Composite Functional and Quality of Life Outcomes in Adults Operated for Cloacal Malformation.

INTRODUCTION: Composite long-term outcomes of cloacal malformations remain unclear. We aimed to evaluate bowel and bladder control, sexual function, reproductive health, and quality of life in adulthood. METHODS: A Nordic multi-center cross-sectional observational study evaluating the outcome of adult patients with cloacal malformations was performed. Patient characteristics were retrieved from case records. Established questionnaires were sent to the patients to evaluate bowel- bladder- and sexual function and quality of life. RESULTS: Thirty-four of 48 (70%) eligible patients with median age 28 years (18-45) responded. Eight (24%) patients had a common channel >3 cm. Imaging-confirmed spinal and sacral abnormalities were present in 4 and 17 patients respectively. 85% (n = 29/34, four patients with permanent urinary diversions after bladder neck closure excluded) reported no urinary leakage without physical activity or urge to urinate. Three patients had a permanent enterostomy. 42 % (n = 11/26) reported acceptable bowel function according to Bowel Function Score (with eventual ongoing bowel management). The median Profile of Female Sexual Function Score was below normative values. Quality of life was comparable to the reference population, but thirty percent scored within the distress domains. Eight patients (24%) had been pregnant (15 pregnancies), resulting in seven live births, but the need of assisted reproduction techniques (40%, n = 6/15) and miscarriage (40%, n = 6/15) was common. CONCLUSIONS: Adequate spontaneous bowel control was rare, while most patients were dry for urine without additional procedures. Cloacal malformation also have a negative impact on sexual function health related quality of life and reproductive health. Long-term follow-up is crucial, not only regarding bowel and bladder function, but also for sexual and reproductive function, which may be important negative factors for health-related quality of life. LEVEL OF EVIDENCE: Level IV.

Gastrointestinal manifestations in patients with gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): a systematic review with analysis of individual patient data.

BACKGROUND AND AIM: Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) is an autosomal dominant syndrome characterized by fundic gland polyps (FGP) as well as an increased risk of gastric cancer. The syndrome has been recognized as a clinical entity for less than a decade. A clinical suspicion may be complex and can vary from incidental findings of FGPs at gastroscopy to obstructive symptoms with dyspepsia and vomiting. The diagnosis is established by genetic detection of a pathogenic variant in the promotor 1B region of the APC gene. As of yet there are no established clinical criteria for the diagnosis. To increase knowledge of the condition and to discuss possible genetic testing and surveillance strategies, we performed a systematic review of all reported patients with GAPPS. METHODS: This review was organized according to PRISMA guidelines. The search, which was conducted on September 7th, 2023, was applied to MEDLINE and restricted to only humans and papers in the English language. Only the studies on patients/families with GAPPS verified by identification of a pathogenic variant in the APC promoter 1B were included. RESULTS: Twelve publications with a total of 113 patients were identified. In all instances the diagnosis was genetically verified with reports of four different variants within the APC promotor 1B region. Eighty-eight patients (90.1%) had gastric polyps, of these seven patients had low-grade dysplasia and five patients had both low- and high-grade dysplasia. Thirty-seven patients (45.7%) underwent gastrectomy. There were no reports of duodenal polyps (0%). Gastric cancer was found in 31 patients (30.1%) with a median age of 48 years (range 19-75). Twenty-six patients died (23.2%) of which 19 had developed gastric cancer (73.1%). One patient was diagnosed with metastatic colorectal cancer (2.2%) and died at 73 years of age. Nineteen patients had colorectal manifestations with < 20 polyps (41.3%). CONCLUSION: Patients with a pathogenic variant in the APC promoter 1B region have an increased risk of gastric polyposis and early-onset gastric cancer. However, there is considerable variation in clinical expression and penetrance, which makes decisions on surveillance and the timing of prophylactic gastrectomy challenging.

Routine postoperative CT to detect anastomotic leakage after low anterior resection for rectal cancer has a low sensitivity and specificity and a poor interobserver agreement.

AIM: To compare the accuracy and interobserver variation of routine computed tomography (CT) on postoperative day 6-8 to detect anastomotic leakage (AL) verified by re-operation and/or endoscopy. A secondary objective was to identify the predictive values of different CT findings as an indicator for AL. MATERIAL AND METHODS: The material for this study originates from two previous prospective multicentre studies including 277 patients who were scheduled for routine abdominal CT postoperative day 6-8. Inclusion criteria for the present study were routine CT without contrast medium followed by CT with rectal contrast medium. Two independent senior radiologists blinded to the clinical outcome reviewed the CT examinations for specific findings according to a predefined scheme. RESULTS: A total of 52 patients fulfilled the inclusion criteria. AL occurred in 14 patients of which nine were clinical and five subclinical. The two radiologists diagnosed AL at unenhanced CT with sensitivities of 71.4% and 50%, respectively, and of 57.1% and 35.7% with rectal contrast medium. The corresponding specificities were 55.3% and 81.6%, and 94.7% and 92.1%. Peri-anastomotic free air and contrast medium leakage had the highest odds ratios for AL. CONCLUSION: The diagnostic sensitivity and specificity of routine postoperative CT to detect AL after low anterior resection for rectal cancer is low and with considerable interobserver variation.

Postoperative complications and waiting time for surgical intervention after radiologically guided drainage of intra-abdominal abscess in patients with Crohn's disease.

BACKGROUND: In patients with active Crohn's disease (CD), treatment of intra-abdominal abscess usually comprises antibiotics and radiologically guided percutaneous drainage (PD) preceding surgery. The aim of this study was to investigate the risk of postoperative complications and identify the optimal time interval for surgical intervention after PD. METHODS: A multicentre, international, retrospective cohort study was carried out. Details of patients with diagnosis of CD who underwent ultrasonography- or CT-guided PD were retrieved from hospital records using international classification of disease (ICD-10) diagnosis code for CD combined with procedure code for PD. Clinical variables were retrieved and the following outcomes were measured: 30-day postoperative overall complications, intra-abdominal septic complications, unplanned intraoperative adverse events, surgical-site infections, sepsis and pathological postoperative ileus, in addition to abscess recurrence. Patients were categorized into three groups according to the length of the interval from PD to surgery (1-14 days, 15-30 days and more than 30 days) for comparison of outcomes. RESULTS: The cohort comprised 335 CD patients with PD followed by surgery. Median age was 33 (i.q.r. 24-44) years, 152 (45.4 per cent) were females, and median disease duration was 9 (i.q.r. 3.6-15) years. Overall, the 30-day postoperative complications rate was 32.2 per cent and the mortality rate was 1.5 per cent. After adjustment for co-variables, older age (odds ratio 1.03 (95 per cent c.i. 1.01 to 1.06), P < 0.012), residual abscess after PD (odds ratio 0.374 (95 per cent c.i. 0.19 to 0.74), P < 0.014), smoking (odds ratio 1.89 (95 per cent c.i. 1.01 to 3.53), P = 0.049) and low serum albumin concentration (odds ratio 0.921 (95 per cent c.i. 0.89 to 0.96), P < 0.001) were associated with higher rates of postoperative complications. A short waiting interval, less than 2 weeks after PD, was associated with a high incidence of abscess recurrence (odds ratio 0.59 (95 per cent c.i. 0.36 to 0.96), P = 0.042). CONCLUSION: Smoking, low serum albumin concentration and older age were significantly associated with postoperative complications. An interval of at least 2 weeks after successful PD correlated with reduced risk of abscess recurrence.

Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood.

BACKGROUND: Surgery for total colonic aganglionosis (TCA) is designed to preserve continence and achieve satisfactory quality of life. This study evaluated a comprehensive group of clinical and social outcomes. METHODS: An international multicentre study from eight Nordic hospitals involving examination of case records and a patient-reported questionnaire survey of all patients born with TCA between 1987 and 2006 was undertaken. RESULTS: Of a total of 116 patients, five (4·3 per cent) had died and 102 were traced. Over a median follow-up of 12 (range 0·3-33) years, bowel continuity was established in 75 (73·5 per cent) at a median age of 11 (0·5-156) months. Mucosectomy with a short muscular cuff and straight ileoanal anastomosis (SIAA) (29 patients) or with a J pouch (JIAA) (26) were the most common reconstructions (55 of 72, 76 per cent). Major early postoperative complications requiring surgical intervention were observed in four (6 per cent) of the 72 patients. In 57 children aged over 4 years, long-term functional bowel symptoms after reconstruction included difficulties in holding back defaecation in 22 (39 per cent), more than one faecal accident per week in nine (16 per cent), increased frequency of defaecation in 51 (89 per cent), and social restrictions due to bowel symptoms in 35 (61 per cent). Enterocolitis occurred in 35 (47 per cent) of 72 patients. Supplementary enteral and/or parenteral nutrition was required by 51 (55 per cent) of 93 patients at any time during follow-up. Of 56 responders aged 2-20 years, true low BMI for age was found in 20 (36 per cent) and 13 (23 per cent) were short for age. CONCLUSION: Reconstruction for TCA was associated with persistent bowel symptoms, and enterocolitis remained common. Multidisciplinary follow-up, including continuity of care in adulthood, might improve care standards in patients with TCA.

ANTECEDENTES: La cirugía de la aganglionosis colónica total (total colonic aganglionosis, TCA) está diseñada para preservar la continencia y lograr una calidad de vida satisfactoria. Este estudio evaluó un gran número de resultados clínicos y sociales. MÉTODOS: Se realizó un estudio internacional multicéntrico en ocho hospitales nórdicos en el que se incluyeron las historias clínicas de todos los pacientes nacidos con TCA entre 1987 y 2006. Se invitó a los pacientes y a sus cuidadores a responder una encuesta sobre la función intestinal, el crecimiento y las necesidades nutricionales, así como la repercusión social de la enfermedad a largo plazo. RESULTADOS: De un total de 116 pacientes, 5 (4,3%) habían fallecido y 102 respondieron la encuesta. Con una mediana de seguimiento de 12 años (rango 0,5-33), se había restablecido la continuidad intestinal en 75/102 (74%) a una mediana de edad de 11 meses (0,5-156). La mucosectomía con un manguito muscular corto y anastomosis ileoanal directa (short muscular cuff and straight ileo-anal anastomosis, SIAA) n = 29 o con reservorio en J (JIAA) n = 26 (55/72, 76%) fueron las reconstrucciones más habituales. Las complicaciones postoperatorias precoces que precisaron una intervención quirúrgica fueron muy poco frecuentes, pero se presentaron en 4/72 (5%) pacientes. Los síntomas a largo plazo relacionados con la función intestinal tras la reconstrucción, valorados en 57 niños mayores de cuatro años, fueron la dificultad para retener la defecación en 14 (25%), la encopresis en 21 (37%), las pérdidas fecales > 1/semana en 9 (16%) y el aumento de la frecuencia de defecación en 51 (89%). A largo plazo, se desarrolló una enterocolitis en 35/72 (47%) pacientes. Se precisó de nutrición enteral y/o parenteral suplementaria en algún momento del período de seguimiento en 51/93 (55%) pacientes. En los pacientes que respondieron a la encuesta entre 2 y 20 años (n = 56) de edad, se detectó un índice de masa corporal menor en 20 (35%) y una altura baja en 13 (23%) para su edad. En 35/57 (61%) pacientes mayores de 4 años con restauración del tránsito intestinal había restricciones sociales debido a los síntomas intestinales, de los que en 10 (17%) casos fueron moderadas o graves. CONCLUSIÓN: La reconstrucción de una TCA se asocia con síntomas intestinales persistentes y la enterocolitis sigue siendo frecuente. Un seguimiento multidisciplinario, incluso en la edad adulta, podría mejorar los resultados en la cirugía de la TCA.

Levels of Intestinal Inflammation and Fibrosis in Resection Specimens after Preoperative Anti-Tumor Necrosis Factor Alpha Treatment in Patients with Crohn's Disease: A Comparative Pilot Study.

BACKGROUND: Strictures are a common complication in Crohn's disease (CD), found in more than 50% of patients. They are characterized by the excessive deposition of extracellular proteins in the tissue as a result of the chronic inflammatory process. The effect of anti-tumor necrosis factor alpha (TNF-α) therapy on the development of fibrosis is not yet fully understood. AIM: To investigate whether the degree of intestinal inflammation and fibrosis is correlated with preoperative anti-TNF-α) therapy on the development of fibrosis is not yet fully understood. METHODS: This unblinded, prospective, single tertiary center, pilot cohort study included all adult patients with CD who underwent elective, laparoscopic, or open intestinal resection. Preoperative investigations included measurement of blood TNF-α) therapy on the development of fibrosis is not yet fully understood. RESULTS: Histopathological specimens from 10 patients with CD who underwent ileocecal or ileocolic resections were retrieved. Four of those patients were on anti-TNF-α) therapy on the development of fibrosis is not yet fully understood. α) therapy on the development of fibrosis is not yet fully understood. α) therapy on the development of fibrosis is not yet fully understood. p=0.01). Anti-TNF-α) therapy on the development of fibrosis is not yet fully understood. α) therapy on the development of fibrosis is not yet fully understood. α) therapy on the development of fibrosis is not yet fully understood. CONCLUSIONS: Patients who underwent preoperative anti-TNF-α treatment had a higher fibrosis score than controls.α) therapy on the development of fibrosis is not yet fully understood.

Detection of early anastomotic leakage by intraperitoneal microdialysis after low anterior resection for rectal cancer: a prospective cohort study.

AIM: Anastomotic leakage (AL) is a common and serious complication following sphincter-preserving surgery for rectal cancer. Early detection and intervention can improve clinical outcomes. The aim of this prospective cohort study was to compare intraperitoneal microdialysis with a clinical scoring system for early detection of AL. METHOD: A microdialysis catheter was anchored near the anastomosis at low anterior resection (LAR) for rectal cancer. Peritoneal fluid samples were analysed (lactate, pyruvate, glucose and glycerol concentration) 4-hourly and compared with a daily clinical leak score (DULK = Dutch leakage). At day 7 a pelvic CT with rectal contrast enema was performed to establish if there had been a radiological leak. RESULTS: In this two-centre study, 129 patients [median age 65 (26-82) years; 60.5% male] underwent LAR. The leak rate was 27% (grade A, n = 11; grade B, n = 12; grade C, n = 12). Receiver operator characteristic analysis demonstrated a lactate cut-off value of 9.8 mm and had 77% sensitivity, 82% specificity, 78% accuracy, a positive predictive value (PPV) of 58, a negative predictive value (NPV) of 88 (CI 79-94) and an area under the curve (AUC) of 0.9 for AL. This compared with a clinical score ≥ 4, which had 57% sensitivity, 79% specificity, 71% accuracy, a PPV of 46, a NPV of 82 and an AUC of 0.7 for AL. The mean day for a positive test when using delta lactate ≥ 6.3 mm was 1.6 days and for leak score ≥ 4 it was 3.3 days (NS). CONCLUSION: When AL occurs, intraperitoneal lactate concentration increases over time, and at a certain cut-off has a higher sensitivity, specificity, accuracy, PPV and NPV than a clinical scoring system.

Colon capsule endoscopy for colonic surveillance.

AIM: Resources used in surveillance colonoscopies are taking up an increasing proportion of colonoscopy capacity. Colon capsule endoscopy (CCE) is a promising technique for noninvasive investigation of the colon. We aimed to investigate CCE as a possible filter in colonic surveillance with the primary outcome of reducing the number of colonoscopies. METHOD: Patients scheduled for follow-up colonoscopy were subjected to a primary CCE and only supplemental conventional endoscopy if significant pathology was detected or if the CCE examination was incomplete. Significant pathology was defined as more than two small polyps, or one polyp greater than 9 mm or any polyp in patients with hereditary nonpolyposis colorectal cancer. Supplemental endoscopy was carried out to the extent needed to resect the detected polyps and investigate the parts of the colon that were not sufficiently visualized by the capsule. RESULTS: A total of 180 patients were included. Seventy-seven patients (43%) had a complete CCE with no significant findings. A complete colonoscopy was carried out in 67 patients (37%) and 36 patients (20%) underwent a sigmoidoscopy. In the 103 patients undergoing endoscopy, 59 (57%) had no adenomas detected, 33 (32%) had 'low-risk' adenomas and 11 (11%) had 'high-risk' adenomas. CONCLUSION: The introduction of CCE as filter test in colonic surveillance reduced colonoscopies by 43%, but this implies that untreated polyps are left behind and is not cost-effective. The CCE completion rate must be improved.

Influence of Prior Psychiatric Disorders on the Treatment Course of Gynaecological Cancer - A Nationwide Cohort Study.

AIMS: To examine the influence of pre-existing psychiatric disorder on the choice of treatment in patients with gynaecological cancer. MATERIALS AND METHODS: The analyses were based on all patients who underwent surgical treatment for endometrial, ovarian or cervical cancer who were registered in the Danish Gynecological Cancer Database in the years 2007-2014 (3059 patients with ovarian cancer, 5100 patients with endometrial cancer and 1150 with cervical cancer). Logistic regression model and Cox regression model, adjusted for relevant confounders, were used to estimate the effect of pre-existing psychiatric disorder on the course of cancer treatment. Our outcomes were (i) presurgical oncological treatment, (ii) macroradical surgery for patients with ovarian cancer, (iii) radiation/chemotherapy within 30 days and 100 days after surgery and (iv) time from surgery to first oncological treatment. RESULTS: In the group of patients with ovarian cancer, more patients with a psychiatric disorder received macroradical surgery versus patients without a psychiatric disorder, corresponding to an adjusted odds ratio of 1.24 (95% confidence interval 0.62-2.41) and the chance for having oncological treatment within 100 days was odds ratio = 1.26 (95% confidence interval 0.77-2.10). As for patients with endometrial cancer, all outcome estimates were close to unity. The adjusted odds ratio for oncological treatment within 30 days after surgery in patients with cervical cancer with a history of psychiatric disorder was 0.20 (95% confidence interval 0.03-1.54). CONCLUSIONS: We did not find any significant differences in the treatment of ovarian and endometrial cancer in patients with pre-existing psychiatric diagnoses. When it comes to oncological treatment, we suggest that increased attention should be paid to patients with cervical cancer having a pre-existing psychiatric diagnosis.

Back-to-back colon capsule endoscopy and optical colonoscopy in colorectal cancer screening individuals.

AIM: The aim was to determine the polyp detection rate and per-patient sensitivity for polyps > 9 mm of colon capsule endoscopy (CCE) compared with colonoscopy as well as the diagnostic accuracy of CCE. METHOD: Individuals who had a positive immunochemical faecal occult blood test during screening had investigator blinded CCE and colonoscopy. Participants underwent repeat endoscopy if significant lesions detected by CCE were considered to have been missed by colonoscopy. RESULTS: There were 253 participants. The polyp detection rate was significantly higher in CCE compared with colonoscopy (P = 0.02). The per-patient sensitivity for > 9 mm polyps for CCE and colonoscopy was 87% (95% CI: 83-91%) and 88% (95% CI: 84-92%) respectively. In participants with complete CCE and colonoscopy examinations (N = 126), per-patient sensitivity of > 9 mm polyps in CCE (97%; 95% CI: 94-100%) was superior to colonoscopy (89%; 95% CI: 84-94%). A complete capsule endoscopy examination (N = 134) could detect patients with intermediate or greater risk (according to the European guidelines) with an accuracy, sensitivity, specificity and positivity rate of 79%, 93%, 69% and 58% respectively, using a cut-off of at least one polyp > 10 mm or more than two polyps. CONCLUSION: CCE is superior to colonoscopy in polyp detection rate and per-patient sensitivity to > 9 mm polyps, but only in complete CCE examinations. The rate of incomplete CCE examinations must be improved.

Pouch failures following ileal pouch-anal anastomosis for ulcerative colitis.

AIM: Ileal pouch-anal anastomosis is a procedure offered to patients with ulcerative colitis who opt for restoration of bowel continuity. The aim of this study was to determine the risk of pouch failure and ascertain the risk factors associated with failure. METHOD: The study included 1991 patients with ulcerative colitis who underwent ileal pouch-anal anastomosis in Denmark in the period 1980-2013. Pouch failure was defined as excision of the pouch or presence of an unreversed stoma within 1 year after its creation. We used Cox proportional hazards regression to explore the association between pouch failure and age, gender, synchronous colectomy, primary faecal diversion, annual hospital volume (very low, 1-5 cases per year; low, 6-10; intermediate 11-20; high > 20), calendar year, laparoscopy and primary sclerosing cholangitis. RESULTS: Over a median 11.4 years, 295 failures occurred, corresponding to 5-, 10- and 20-year cumulative risks of 9.1%, 12.1% and 18.2%, respectively. The risk of failure was higher for women [adjusted hazard ratio (aHR) 1.39, 95% CI 1.10-1.75]. Primary non-diversion (aHR 1.63, 95% CI 1.11-2.41) and a low hospital volume (aHR, very low volume vs high volume 2.30, 95% CI 1.26-4.20) were also associated with a higher risk of failure. The risk of failure was not associated with calendar year, primary sclerosing cholangitis, synchronous colectomy or laparoscopy. CONCLUSION: In a cohort of patients from Denmark (where pouch surgery is centralized) with ulcerative colitis and ileal pouch-anal anastomosis, women had a higher risk of pouch failure. Of modifiable factors, low hospital volume and non-diversion were associated with a higher risk of pouch failure.

Vacuum-Assisted Abdominal Closure Is Safe and Effective: A Cohort Study in 74 Consecutive Patients.

BACKGROUND: Vacuum-assisted closure (VAC) has, in many instances, become the treatment of choice in patients with abdominal catastrophes. This study describes the use and outcome of ABThera KCI® VAC in the Region Southern Denmark covering a population of approximately 1.202 mill inhabitants. METHOD: A prospective multicenter study including all patients treated with VAC during an eleven-month period. RESULTS: A total of 74 consecutive patients were included. Median age was 64.4 (9-89) years, 64% were men, and median body mass index was 25 (17-42). Duration of VAC treatment was median 4.5 (0-39) days with median 1 (0-16) dressing changes. Seventy per cent of the patients attended the intensive care unit. The 90-day mortality was 15%. A secondary closure of the fascia was obtained in 84% of the surviving patients. Only one patient developed an enteroatmospheric fistula. Patients with secondary closure were less likely to develop large hernias and had better self-evaluated physical health score (p < 0,05). No difference in mental health was found. CONCLUSION: The abdominal VAC treatment in patients with abdominal catastrophes is safe and with a relative low complication rate. Whether it might be superior to conventional treatment with primary closure when possible has yet to be proven in a randomized study.

Randomized clinical trial of laparoscopic ultrasonography before laparoscopic colorectal cancer resection.

BACKGROUND: Intraoperative ultrasonography during open surgery for colorectal cancer may be useful for the detection of unrecognized liver metastases. Laparoscopic ultrasonography (LUS) for the detection of unrecognized liver metastasis has not been studied in a randomized trial. This RCT tested the hypothesis that LUS would change the TNM stage and treatment strategy. METHODS: Patients with colorectal cancer and no known metastases were randomized (1 : 1) to laparoscopic examination (control or laparoscopy plus LUS) in three Danish centres. Neither participants nor staff were blinded to the group assignment. RESULTS: Three hundred patients were randomized, 150 in each group. After randomization, 43 patients were excluded, leaving 128 in the control group and 129 in the LUS group. Intraoperative T and N categories were not altered by LUS, but laparoscopy alone identified previously undetected M1 disease in one patient (0·8 per cent) in the control group and three (2·3 per cent) in the LUS group. In the latter group, LUS suggested that an additional six patients (4·7 per cent) had M1 disease with liver (4) or para-aortal lymph node (2) metastases. The change in treatment strategy was greater in the LUS than in the control group (7·8 (95 per cent c.i. 3·8 to 13·8) and 0·8 (0 to 4·2) per cent respectively; P = 0·010), but the suspected M1 disease was benign in half of the patients. CONCLUSION: Routine LUS during resection of colorectal cancer is not recommended. Registration number: NCT02079389 (http://www.clinicaltrials.gov).

Postoperative complications following percutaneous endoscopic gastrostomy are common in children.

AIM: Inserting a feeding tube using percutaneous endoscopic gastrostomy may be necessary to ensure that children with eating problems receive sufficient enteral nutrition. The aim of this study was to investigate the perioperative and postoperative complications of percutaneous endoscopic gastrostomy when the pull-through method was the standard procedure. METHODS: This was a retrospective review of 229 children (50.7% male) who underwent a gastrostomy procedure at Odense University Hospital, Denmark, from January 1, 2000 to December 31, 2012. The median age of the children was 1.6 years (range: 0-14.9), and the follow-up period was 36 months. Complications were graded according to the Clavien-Dindo classification. RESULTS: A total of 167 postoperative complications occurred in 118 of the 229 patients (51.5%). Of these, 89 were grade 1 complications, 49 were grade 2 complications, and 29 were grade 3b complications. No gastrostomy-related deaths were observed, and no single preoperative risk factor was identified. Perioperative complications were experienced by 2.6% of the patients. CONCLUSION: Gastrostomy feeding tube placement was associated with a high rate of postoperative complications of various grades when the pull-through method was the standard procedure. A consensus on how to report and grade complications arising from this procedure is warranted.

Disease pattern in Danish patients with Peutz-Jeghers syndrome.

PURPOSE: In this paper, we aimed to collect genetic and medical information on all Danish patients with Peutz-Jeghers syndrome (PJS), in order to contribute to the knowledge of phenotype and genotype. Peutz-Jeghers syndrome is a hereditary syndrome characterized by multiple hamartomatous polyps in the GI tract, mucocutaneous pigmentations, and an increased risk of cancer in the GI tract and at extraintestinal sites. Over 90 % of patients harbour a pathogenic mutation in STK11. METHODS: Based on the Danish Pathology Data Bank, the Danish National Patient Register, as well as information from relevant departments at Danish hospitals, we identified patients and collected clinical and genetic information. RESULTS: We identified 43 patients of which 14 were deceased. The prevalence was estimated to be ∼1 in 195,000 individuals. The median age at first symptom was 27.5 with invagination of the small bowel as the most frequent presenting symptom. We noted 18 occurrences of cancer at various anatomical sites, including a case of thyroid cancer and penile cancer. Eight of the deceased patients had died of cancer. Eighteen different mutations in STK11 had been detected in 28 patients. CONCLUSION: This is the first comprehensive study of patients with Peutz-Jeghers syndrome in the Danish population identified from nationwide registers and databases. We have demonstrated that the expressivity of Peutz-Jeghers syndrome varies greatly among the patients, even within the same families, underlining the great phenotypic spectrum. Patients with PJS should be offered surveillance from childhood in order to prevent morbidity and reduce mortality.

JP-HHT phenotype in Danish patients with SMAD4 mutations.

Patients with germline mutations in SMAD4 can present symptoms of both juvenile polyposis syndrome (JPS) and hereditary hemorrhagic telangiectasia (HHT): the JP-HHT syndrome. The complete phenotypic picture of this syndrome is only just emerging. We describe the clinical characteristics of 14 patients with SMAD4-mutations. The study was a retrospective, register-based study. SMAD4 mutations carriers were identified through the Danish HHT-registry, the genetic laboratories - and the genetic departments in Denmark. The medical files from relevant departments were reviewed and symptoms of HHT, JPS, aortopathy and family history were noted. We detected 14 patients with SMAD4 mutations. All patients had polyps removed and 11 of 14 fulfilled the diagnostic criteria for JPS. Eight patients were screened for HHT-symptoms and seven of these fulfilled the Curaçao criteria. One patient had aortic root dilation. Our findings support that SMAD4 mutations carriers have symptoms of both HHT and JPS and that the frequency of PAVM and gastric involvement with polyps is higher than in patients with HHT or JPS not caused by a SMAD4 mutation. Out of eight patients screened for aortopathy, one had aortic root dilatation, highlighting the need for additional screening for aortopathy.

Poor Outcomes of Complicated Pouch-Related Fistulas after Ileal Pouch-Anal Anastomosis Surgery.

BACKGROUND AND AIMS: Development of a pouch-related fistula tract is an uncommon but highly morbid complication to restorative proctocolectomy with ileal pouch-anal anastomosis. Pouch failure with permanent ileostomy is reported in 21%-30% of patients, yet the factors contributing to pouch excision remain poorly defined. The aim of this study was to determine the incidence and treatment results of complicated pouch-related fistula, as well as to evaluate factors involved in excision after pouch failure. MATERIAL AND METHODS: The study was conducted as a retrospective study. All patients with diagnosed pouch-related fistulas were registered with information related to fistula classification, treatments, and outcome. RESULTS AND CONCLUSION: The final analysis included 48 (10.7%) of the 447 total ileal pouch-anal anastomosis patients with complicated pouch-related fistulas. Pouch-vaginal fistulas, pouch-perianal fistulas, and other pouch-related fistulas were observed in 19 (63%), 29 (60%), and 10 (21%) patients, respectively, corresponding to an accumulated risk of 8%, 6%, and 2%, respectively. Time from ileal pouch-anal anastomosis surgery to fistula presentation was 24 (0.2-212) months. Overall pouch failure, defined as pouch excision or a diverting stoma, was seen in 34 (71%) patients, while pouch excision was seen in 23 (48%) of the patients. Patients who developed Crohn's disease had a significantly higher risk of pouch excision, as did patients with an early onset of the fistula after ileal pouch-anal anastomosis (P = 0.006 and P = 0.007, respectively). In conclusion, the present study demonstrated a high risk of pouch failure in patients with complicated pouch-related fistulas. Furthermore, it showed that Crohn's disease and the development of early onset fistulas are associated with pouch excision.

Leakage after Surgery for Rectum Cancer: Inconsistency in Reporting to the Danish Colorectal Cancer Group.

Purpose. Anastomotic leakage accounts for up to 1/3 of all fatalities after rectal cancer surgery. Evidence suggests that anastomotic leakage has a negative prognostic impact on local cancer recurrence and long-term cancer specific survival. The reported leakage rate in 2011 in Denmark varied from 7 to 45 percent. The objective was to clarify if the reporting of anastomotic leakage to the Danish Colorectal Cancer Group was rigorous and unequivocal. Methods. An Internet-based questionnaire was e-mailed to all Danish surgical departments, who reported to Danish Colorectal Cancer Group (DCCG) in 2011. There were 23 questions. Four core questions were whether pelvic collection, fecal appearance in a pelvic drain, rectovaginal fistula, and "watchfull" waiting patients were reported as anastomotic leakage. Results. Fourteen out of 17 departments, who in 2011 according to DDCG performed rectal cancer surgery, answered the questionnaire. This gave a response rate of 82%. In three of four core questions there was disagreement in what should be reported as anastomotic leakage. Conclusion. The reporting of anastomotic leakage to the Danish Colorectal Cancer Group was not rigorous and unequivocal. The reported anastomotic leakage rate in Danish Colorectal Cancer Group should be interpreted with caution.

Poly-ε-caprolactone mesh as a scaffold for in vivo tissue engineering in rabbit esophagus.

Repair of long-gap esophageal atresia is associated with a high degree of complications. Tissue engineering on a scaffold of a bioresorbable material could be a solution. The aim of the present study was to investigate the in vivo tissue engineering of smooth muscle cells and epithelium on a poly-ε-caprolactone mesh in rabbit esophagus. Twenty female rabbits had a window of 0.6 × 1 cm cut in the abdominal part of the esophagus. The defect was covered with a poly-ε-caprolactone mesh. The rabbits were killed on postoperative day 28-30, and mesh with surrounding esophagus was removed for histological examination. Fifteen rabbits survived the trial period. Six had no complications and had the mesh in situ. They all had ingrowth of epithelial and smooth muscle cells and an almost completely degraded mesh. Nine rabbits developed pseudo-diverticula. It proved possible to engineer both epithelial and smooth muscle cells on the poly-ε-caprolactone mesh in spite of a fast mesh degradation. The latter may be the explanation to the development of pseudo-diverticula; this is a problem that needs attention in future experimental trials.

Cytokine response in peripheral blood indicates different pathophysiological mechanisms behind anastomotic leakage after low anterior resection: a pilot study.

BACKGROUND: Anastomotic leakage (AL) after rectosigmoid resection is a serious complication associated with high morbidity and mortality. This case-control pilot study investigated the changes in blood concentration of 10 different cytokines and 2 complement factors in relation to symptomatic AL after low anterior resection for rectosigmoid cancer. METHODS: Fifty patients scheduled for resection of rectosigmoid cancer had blood samples taken the day before surgery and on post-operative days 1, 3 and 5. Four patients with symptomatic AL were identified. Twenty-two age- and disease-matched patients constituted the control group. The concentration of 10 cytokines (granulocyte macrophage colony-stimulating factor, interferon-γ, interleukin-1ß, interleukin-2, interleukin-4, interleukin-5, interleukin-6, interleukin-8, interleukin-10 and tumour necrosis factor-α) and 2 complement factors (mannan-binding lectin and membrane attack complex) were measured. RESULTS: The present study demonstrated that plasma concentration of interleukin-1ß, interleukin-6, interleukin-8 and interleukin 10 within the first 5 post-operative days was increased in patients who developed early clinical AL, whereas there were no changes in patients with late-onset AL. CONCLUSIONS: The demonstrated differences in the cytokine response in early and late AL may support the theory of different pathological mechanisms of AL.