Modified Sugiura Operation for Idiopathic Portal Hypertension with Bleeding Oesophageal Varices. A Case Report.

A case of a 36 years old man presenting massive upper GI bleeding due to oesophageal varices developed in the context of an idiopathic portal cavernoma and extensive porto-splenic thrombosis is discussed. He underwent a successful modified Sugiura operation (oesophago-gastric devascularisation and splenectomy [OGDS]) completed with interventional endoscopic treatment of residual oesophageal varices. The benefit of the modified Sugiura procedure proposed for the treatment of upper GI variceal bleeding developed in the context of splanchnic venous thrombosis is discussed. The procedure is a valid therapy in the treatment of symptomatic extra-hepatic hypertension when other options are inapplicable.

[Incidence of colorectal carcinoma and patient survival in the Grand-Duchy of Luxembourg]. / Inzidenz des kolorektalen Karzinoms und Uberleben der patienten im Grossherzogtum Luxemburg.

As in many other Western countries, colorectal cancer is one of the most common malignant tumours in Luxembourg. It is thus an important target for prevention measures. In light of the discussions on the introduction of organized screening programmes in Luxembourg a description of the current cancer incidence and relative survival rates, as well as a comparison with neighbouring countries are important. The tumour incidence is comparable to that in other countries with similar socio-economic development. The standardized incidence rate (world population) in 2010 was 26.4 and 44.4 per 100,000 for women and men, respectively. In some, but not all, Western European countries, a decreasing trend of the standardized incidence is emerging. No such trend is currently observed in Luxembourg. The mortality and relative survival rates are comparable to those in other European countries. The proportion of colorectal carcinomas of stages T1 + T2, 18.7% in the years 2000 to 2011, is markedly lower in Luxembourg than for example in Rhineland -Palatinate (29.4%), while the proportion of carcinomas of stage T4 is higher (17.4% versus 13.9%). This suggests that further successes in prevention are possible and illustrates the benefits of an organized screening colonoscopy program compared with opportunistic screening.

Problems of oral anticoagulation in an adult with homozygous protein C deficiency and late onset of thrombosis.

We describe a 57-year-old woman with homozygous protein C deficiency and mild thrombotic manifestations consisting of three spontaneous distal deep vein thromboses occurring after the age of 45. Previous surgery and pregnancies had been uneventful. Low but detectable protein C antigen and activity levels (both 20%) were discovered on the occasion of skin necrosis induced by oral anticoagulation. This therapy was interrupted because of skin necrosis and several episodes of disseminated intravascular coagulation (DIC) at the initiation of treatment despite a cautious protocol. No recurrent thromboembolic event has occurred in our patient using prophylactic doses of low molecular weight heparin for 24 months. New therapeutic approaches might be the administration of low molecular weight heparin or oral anticoagulation associated with protein C replacement in the induction period. This case reflects the variability of expression of protein C deficiency as well as the potential hazards of antivitamin K anticoagulation in this disorder.

[Fatal fulminant hepatitis induced by nilutamide (Anandron)]. / Hépatite fulminante mortelle imputable au nilutamide (Anandron).

The first case of fatal fulminant hepatitis probably caused by nilutamide, a non steroidal antiandrogen derivative, is reported. Nilutamide administration had been continued for 6 days after the occurrence of jaundice, 52 days after beginning of treatment. The rapidly fatal outcome might have been promoted by coadministration of phenobarbital.

[Mucoviscidosis discovered after the age of 25 years. Review and follow-up of cases of the literature]. / Mucoviscidose reconnue après l'âge de 25 ans. Revue et devenir des cas de la littérature.

We report a case of cystic fibrosis diagnosed in a 27-year-old man complaining of diarrhoea which was present for 2 years. The diagnosis was suspected upon the association of exocrine pancreatic insufficiency, massive hepatic steatosis, pulmonary infiltrates on chest radiograph, and moderate alterations of pulmonary function tests. It was confirmed by positive sweat tests. Study of the cystic fibrosis gene demonstrated a compound heterozygosity for delta F508 deletion and for mutation W1282X. Diagnosis of cystic fibrosis after the age of 25 is a rare event and the 25 hitherto published case reports are analysed after obtention of more detailed information for the authors. The existence of cases of late diagnosis might be explained by genetic heterogeneity.

[Digestive lymphomatous polyposis]. / Polypose lymphomateuse digestive.

We report 7 prospectively followed cases of lymphomatous polyposis of the gastrointestinal tract. They were characterized by multiple polypoid lesions affecting several segments of the gastrointestinal tract always involving the colon and the rectum. An ileocecal mass was present in 4 cases. Regional lymph node involvement was constant. Peripheral lymphadenopathy was frequent (5 cases out of 7), as was other extra-digestive extension to the bone marrow (4 cases out of 7) and cavum (3 cases out of 7). The histopathological aspect was that of a small cleaved cells (working formulation) or centrocytic (Kiel classification) non-Hodgkin's lymphoma. The peculiar morphology and phenotype of the tumoral B-lymphocytes suggest their possible follicle marginal zone origin. Lymphomatous polyposis bore a rapidly fatal prognosis in every case (mean survival 20 months). This study of seven patients together with the 20 well-documented cases of the literature confirms the existence of lymphomatous polyposis as a distinctive clinicopathological entity among gastrointestinal non-Hodgkin's lymphoma.

Review of laparoscopic and open colorectal surgery in the "Zitha" Hospital (Luxembourg) in the year 2002.

BACKGROUND: A review and outcome of laparoscopic colorectal surgery in the visceral surgery unit in the Zitha Hospital for the year 2002. METHODS: All patients, presenting with benign or malignant disease of the colon or rectum were evaluated. Depending on the tumour size, a laparoscopic approach is advocated whenever possible. Operation and patient specific factors (age, duration of the procedure and duration of hospital stay, diagnosis of malignant tumour versus benign disease, conversion and complication rate) are presented. Patients who had to be converted are classified under the open surgery group. RESULTS: A total of 103 laparoscopic or open procedures on the colon/rectum were performed between the 1st of January 2002 and the 31st of December 2002. 74% were treated by a laparoscopic and 26% by an open approach. 10 out of 103 patients did not undergo a colonic resection but instead an emergency colostomy/ ileostomy or oversewing of a bowel leak, in 8 cases performed laparoscopically. 44 patients have been operated for a benign disease (36 laparoscopic and 8 open procedures) and 59 patients have been treated for a malignant disease (40 laparoscopic and 19 open procedures). The average operating time for the laparoscopic resection was 160 minutes in comparison to 182 minutes for the open resection. The conversion rate (inclusive of non-resection procedures) was 5% for a benign disease and 11% for colorectal cancer. The minor complication rate was 16% in laparoscopic surgery and 30% in open surgery. We saw two major complications in both the open and laparoscopic groups (4%). Reoperation was necessary in one patient out of 103. The average postoperative hospital stay was 7.78 days for the laparoscopic and 16.6 days for the open group. The hospital mortality was 2.9%. CONCLUSION: The laparoscopic colorectal resection is a safe and beneficial procedure for the patient when used in experienced hands.

[Acute pseudo-obstruction of the colon (Ogilvie syndrome) after Cesarean section]. / Akute Pseudoobstruktion des Kolons (Ogilvie-Syndrom) nach Sectio caesarea.

In a 27-year-old woman a spontaneous perforation of the bowel developed after cesarean section. The clinical picture, pathophysiological aspects and prophylactic treatment of Ogilvie's syndrome are discussed. This rare but serious complication could have been avoided if the clinical picture and its simple conservative treatment had been known.

Evolution of colorectal cancer epidemiology in a setting of opportunistic screening. A 20 year national survey in Luxembourg.

UNLABELLED: Invasive colorectal cancer (CRC) is the second leading cause of cancer death in Luxembourg. There is no organized screening programme in Luxembourg. This study aims to obtain a precise epidemiological description of the evolution of invasive CRC and high grade intraepithelial neoplasia (HGIEN) from 1990 to 2009, extracted from the database of the Morphologic Tumor Registry. Tumor stages and observed survival rates were also recorded. They were compared to the change in use of colonoscopic procedures. During the 20-year period, 4810 invasive CRC cases and 1180 HGIEN were recorded. Incidence rose from 1990 to 2005 and declined thereafter, especially in women. A sharp rise in HGIEN was noted from 2004 onwards, paralleling the rates of colonoscopies. 76% of CRC cases were found in advanced stages pT3 and pT4. The pT stage distribution did not change over the observation period. Observed survival rates improved during the study period. CONCLUSION: Under opportunistic screening conditions, mainly through colonoscopy, the incidence of CRC was declinig in recent years, whereas HGIEN incidence is rising in Luxembourg. Tumor pT staging remained unchanged whereas survival rates improved. We conclude that opportunistic screening is of little benefit for CRC prevention. A national organized screening programme is warranted.

Oesophageal intraepithelial and invasive neoplasia of squamous cell type: epidemiology and outcome in Luxembourg, 1980-2001.

BACKGROUND AND STUDY AIMS: Oesophageal intraepithelial neoplasia of squamous cell type (INSC) and invasive oesophageal squamous cell carcinoma (IOSCC) are infrequent diseases in Western Europe. The aim of the present study was to collect population-based data of both entities over a 20 year-period and to look for concomitant neoplastic affections in order to define an adequate diagnostic strategy. PATIENTS AND METHODS: The National Morphologic Tumour Registry allowed to review the data of all patients with INSC and IOSCC diagnosed between 1980 and 2001 and to record the time trends in incidence, the oncologic co-morbidity and the outcome of the patients. RESULTS: 29 patients with INSC and 363 cases of IOSCC were identified. The overall age-standardized (world) incidence rate of intraepithelial neoplasia and invasive squamous cell carcinoma were 0.2 and 4.2 per 10(5), respectively, the M/F-ratio for both 3:1. During the study period, the incidence rate of invasive cancer remained stable in males but showed a 3-fold increase in females. There was a 2-fold increase of the intraepithelial neoplasia incidence in the last decade. The precancerous/cancerous-ratio increased slightly over the last 5 years. 31% of the patients with an INSC and 17.6% of those with IOSCC had concomitant precancerous and cancerous lesions especially of head and neck (laryngopharyngeal) or pulmonary origin. The observed 5-year survival rate was 8.8 +/- 3% (95% confidence interval) for IOSCC and 27.6% +/- 17% for INSC. CONCLUSIONS: The incidence of invasive oesophageal squamous cell carcinomas remains stable whereas that of detected intraepithelial squamous cell neoplasias is remarkably low, indicating potential underdiagnosis. Considering the overall low incidence rates, mass screening for oesophageal cancer does not seem reasonable in Luxembourg. Nevertheless, patients at high-risk for oesophageal or head and neck or broncho-pulmonary cancer should be identified and surveilled by endoscopy, possibly with vital staining.

A severe complication of the new self-expanding spiral nitinol biliary stent.

The new spiral nitinol biliary stent offers some very promising features, including its long patency and retrievability. We report here the case of a patient in whom this type of stent was inserted for palliative treatment of a periampullary tumour. The stent migrated distally, and could not be withdrawn. The further course was complicated by duodenal obstruction, repeated bouts of cholangitis, and fatal hemorrhage after surgical removal of the endoprosthesis. The design of this new stent should be improved.

The value of histological analysis of occluded biliary endoprostheses.

BACKGROUND AND STUDY AIMS: Histological diagnosis of biliary strictures remains unsatisfactory, despite the availability of various endoscopic sampling procedures. The aim of our study was to assess the potential diagnostic yield of histological processing of occluded biliary endoprostheses inserted for palliation of malignant biliary stenoses. PATIENTS AND METHODS: Over a period of one year, we prospectively collected biliary endoprostheses at the time of stent removal due to stent obstruction. Thirty-nine stents, inserted a mean of 94 days earlier for presumed malignant biliary strictures, were recovered. Their contents were examined histologically by two independent pathologists. RESULTS: Malignancy was found in 14 of 36 patients (39%). The sensitivity was highest in gallbladder carcinoma (66%), followed by choledochal and ampullary carcinoma (50%), metastatic carcinoma (33%), and pancreatic carcinoma (25%). The specificity of the method was 100%. CONCLUSIONS: We advocate systematic histological or cytological examination, or both, of occluded biliary endoprostheses, since the technique is of low invasiveness, has low costs, and is easy to perform, especially if other methods of tissue diagnosis have failed.

Fibrin sealing in peptic ulcer bleeding: the fate of the clot.

BACKGROUND AND STUDY AIMS: The injection of fibrin tissue glue is a promising endoscopic method for hemostasis of peptic ulcer bleeding. So far, no clinical study has focused on the ulcer healing process after endoscopic fibrin injection. PATIENTS AND METHODS: A morphological study was performed on all resection specimens from patients operated on between 1 January 1994 and 31 December 1996 for gastroduodenal ulcer bleeding with prior endoscopic injection of fibrin glue. The fibrin clot was characterized histologically for its size, location and aspect. RESULTS: Of 227 patients endoscopically treated with a double-lumen needle, 20 underwent resection. The interval between fibrin injection and resection ranged from 6 hours to 9 days. In 15 patients the ulcer was identified in the resection specimen. No fibrin remnants were detectable in three, sparse fibrin deposits were seen in eight and large amounts of fibrin were noted in the submucosa or subserosa in four specimens. With increasing time, the clot was gradually organized by phagocytes and angio-fibroblasts and was finally replaced by endogenous granulation tissue. An exuberant or tissue-destructive reaction did not appear. CONCLUSIONS: In this study, endoscopic fibrin sealing of bleeding ulcers resulted in appearance of a bland fibroblast-rich granulation tissue. The depth of fibrin glue injection is difficult to standardize with the aid of double-lumen needles.

Hemobilia, a rare cause of acute pancreatitis after percutaneous liver biopsy: diagnosis and treatment by endoscopic retrograde cholangiopancreatography.

We here report the case history of a 75-yr-old woman who developed pancreatitis and recurrent symptomatic, cholestasis-induced hemobilia after percutaneous liver biopsy. An endoscopic sphincterotomy with clot extraction led to relief of symptoms. The risk of hemobilia after percutaneous liver biopsy is less than one per 1000 procedures, and only two cases of acute pancreatitis after percutaneous liver biopsy have previously been reported. To our knowledge, this is the first case in which endoscopic retrograde cholangiopancreatography was used to both diagnostic and therapeutic ends.

Combined laparoscopic-endoscopic method using an omental plug for therapy of gastroduodenal ulcer perforation.

BACKGROUND: Laparoscopic techniques have been proposed as an alternative to open surgery for therapy of peptic ulcer perforation. They provide better postoperative comfort and absence of parietal complications, but leakage occurs in 5% of cases. We describe a new method combining laparoscopy and endoluminal endoscopy, designed to ensure complete closure of the perforation. METHODS: Six patients with anterior ulcer perforations (4 duodenal, 2 gastric) underwent a concomitant laparoscopy and endoluminal endoscopy with closure of the orifice by an omental plug attracted into the digestive tract. RESULTS: All perforations were sealed. The mean operating time was 72 minutes. The mean hospital stay was 5.5 days. There was no morbidity and no mortality. At the 30-day evaluation all ulcers but one (due to Helicobacter pylori persistence) were healed. CONCLUSIONS: This method is safe and effective. Its advantages compared with open surgery or laparoscopic patching as well as its cost-effectiveness should be studied in prospective randomized trials.

Ultrafast magnetic resonance imaging improves the staging of pancreatic tumors.

OBJECTIVE: This prospective study was undertaken to evaluate the accuracy of a noninvasive "all-in-one" staging method in predicting surgical resectability in patients with pancreatic or periampullary tumors. SUMMARY BACKGROUND DATA: Despite progress in imaging techniques, accurate staging and correct prediction of resectability remains one of the chief problems in the management of pancreatic tumors. Staging algorithms designed to separate operable from inoperable patients to save the latter an unnecessary laparotomy are becoming increasingly complex, expensive, time-consuming, invasive, and not without risks for the patient. METHODS: Between August 1996 and February 1997, 58 consecutive patients referred for operation of a pancreatic or periampullary tumor were examined clinically and by 5 staging methods: 1) percutaneous ultrasonography (US); 2) ultrafast magnetic resonance imaging (UMRI); 3) dual-phase helical computed tomography (CT); 4) selective visceral angiography; and 5) endoscopic cholangiopancreatography (ERCP). The assessment of resectability by each procedure was verified by surgical exploration and histologic examination. RESULTS: The study comprised 40 male and 18 female patients with a median age of 63 years. Thirty-five lesions were located in the pancreatic head (60%), 11 in the body (19%), and 1 in the tail of the gland (2%); there were 9 tumors of the ampulla (16%) and 2 of the distal common duct (3%). All five staging methods were completed in 36 patients. For reasons ranging from metallic implants to contrast medium allergy or because investigations already had been performed elsewhere, US was completed in 57 (98%), UMRI in 54 (93%), CT in 49 (84%), angiography in 48 (83%), and ERCP in 49 (84%) of these 58 patients. Signs of unresectability found were vascular involvement in 22 (38%), extrapancreatic tumor spread in 16 (26%), liver metastases in 10 (17%), lymph node involvement in 6 (10%), and peritoneal nodules in only 2 patients (3%). These findings were collated with those of surgical exploration in 47 patients (81 %) and percutaneous biopsy in 5 (9%); such invasive verification was deemed unnecessary and therefore unethical in 6 clearly inoperable patients (10%). In assessing the four main signs of unresectability (extrapancreatic tumor spread, liver metastases, lymph node involvement, and vascular invasion), the overall accuracy of UMRI was 95.7%, 93.5%, 80.4%, as compared to 85.1%, 87.2%, 76.6% for US and 74.4%, 87.2%, 69.2% for CT. In assessing vascular invasion, the sensitivity, specificity, and overall accuracy of angiography were 42.9%, 100%, and 68.8%, respectively. There were 3 complications (12.5%) after 24 resections, 5 in 17 palliative procedures, and none after 6 explorations only. The hospital stay was 14 days after resection, 13 after palliative bypass, and 6 after exploration alone. There was no operative or hospital mortality in these 58 cases. CONCLUSIONS: Although it is by no means 100% accurate, UMRI is equal or even superior to all other staging methods. It probably will replace most of these, because it provides an "all-in-one" investigation avoiding endoscopy, vascular cannulation, allergic reactions, and x-radiation. But because even UMRI is not perfect, the final verdict on resectability of a tumor still will depend on surgical exploration in some cases.

Diagnostic accuracy and interobserver agreement of CT colonography (virtual colonoscopy).

BACKGROUND AND AIMS: Computed tomographic (CT) colonography or virtual colonoscopy (VC) is a non-invasive imaging method proposed for screening patients with colorectal neoplasias. Our aims were to study the diagnostic accuracy and interobserver agreement of VC for correct patient identification compared with conventional colonoscopy (CC). METHODS: This was a prospective study of 50 patients successively undergoing VC and CC. Multiplanar two dimensional CT images and three dimensional VC were constructed using surface rendering software and interpreted by two independent investigator teams. VC findings were compared with those of CC. Interobserver agreement was determined using kappa statistics. RESULTS: CC found 65 polyps in 24 patients. For identification of patients with polyps > or =10 mm, the sensitivity of VC was 38% and 63%, and specificity was 74% and 74% for teams 1 and team 2. Interobserver agreement was good (kappa 0.72). For patients with polyps of any size, the sensitivity of VC was 75% and 71%, and specificity was 62% and 69% for teams 1 and 2. Interobserver agreement was fair (kappa 0.56). Accuracy improved when comparing the results of the first 24 with the last 26 patients. CONCLUSIONS: In our experience, VC had a low diagnostic value for identification of patients with colorectal neoplasias. Interobserver agreement for VC interpretation was fair. These results may be explained by software imperfections and a learning curve effect.

Serological markers for prediction of response to anti-tumor necrosis factor treatment in Crohn's disease.

OBJECTIVES: The use of monoclonal anti-tumor necrosis factor (TNF) antibodies (infliximab, Remicade) is a new therapeutic approach for severe refractory luminal or fistulizing, Crohn's disease (CD). However, up to 30% of patients do not respond to this treatment. So far, no parameters predictive of response to anti-TNF have been identified. Our aim was to determine whether serological markers ASCA (anti-Saccharomyces cerevisiae antibodies) or pANCA (perinuclear antineutrophil cytoplasmic antibodies) could identify Crohn's patients likely to benefit from anti-TNF therapy. METHODS: Serum samples of 279 CD patients were analyzed for ASCA and pANCA before anti-TNF therapy. A blinded physician determined clinical response at week 4 (refractory luminal CD) or week 10 (fistulizing CD) after the first infusion of infliximab (5 mg/kg). RESULTS: Overall, there was no relationship between ASCA or pANCA and response to therapy. However, lower response rates were observed for patients with refractory intestinal disease carrying the pANCA+/ASCA- combination, although this lacked significance (p = 0.067). CONCLUSIONS: In this cohort of infliximab-treated patients, neither ASCA nor pANCA could predict response to treatment. However, the combination pANCA+/ASCA- might warrant further investigation for its value in predicting nonresponse in patients with refractory luminal disease.

A positive response to infliximab in Crohn disease: association with a higher systemic inflammation before treatment but not with -308 TNF gene polymorphism.

BACKGROUND: Two-thirds to three-fourths of patients with either refractory luminal or fistulizing Crohn disease respond to infliximab treatment. The ability or inability to respond seems to persist over time. Biological characteristics and/or genetic background can influence the response to treatment. The aim was to assess the value of C-reactive protein and TNF-alpha serum levels before treatment as well as the TNF -308 gene polymorphism in the prediction of response to infliximab treatment in Crohn disease. METHODS: Two-hundred-and-twenty-six Crohn disease patients treated in the setting of an expanded access programme to infliximab in Belgium were studied. There were 136 refractory luminal diseases and 90 refractory fistulizing diseases. Luminal diseases were treated with one single infusion; fistulizing diseases with three infusions at weeks 0, 2 and 6. A clinical response to treatment was defined as either a Crohn disease activity index <150 (complete) or a drop of 70 points (partial) at week 4, for luminal disease, and as either complete fistula healing (complete) or a decrease of at least 50% of the number of draining fistulas on two consecutive visits between weeks 0 and 18, for fistulizing disease. CRP and serum TNF-alpha levels were measured at week 0 before treatment and were compared between responders and non-responders. Patients were genotyped for the -308 TNF gene polymorphism, and allelic as well as genotype frequencies were compared between responders and non-responders. RESULTS: There were 73.2% responders (46.4% complete and 26.8% partial) and 26.8% non-responders. Response rates were similar in luminal and fistulizing diseases. CRP level before treatment was significantly higher in responders than in non-responders (16.8 mg/l (5-160) versus 9.6 mg/l (5-143); P = 0.02). Furthermore, response rate was significantly higher in patients with elevated CRP (>5 mg/l) than in patients with a normal CRP value (<5 mg/l) before treatment (76% versus 46%; P=0.004; OR: 0.26 (0.11-0.63)). Allelic and genotype frequencies for -308 TNF gene polymorphism were not significantly different between responders and non-responders--with the exception of a slightly higher TNF2 frequency in non-responders in luminal disease (22.1 % versus 11.6%; P = 0.04). However, this was not associated with a significant difference in genotype frequencies. CONCLUSION: A positive clinical response to infliximab was associated with a higher CRP level before treatment in our population of Crohn disease patients, but there was no relevant association with -308 TNF gene polymorphism. We therefore suggest that CRP level may help to identify better candidates for infliximab treatment.