, 23 ypT1, 18 ypT2 and one ypT3. Preoperative uT category was significantly associated with a complete pathological response, which was achieved in 13 of 27 patients with uT0/uT1 disease and in five of 29 with uT2 (P = 0·010). Acute complications affected 19 patients, the majority following TEMS. No fistulas occurred and no stomas were formed. Surveillance detected four intraluminal local recurrences at a median follow-up of 13 months, all in patients with tumours staged as ypT2. Salvage TME achieved R0 resection in three patients and a stent was placed in one patient owing to co-morbidities. CONCLUSION: SCRT with TEMS was effective in the majority of patients considered high risk for (or who refused) TME surgery.

Contemporary use of social media by consultant colorectal surgeons.

AIM: There is evidence of significant growth in the engagement of UK health-care professionals with 'open' social media platforms, such as Twitter and LinkedIn. Social media communication provides many opportunities and benefits for medical education and interaction with patients and colleagues. This study was undertaken to evaluate the uptake of public social media membership and the characteristics of use of such media channels amongst contemporary UK consultant colorectal surgeons. METHOD: Colorectal surgeons were identified from the Association of Coloproctology of Great Britain and Ireland (ACPGBI) national registry of colorectal mortality outcomes and were cross-referenced with the General Medical Council (GMC) register. Individuals were identified by manual searching on a number of social media platforms. Matching accounts were then examined to confirm ownership and to evaluate key markers of use. RESULTS: Six-hundred and eighteen individual consultant colorectal surgeons from 142 health authorities were studied (79.5% were ACPGBI members and 90.8% were male). Two-hundred and twenty-nine (37.1%) had LinkedIn profiles (37.7% male surgeons, 29.8% female surgeons; P = 0.2530). LinkedIn membership was significantly higher in ACPGBI members (P < 0.001) and in those with GMC registration before 1997 vs after this date (39% before 1997 vs 30% after 1997; P = 0.03). LinkedIn members had a mean of 62 connections (median = 22), and 19 (3.1%) surgeons had Twitter profiles with a mean of 82 (median = 16; range: 0-914) followers and their accounts were followed by a mean of 87 (median = 27; range: 0-642) persons. CONCLUSION: UK consultant colorectal surgeons are less engaged with social media than reported studies from other health-care professional groups. Further education and appropriate guidance on usage may encourage uptake and confidence, particularly in younger consultants.

Hyperplastic polyps are innocuous lesions in hereditary nonpolyposis colorectal cancers.

Aims. To compare methylation profiles, protein expression, and microsatellite instability (MSI) of sporadic, HNPCC, and familial hyperplastic polyps (HPs). Methods. Methylation-specific PCR (MSP) and pyrosequencing assessed p16, MGMT, hMLH-1, MINT 1, and MINT 31 methylation. IHC (Immunohistochemistry) assessed Ki67, CK20, hMLH-1, hMSH-2, and hMSH-6 protein expression. MSI analysis was performed on those polyps with adequate DNA remaining. Results. 124 HPs were identified 78 sporadic, 21 HNPCC, 25 familial, and the HNPCC group demonstrated no significant differences in overall methylation (P = .186 Chi(2)). The familial group demonstrated significantly less over all methylation levels (P = .004 Chi(2)). Conclusions. HPs that occur in HNPCC have no more worrying features at a molecular level than those patients with HPs in a sporadic setting.

Multicentre review of lymph node harvest in colorectal cancer: are we understaging colorectal cancer patients?

BACKGROUND: Lymph node examination in colorectal cancer is of vital importance for accurate staging. Patients who have fewer nodes examined may be understaged and not offered adjuvant chemotherapy. The national institute of clinical excellence and the association of coloproctology of Great Britain and Ireland both recommend that 12 nodes should be examined for accurate staging. The aim of this study was to assess lymph node harvest at five hospitals in the northwest of England in respect to these guidelines. MATERIALS AND METHODS: This study is a retrospective review of all colorectal cancer resections over a 1-year period at five hospitals. RESULTS: Two hospitals met the national guidelines of a median of 12 or more nodes. Overall, over 50% of colorectal cancers contained fewer than 12 nodes. Fifty-three point seven percent (53.7%) of Dukes B patients did not have 12 nodes in their specimens and may therefore be understaged. There was a significant variation between hospitals in terms of the number of cancers with 12 or more nodes (P < 0.0001) and the number of Dukes B cancers with 12 or more nodes (P < 0.008). CONCLUSION: Over 50% of all colorectal cancer specimens contain fewer than 12 lymph nodes despite clear national guidelines. This is of particular importance to Dukes B cancers where over 53% of cases may be understaged and not offered adjuvant therapy. Significant variation exists between hospitals within the same region.

Who should be followed up after transanal endoscopic resection of rectal tumours?

OBJECTIVE: To determine follow-up requirements following transanal endoscopic microsurgery (TEM) for rectal tumours based on clinical and histopathological assessment of resection specimens. METHOD: A consecutive series of 117 patients undergoing TEM between 1997 and 2005 was studied. The excised specimens were classified as intact with clear surgical resection margins, macroscopically intact specimens with microscopically involved resection margins or piecemeal. Recurrence rates were determined for the three groups. RESULTS: Of the 117 procedures performed, 80 were for benign disease and 37 for malignancy. Within the benign group 39 (49%) resections were intact with clear surgical resection margins and yielded zero recurrences; 22 (27%) resections were macroscopically intact with microscopically involved surgical resection margin and yielded two recurrences; and 19 (24%) resections were piecemeal and yielded eight recurrences. Within the malignant group all 37 patients had resection specimens which were intact with clear surgical resection margins. Two patients had immediate salvage surgery. Of the 35 who went on to long-term follow-up post-TEM (0.6-8.1 years, median 4) four developed recurrent cancer (two local with submucosal disease and two liver metastases). CONCLUSION: For benign rectal neoplasms, resection of an intact specimen with histologically clear surgical resection margins was associated with no observed mucosal recurrence. Local recurrence after TEM is significantly more frequent when histological examination reveals involved margins or when resection is piecemeal. Early endoscopic follow up is required for the latter two groups. Local recurrence for malignant cases was submucosal and detected by palpation.

Desmoid tumours in patients with familial adenomatous polyposis and desmoid region adenomatous polyposis coli mutations.

BACKGROUND: :The aim of this study was to determine the proportion of patients with familial adenomatous polyposis (FAP) who had mutations in the desmoid region of the adenomatous polyposis coli (APC) gene that phenotypically expresses desmoid disease, and to determine the role for surgery in these patients. METHODS: Data from the North West Region FAP database and case notes were analysed retrospectively. RESULTS: Of 363 patients with FAP, 47 from ten families had APC mutations in the desmoid region 3' to codon 1399. Of 22 patients undergoing surgery, 16 developed desmoids, and of these 12 had mesenteric desmoid disease. Complications from mesenteric desmoids were death (two patients), enterectomy (three), local resection (three), fistula (one), cholangitis and local resection (one), bowel obstruction (one) and bowel and ureteric obstruction (one). Preoperative polyp burden ranged from 0 to 100 in eight patients (median age 24.5 (range 16-39) years) and more than 100 in seven (median age 39 (range 31-64) years). One patient had no record of polyp burden. CONCLUSION: In individuals with 3' APC mutations, abdominal surgery is associated with a 65 per cent risk of developing mesenteric desmoids. An alternative strategy might be to attempt to manage the polyps endoscopically.

Management of duodenal involvement in locally advanced colonic carcinoma.

Removal of locally advanced right-sided colonic carcinoma involving the duodenum can be challenging. There are few data on the optimal surgical approach. Adjacent organ involvement is associated with a poor prognosis and may be classified as inoperable with patients having palliative bypass procedures rather than primary resection. Survival is however improved after en bloc resection of adjoining viscera compared with intestinal bypass. We present a variety of surgical options depending on the extent of local invasion and the debility of the patient.

Inherited association of breast and colorectal cancer: limited role of CHEK2 compared with high-penetrance genes.

We assessed the association between breast cancer (BC) and colorectal cancer (CRC) from referral pattern to the Regional Genetics Service including molecular analysis. Hospital computer records and/or department referral books were used to identify cases referred to the Regional Genetic Service during a 16-year period (1990-2005 inclusive). All files were reviewed along with associated demographic data, risk assessments, referral details and results from mutation testing. Families were assessed for hereditary breast and colorectal cancer (HBCC) criteria, and all families with eligible individuals were tested for the 1100delC mutation in CHEK2. A total of 8,612 families were identified. One hundred and sixteen of 1,631 (7.5%) families with a primary referral for CRC fulfilled the criteria for HBCC, whereas only 68/6981 (1%) BC referrals fulfilled these criteria. Blood samples were obtained from 113 individuals from 83/184 families. Only 1/113 (1%) has screened positive for the CHEK2 mutation, whereas 14 (17%) families segregate BRCA1/2 mutations and at least 7 (8.5%) carry MLH1/MSH2 mutations. HBCC syndrome, if it exists as a separate entity, is not likely to be due to CHEK2 mutations. Many families are explicable by existing high-penetrance genes, and further work is necessary to elucidate whether the remainder is due to chance or as yet undiscovered genes.

Health perceptions and lifestyles of the elderly.

Adoption of a healthy lifestyle can slow physical decline and improve well being of the elderly. The purpose of this study was to examine the relationship of six aspects of a healthy lifestyle (nutrition, exercise, stress management, self actualization, health responsibility, and interpersonal support) with selected demographic variables, perceived health status, and health locus of control in the well elderly. The sample consisted of 297 volunteers. Person product-moment correlations and stepwise multiple regression procedures revealed that perceived health status and health locus of control were significant predictors of healthy lifestyles. Demographic variables, posited as modifying factors, were found to be associated with perceived health and locus of control but were not predictive of lifestyle practices.