Management of desmoid tumours: A large national database of familial adenomatous patients shows a link to colectomy modalities and low efficacy of medical treatments.

BACKGROUND: Desmoid tumours represent a major complication of familial adenomatous polyposis. Our aims were to study the factors associated with the development of desmoid tumours in familial adenomatous polyposis patients, and to describe presentation and management of desmoid tumours. METHODS AND PATIENTS: We reviewed all patients with familial adenomatous polyposis followed at our institution between 1965-2013, with either identified adenomatous polyposis coli gene mutation, or a personal and family history suggesting adenomatous polyposis coli-related polyposis. Response to treatment of desmoid tumours was assessed by Response Evaluation Criteria In Solid Tumor (RECIST) criteria. RESULTS: A total of 180 patients with familial adenomatous polyposis were included with a median follow-up of 19 years since diagnosis. Thirty-one (17%) patients developed 58 desmoid tumours, a median (range) 4.7 (0.8-41.6) years after their diagnosis of familial adenomatous polyposis. The only factor significantly associated with occurrence of desmoid tumours was the type of surgery: 12 (12%) desmoid tumours in 104 patients treated by colectomy, versus 19 (25%) desmoid tumours in 76 patients treated by proctocolectomy, p = 0.027. The localisation of desmoid tumours was: mesenteric (n = 25), abdominal wall (n = 30) or extra-abdominal (n = 3). Nineteen patients underwent 36 surgical procedures for desmoid tumours. Recurrence occurred in 26 (72%) cases and the recurrence-free survival was 2.6 (95% confidence interval (CI), 0.2-5.9) years. Thirteen patients received 27 medical treatments over a median 14 months. Objective response was observed in four (15%) patients and the median progression-free survival was nine (95% CI, 1.1-16.9) months. CONCLUSION: If confirmed, colectomy (versus proctocolectomy) should be performed in adenomatous polyposis coli-related familial adenomatous polyposis patients to avoid desmoid tumours. We show that there is a high prevalence of post-surgical recurrence and the low efficacy of available medical treatments for desmoid tumours.

Extensive preoperative workup in diffuse esophageal leiomyomatosis associated with Alport syndrome influences surgical treatment: A case report.

INTRODUCTION: Diffuse esophageal leiomyomatosis is a rare disease. Misdiagnosis is frequent and previous surgeries can complicate surgical management. The only treatment described for severe symptomatic cases is esophagectomy. PRESENTATION OF CASE: We describe a case of diffuse esophageal leiomyomatosis associated with Alport syndrome in a 21 year-old female where endoscopic ultrasonography (EUS) with concomitant fluoroscopy and 3D-gastric computed tomography (3D-GCT) modified surgical management. DISCUSSION: The diagnosis of diffuse esophageal leiomyomatosis is difficult but can be greatly facilitated by extensive endoscopic and radiologic workup. Esophagectomy should only be entertained after complete anatomic mapping of the lesions, especially after previous surgeries. CONCLUSION: EUS and 3D-GCT should strongly be considered as part of routine preoperative workup in these patients.

Greater weight loss with the omega loop bypass compared to the Roux-en-Y gastric bypass: a comparative study.

BACKGROUND: Despite similar initial results on weight loss and metabolic control, with a better feasibility than the Roux-en-Y gastric bypass (RYGBP), the omega loop bypass (OLB) remains controversial. The aim of this study was to compare the short-term outcomes of the laparoscopic OLB versus the RYGBP in terms of weight loss, metabolic control, and safety. METHODS: Two groups of consecutive patients who underwent laparoscopic gastric bypass surgery were selected: 20 OLB patients and 61 RYGBP patients. Patients were matched for age, gender, and initial body mass index (BMI). Data concerning weight loss, metabolic outcomes, and complications were collected prospectively. RESULTS: Mean duration of the surgical procedure was shorter in the OLB group (105 vs. 152 min in the RYGBP group; p < 0.001). Mean excess BMI loss percent (EBL%) at 6 months and at 1 year was greater in the OLB group (76.3 vs. 60.0%, p = 0.001, and 89.0 vs. 71.0%, p = 0.002, respectively). After adjustment for age, sex, initial BMI, and history of previous bariatric surgery, the OLB procedure was still associated with a significantly greater 1-year EBL%. Diabetes improvement at 6 months was similar between both groups. The early and late complication rates were not statistically different. There were three anastomotic ulcers in the OLB group, in smokers, over 60 years old, who were not taking proton pump inhibitor medication. CONCLUSIONS: In this short-term study, we observed a greater weight loss with OLB and similar efficiency on metabolic control compared to RYGBP. Long-term evaluation is necessary to confirm these outcomes.

Achalasia-like disorder after laparoscopic adjustable gastric banding: a reversible side effect?

Literature data concerning the effect of laparoscopic adjustable gastric banding (LAGB) on esophageal motility are conflicting. Achalasia-like disorder involving the absence of esophageal peristalsis and impaired esophago-gastric junction (EGJ) is probably under-estimated and can result in failure and band removal. The aim of our study was to focus on cases of achalasia-like disorder and study its evolution after band deflating or removal. LAGB patients with food intolerance and whose esophageal manometry confirmed dysmotility were selected from our database. Achalasia-like disorder was defined as the absence of esophageal peristalsis (< 20% contraction waves) with impairment of EGJ relaxation. Manometric control was performed after removal or band deflating; functional results were assessed. Eleven patients among 20 (55%) with esophageal motility disorders (EMD) fitted the manometric criteria of achalasia-like disorder with a mean EGJ resting pressure of 32.1 cmH(2)O and a EGJ relaxation pressure of 24.2. Nine patients out of 11 underwent band removal which resulted in the resolution of their symptoms. The other two underwent band deflation. Manometric control after band removal showed both a decrease in resting and relaxation EGJ pressures (mean of 9.5 and 6.5 cmH(2)O) and a recovery of wave contractions in 87.5% of cases. Four patients underwent revision surgery due to weight regain with a successful outcome. Achalasia-like disorder is a manometric diagnosis and accounts for a significant part of symptomatic EMD after LAGB. It often results in band removal, allowing some reversibility of the disorders.

Persistent dysphagia after removal of an adjustable gastric band for morbid obesity: a rare complication.

Esophageal and gastric pouch dilatations are not uncommon after laparoscopic adjustable gastric banding for morbid obesity. Most of the cases are treated by gastric band deflation or removal. We report here the case of a 44-year-old woman with vomiting and severe dysphagia persisting despite gastric band removal, in relation with a scar stenosis and a gastric pouch trapped in the thorax, treated by laparoscopic surgery. This case underlines the usefulness of high-resolution manometry in the diagnostic work-up of these often difficult cases.

Natural history of acute colonic diverticular bleeding: a prospective study in 133 consecutive patients.

BACKGROUND: Bleeding recurrence rate after spontaneous haemostasis of colonic diverticular haemorrhage varies in the literature, and a small minority of patients will require endoscopic, radiological or surgical intervention. AIM: To study the natural history of colonic diverticular bleeding in consecutive patients. METHODS: We studied prospectively consecutive patients admitted for colonic diverticular bleeding from 1997 to 2005. Data on age, gender, 30-day mortality, therapeutic modality for bleeding management and subsequent rebleeding were collected. RESULTS: One hundred and thirty-three patients (mean age 75.7 years) were recruited. Bleeding stopped spontaneously in 123 patients (92.4%). A more interventional approach was necessary in 10 patients. Thirty-day mortality rate for first bleeding was 2.25%. Out of the 123 patients managed conservatively and submitted to an average follow-up of 47.5 months, 17 (13.8%) presented at least one recurrent diverticular bleeding. Spontaneous haemostasis was obtained in all recurrent cases except one, who died. The estimated bleeding recurrence rate was 3.8% at 1 year, 6.9% at 5 years and 9.8% at 10 years. CONCLUSIONS: The low estimated rebleeding rate and the fact that rebleeding can be treated conservatively in most cases suggest that an aggressive approach with intervention is not justified.

Results of laparoscopic Heller myotomy without anti-reflux procedure in achalasia. Monocentric prospective study of 106 cases.

BACKGROUND: Heller myotomy (HM) combined with an anti-reflux procedure has been shown to be effective for the treatment of achalasia, as postoperative gastro-esophageal reflux (GER) is observed in about 10% of the cases. Laparoscopy has brought an undeniable benefit in providing excellent visualisation of the gastro-esophageal junction (GEJ) without lateral and posterior dissection. Respecting the anatomical fixation of the GEJ seems to permit the performing of HM without an anti-reflux procedure, the need for which is therefore debatable. The purpose of this study was to analyse the results of this controversial procedure. METHODS: A monocentric prospective study was carried out on 106 patients who underwent HM without an anti-reflux procedure. The postoperative assessment consisted of a manometry and a 24-hour pH study two months after surgery, and a yearly clinical examination for a minimum of five years. The data capture was done using a statistical analysis. RESULTS: There was no mortality, one conversion to an open procedure, and four mucosal perforations. Postoperative morbidity was 2%. The average follow-up period was 55 months (range, 2 to 166), with 10 patients lost to follow-up. Good functional results were observed in 91.4% of patients at one year, and 78.6% at five years. Two months after surgery, a 9.4% prevalence of GER was detected in the pH study, and the lower esophageal sphincter pressure had significantly decreased. After a long term follow-up we observed an 11.3% global rate of GER. No repeat surgery was necessary to control postoperative GER. CONCLUSIONS: Laparoscopic HM without anti-reflux procedure gives good functional results provided the anatomical fixation of the GOJ is respected.

In-vivo high-resolution X-ray microtomography for liver and spleen tumor assessment in mice.

The present study sought to validate the use of glycery1-2-oley-1,3-bis-[7-(3-amino-2,4,6-triiodophenyl)- heptanoate] (DHOG) contrast agent for mouse spleen tumor and liver metastasis imaging by high-resolution X-ray microtomography. Three groups of female nude mice were compared: controls (n = 5), and mice injected with 2.5 x 10(6) STC1 tumor cells in the spleen, imaged at 15 days (group G15, n = 5) and at 30 days (group G30, n = 5, of which one died before imaging). Micro-CT scans (X-ray voltage, 50 kVp; anode current, 200 microA; exposure time, 632 ms; 180 rotational steps resulting in 35 microm isotropic spatial resolution) were acquired at 0, 0.75, 2 and 4 h after i.v. injection of DHOG. CT number (Hounsfield units: HU) and contrast-to-noise ratios (CNR) were determined in three organs. Statistical analysis was performed by Mann-Whitney U-test. Contrast enhancement in normal spleen and liver increased, respectively to 1020 +/- 159 and 351 +/- 27 HU over baseline at 4 h, and 482 +/- 3 and 203 +/- 14 HU on day 6 after a single contrast injection. Automated three-dimensional reconstruction and modeling of the spleen provided accurate and quantifiable images. Spleen tumor and liver metastases did not take up DHOG, making them detectable in contrast to the increased signal in normal tissue. The smallest liver metastasis detected measured 0.3 mm in diameter. High-resolution X-ray micro-CT in living mice using DHOG contrast agent allowed visualization and volume quantification of normal spleen and of spleen tumor and its liver metastases.

Characterization of reflux events after fundoplication using combined impedance-pH recording.

BACKGROUND: Laparoscopic fundoplication effectively controls symptoms of gastro-oesophageal reflux disease (GORD) and decreases acid reflux, but its impact on non-acid reflux is not known. The aim of the study was to characterize reflux events after fundoplication using oesophageal combined multichannel intraluminal impedance (MII)-pH monitoring, to demonstrate its efficacy on acid as well as non-acid reflux events. METHODS: Thirty-six patients in whom ambulatory MII-pH recording was performed after laparoscopic fundoplication were reviewed retrospectively. There were 23 symptomatic and 13 asymptomatic patients, whose results were compared with those of 72 healthy volunteers. RESULTS: Oesophageal acid exposure was low in all but one operated patient, and there was no difference between those with and without symptoms. The median number of reflux events over 24 h was lower after fundoplication (11 in operated patients compared with 44 in healthy volunteers; P < 0.001). Almost all reflux events were non-acid after surgery whereas acid reflux episodes were predominant in healthy volunteers. Proximal reflux events were less common in operated patients. Non-acid reflux events were significantly associated with symptoms after surgery in some patients. CONCLUSION: Fundoplication restores a competent barrier for all types of reflux. Reflux events are mostly non-acid after surgery, and such events may be positively correlated with symptoms.

Expression of beta-catenin in gastroenteropancreatic endocrine tumours: a study of 229 cases.

AIMS: To clarify the role of beta-catenin in digestive endocrine carcinogenesis, a large and representative series of gastroenteropancreatic endocrine tumours was analysed in order to determine the incidence and pattern of beta-catenin changes and to analyse the clinical and histological characteristics of the tumours presenting immunohistochemically detectable changes in beta-catenin expression. METHODS: 229 cases of gastroenteropancreatic endocrine tumours (stomach, 11; duodenum and ampulla, 29; jejunum and ileum, 51; appendix, 13; colon and rectum, 17; and pancreas, 108) were studied by immunohistochemistry to assess the pattern of distribution of beta-catenin (membranous, cytoplasmic or nuclear). DNA was analysed to detect mutations in exon 3 of the CTNNB1 gene. RESULTS: The distribution of immunoreactive beta-catenin protein was membranous in 164 cases, cytoplasmic in 58 cases and nuclear in seven cases. No mutation was detected in exon 3 of the CTNNB1 gene in any case. The seven cases with nuclear accumulation of beta-catenin were large tumours (mean size 44 (standard deviation (SD) 18.5) mm) with metastases, including liver metastases in five cases, high Ki-67 index (mean 34% (SD 16.5%)) and cyclin D1 overexpression; p53 accumulation was detected in six cases. Five patients died of disease; the mean (SD) survival was 13.6 (4.8) months. CONCLUSIONS: Immunohistochemically detectable nuclear accumulation of beta-catenin is infrequent in gastroenteropancreatic endocrine tumours and is usually not associated with mutations in CNNTB1 exon 3. Changes in beta-catenin expression are late events in digestive endocrine carcinogenesis, associated with tumour progression and dissemination.

Unresectable hepatocellular carcinoma: survival and prognostic factors after lipiodol chemoembolisation in 89 patients.

BACKGROUND: The majority of patients with hepatocellular carcinoma are not eligible for surgical radical treatment (resection or liver transplantation) and lipiodol chemoembolisation is an efficient alternative procedure in this indication. AIMS: To identify prognostic factors in patients treated with lipiodol chemoembolisation. PATIENTS AND METHODS: During 10 years, 89 consecutive patients with unresectable hepatocellular carcinoma underwent lipiodol chemoembolisation as a single treatment. There were 80 males and 9 females, with a median age of 65 years. Treatment consisted of one to six courses of hepatic intra-arterial lipiodol with doxorubicine and gelatin sponge. RESULTS: The median survival was 13 months with a 13.6% survival rate at 4 years. Univariate analysis showed that serum levels of albumin, bilirubin, alkaline phosphatase and alpha-fetoprotein, Child's class, tumour type, tumour size and intensity of lipiodol capture after the first course of lipiodol chemoembolisation were significant prognostic factors of survival. In the multivariate analysis, four parameters remained associated with a significantly better outcome: Child's class A, largest lesion<5 cm, uninodular tumour and intense lipiodol capture. CONCLUSIONS: While lipiodol chemoembolisation is associated with good results only in some patients, in the absence of lipiodol capture, it should be ruled out.

Unusual cystic presentation of an endocrine carcinoma of the jejunum.

The cystic presentation of endocrine tumours is rare and raises difficult diagnostic problems. So far, the only cases of cystic digestive endocrine tumours reported in the literature are of pancreatic origin. We report the unusual observation of a jejunal endocrine carcinoma presenting as a cystic abdominal mass. A 59-year-old woman was referred for chest and abdominal pain. Imaging studies revealed multiple cystic nodules in the liver and a large sus-mesocolic cystic lesion of probable intestinal origin. Biopsies of the extra-hepatic mass and liver nodules showed endocrine tumour. Surgical resection of the jejunal mass and of liver segment III were performed. Histological examination confirmed the diagnosis of jejunal endocrine carcinoma metastatic to the liver. Large areas of the primary and secondary tumours presented an unusual vesicular architecture, responsible for the cystic presentation. No adjuvant treatment was attempted. This observation underlines the difficult diagnostic problems raised by the cystic presentation of digestive endocrine tumours.

Diagnosis and treatment of biliary obstruction caused by portal cavernoma.

While hemorrhagic complications of portal cavernoma are frequent, compression of the bile ducts by portal cavernoma is uncommon and treatment is still a matter for debate. We report here six new cases in order to describe: (a) the clinical, biological, and morphological features of this condition, and (b) the long-term results of a combined endoscopic and surgical treatment. The median age of patients at the time of diagnosis was 36.5 years. The circumstances of diagnosis were acute cholangitis (n=3), asymptomatic biological cholestasis (n=1), pruritus, jaundice and asthenia (n=1) and jaundice alone (n=1). Portal cavernoma and bile duct dilatation were confirmed by abdominal ultrasonography with pulsed color doppler and endoscopic retrograde cholangiography (ERC). Gallstones were found in four patients. Following stenting of the bile duct, there was a good outcome in two patients. In four patients, after failure of prolonged endoscopic treatment, second-line surgical portal-systemic shunting allowed removal of the biliary stent, and no recurrence of disease. In conclusion, biliary involvement in portal cavernoma is now a well-recognized entity, and our results suggest that combined endoscopic and surgical treatment could be required.