Necrotising fasciitis of the left leg caused by perforated caecal adenocarcinoma.

An 82-year-old man presented with abdominal pain and a painful and swollen left thigh. On examination there was oedema, tenderness and crepitus with skin discoloration of the left thigh. Computed tomography showed retroperitoneal perforation of the caecum and necrotising fasciitis of the left thigh. A right hemicolectomy and repeated fasciotomies of the left thigh with debridement of necrotic tissue were performed but the patient died of multi-organ failure. Histology showed a pT4aN2c caecal adenocarcinoma. This is a unique presentation of a retroperitoneal caecal perforation and acts as a reminder that unexplained severe fasciitis of the thigh may be caused by an intra-abdominal pathology.

High serum CA 19-9 but not tumor size should select patients for staging laparoscopy in radiological resectable pancreas head and peri-ampullary cancer.

BACKGROUND: The objective of this study was to validate current recommendations for the selective use of staging laparoscopy in patients with radiological resectable pancreas head and peri-ampullary tumors. METHODS: Data from a prospectively collected database (2007-2013) of 136 patients with peri-pancreatic head cancer were analyzed. RESULTS: Over a 6 year time period, 136 patients were evaluated, 126 patients were deemed radiological resectable and underwent laparotomy and 10 patients were characterized radiological unresectable. There were 111 patients with pancreas head resection and 15 without resection (8 due to extensive vascular involvement and 3 due to peritoneal/liver metastases). The sensitivity, specificity, PPV and NPV of pre-operative radiological imaging in determining unresectability due to liver/peritoneal metastases were 42%, 100%, 100% and 94.7% respectively. There was a significant difference in CA 19-9 values between metastatic and non-metastatic disease (p = 0.020). ROC curve analysis calculated the optimal CA 19-9 cutoff point for predicting metastasis at 215.37 U/ml with a sensitivity of 72.7%, a specificity of 58.3%, PPV of 15.1% and NPV of 95.5%. Tumor diameter was not a significant factor in predicting resectability. Laparoscopy would have been useful in only 5.3% of patients in the present series. CONCLUSION: High CA 19-9 values (>215 U/ml) and not tumor size should select patients with radiological resectable peri-pancreatic cancer for staging laparoscopy.

Long-term changes in the ghrelin-CB1R axis associated with the maintenance of lower body weight after sleeve gastrectomy.

OBJECTIVES: In the hypothalamus, the molecular actions of receptors for growth hormone secretagogue (ghrelin) receptor-GHSR, leptin receptor-b (LEPRb), Melanocortin-4 receptor (MC4R) and Cannabinoid-1 receptor (CB1R) regulate energy homeostasis and body weight. We hypothesized that the acute loss of stomach tissue upon sleeve gastrectomy (SG), performed to treat obesity, imposes modulations on the expression of these receptors in the brain to sustain weight loss. METHODS: Rats, induced to obesity with high-fat diet were randomized to SG- or sham-operation groups and killed at 30 or 90 days post surgery, when the expression of Ghrl, Mboat4 and Cnr1 in the stomach, and Ghsr, Leprb, Mc4r and Cnr1 in distinct brain areas was assessed by reverse transcription-PCR and western blotting. RESULTS: SG acutely reduced body weight and fat mass and suppressed the remnant stomach mRNA levels of preproghrelin and ghrelin O-acyltransferase, which correlated well with long-term decreases in CB1R mRNA. In the hypothalamus, increases in GHSR and decreases in CB1R and LEPRb by 30 days were followed by further downregulation of CB1R and an increase in MC4R by 90 days. CONCLUSIONS: Post SG, acyl-ghrelin initiates a temporal hierarchy of molecular events in the gut-brain axis that may both explain the sustained lower body weight and suggest intervention into the cannabinoid pathways for additional therapeutic benefits.

Pancreatic neuroendocrine tumours.

Pancreatic neuroendocrine tumours are rare tumours ( approximately 1/100,00 population/year) of which 60% are non-functioning. Except for insulinoma all types are malignant in >50% of cases. In multiple endocrine neoplasia (MEN)1, pancreatic neuroendocrine tumours occur in 40-80% of patients and are mostly non-functioning tumours or gastrinomas. Insulinomas are benign in approximately 90%, solitary in 95% of sporadic cases whilst multiple in 90% of MEN1 patients. In contrast approximately 50% gastrinomas and the majority of non-functioning pancreatic neuroendocrine tumours are malignant. Pancreatic neuroendocrine tumours occur in 10-15% of patients with Von Hippel-Lindau (VHL) and are frequently multiple (>30%). Surgical excision is a key aspect of treatment for all cases of sporadic gastrinoma and if >2.5 cm in MEN1. Insulinomas are enucleated if solitary and may require pancreatectomy if multiple. Non-functioning tumours should also be resected if sporadic and if >2 cm in MEN1 or if >2-3 cm in VHL. Tumours <1cm require yearly follow-up by CT or MRI from an early age in VHL. The local treatment for liver metastases is now well established and options include liver resection, chemoembolisation and radiofrequency ablation. Systemic therapies have also been better defined and include radionuclide therapy against somatostatin receptors or MIBG and chemotherapy especially for poorly differentiated tumours. A number of novel agents are currently in clinical development.

Liver histology alterations during carbon dioxide pneumoperitoneum in a porcine model.

BACKGROUND: This study aimed to investigate the time course changes in liver histology during carbon dioxide (CO(2)) pneumoperitoneum in a large animal model. METHODS: For this study, 14 white pigs were anesthetized. Liver biopsies performed 0, 1, and 2 h after establishment of CO(2) pneumoperitoneum (at 12 mmHg) and after peritoneal desufflation were sent for histologic examination. Heart rate, mean blood pressure, hepatic artery flow, portal vein flow, and aortic flow were recorded in 10-min increments. Three animals served as control subjects. RESULTS: A statistically significant time course increase was observed in portal inflammation, intralobular inflammation, edema, sinusoidal dilation, sinusoidal hyperemia, centrilobular dilation, centrilobular hyperemia, pericentrilobular ischemia, and focal lytic necrosis scores. There were no significant changes in the control group. This eliminated an effect of anesthesia only. The portal vein flow increased as much as 21%, and the hepatic artery flow decreased as much as 31% of baseline, but these differences did not attain statistical significance. Aortic flow remained relatively stable. CONCLUSION: Histomorphologic changes occurred, indicating liver tissue injury during CO(2) pneumoperitoneum at an intraabdominal pressure of 12 mmHg in the porcine model. Portal vein flow increased, and hepatic artery flow decreased, whereas aortic flow remained relatively unaffected in this experiment.

When is pancreatitis considered to be of biliary origin and what are the implications for management?

Acute pancreatitis is a disease caused by gallstones in 40-60% of patients. Identification of these patients is extremely important, since there are specific therapeutic interventions by endoscopic sphincterotomy and/or cholecystectomy. The combination of trans-abdominal ultrasound (stones in the gallbladder and/or main bile duct) and elevated serum alanine transaminase (circa >60 IU/l within 48 h of presentation) indicates gallstones as the cause in the majority of patients with acute pancreatitis. In the presence of a severe attack this is a strong indication for intervention by endoscopic sphincterotomy. The presence of a significant main bile duct dilatation is also strongly indicative of gallstones and should prompt the use of endoluminal ultrasonography: >8 mm diameter with gallbladder in situ, or >10 mm following cholecystectomy if aged <70 years and >12 mm, respectively, if > or = 70 years. In mild pancreatitis surgically fit patients should be treated by cholecystectomy, and intra-operative cholangiography, as pre-operative biliary imaging is not efficient in this setting. Patients who are not fit for cholecystectomy should undergo prophylactic endoscopic sphincterotomy to prevent further attacks. In the post-acute-phase, pancreatitis patients in whom the aetiology is uncertain should undergo endoluminal ultrasonography. Thisis the most sensitive method for the detection of cholelithiasis and choledocholithiasis and may reveal alternative aetiological factors such as a small ampullary or pancreatic cancer. A number of recent studies have shown that bile crystal analysis, a marker for microlithiasis, increases the yield of positive results over and above endoluminal ultrasonography, and should be considered as part of the modern investigative algorithm.

Meta-analysis of endoscopy and surgery versus surgery alone for common bile duct stones with the gallbladder in situ.

BACKGROUND: There is no clear consensus on the better therapeutic approach (endoscopic versus surgical) to choledocholithiasis. This study is a meta-analysis of the available evidence. METHODS: A search of the Medline and ISI databases identified 12 studies that met the inclusion criteria for data extraction. The analysis was performed using a random-effects model. The outcome was calculated as an odds ratio (OR) or relative risk (RR) with 95 per cent confidence intervals (c.i.). RESULTS: Outcomes of 1357 patients were studied. There was no significant difference in successful duct clearance (OR 0.85 (95 per cent c.i. 0.64 to 1.12); P = 0.250), mortality (RR 1.79 (95 per cent c.i. 0.66 to 4.83); P = 0.250), total morbidity (RR 0.89 (95 per cent 0.71 c.i. to 1.13); P = 0.350), major morbidity (RR 1.34 (95 per cent c.i. 0.92 to 1.97); P = 0.130) or need for additional procedures (OR 1.37 (95 per cent c.i. 0.82 to 2.29); P = 0.230) between the endoscopic and surgical groups. There was also no significant difference between the endoscopic and laparoscopic surgery groups. CONCLUSION: Both approaches have similar outcomes, and treatment should be determined by local resources and expertise.

Algorithm for the diagnosis and treatment of acute biliary pancreatitis.

Gallstones are the commonest cause of acute pancreatitis in developed countries. There is now a considerable evidence base consolidated by a series of systematic reviews, meta-analyses and guidelines that has established a clear algorithm for diagnosis and management. In the majority of patients the combination of ultrasonography and serum alanine transaminase > or = 60 iu/l < or = 48 hours of symptoms will identify gallstones as the cause. The simplest method of severity assessment is a high level of serum C-reactive protein (> 150 mg/l up to 72 hours after symptoms). In mild disease, all fit patients must undergo laparoscopic cholecystectomy with intraoperative cholangiography or if not fit for surgery then endoscopic sphincterotomy during the same admission to prevent further attacks. All patients with severe disease should undergo endoscopic sphincterotomy in less than 72 hours. Patients with > 30% necrosis should undergo fine needle aspiration for bacteriology. Necrosectomy is indicated for infected necrosis or sterile necrosis if there are persisting clinically significant symptoms. There is increasing evidence for the use of minimally invasive pancreatic necrosectomy. Enteral nutrition should be instituted whenever possible but antibiotics should be reserved for patients with proven sepsis. The presence of fungal infection requires active anti-fungal therapy. Patients with severe disease should undergo cholecystectomy at a later stage. Patients who have undergone necrosectomy require long-term follow-up because of delayed complications.

Meta-analysis of the value of somatostatin and its analogues in reducing complications associated with pancreatic surgery.

BACKGROUND: The role of somatostatin and its analogues in reducing complications after pancreatic resection is controversial. This is a meta-analysis of the evidence of benefit. METHODS: A literature search using Medline and ISI Proceedings with exploration of the references identified 22 studies. Of these, ten met the inclusion criteria for data extraction. Estimates of effectiveness were performed using fixed- and random-effects models. The effect was calculated as an odds ratio (OR) with 95 per cent confidence intervals (c.i.) using the Mantel-Haenszel method. Level of significance was set at P < 0.050. RESULTS: Outcomes for 1918 patients were compared. Somatostatin and its analogues did not reduce the mortality rate after pancreatic surgery (OR 1.17 (0.70 to 1.94); P = 0.545) but did reduce both the total morbidity (OR 0.62 (0.46 to 0.85); P = 0.003) and pancreas-specific complications (OR 0.56 (0.39 to 0.81); P = 0.002). Somatostatin and its analogues reduced the rate of biochemical fistula (OR 0.45 (0.33 to 0.62); P < 0.001) but not the incidence of clinical anastomotic disruption (OR 0.80 (0.44 to 1.45); P = 0.459). CONCLUSION: Somatostatin and its analogues reduce the incidence of complications after surgery.

Serum CA19-9 measurement increases the effectiveness of staging laparoscopy in patients with suspected pancreatic malignancy.

BACKGROUND/AIMS: Staging laparoscopy for suspected pancreatic neoplasia is not widely accepted due to its low yield. The aim of this study was to determine if serum carbohydrate antigen (CA19-9) levels could be used to improve the selection of patients for staging laparoscopy. METHODS: The data from a prospectively collected database (1997-2004) with 159 patients who had computed tomography-predicted resectable disease and who had undergone laparoscopic staging were analysed to determine if a low preoperative CA19-9 level (< or =150 kU/l, or < or =300 kU/l with a bilirubin >35 micromol/l) identified patients in whom laparoscopy was not useful. RESULTS: The CA19-9 level was >150 kU/l in 96 patients of whom 75 (78%) were considered resectable following laparoscopic assessment. There were 63 patients with a CA19-9 < or =150 kU/l of whom 60 (95%) were considered resectable following laparoscopic assessment. The sensitivity, specificity, positive predictive value and negative predictive value for CA19-9 < or =150 kU/l in predicting that laparoscopic assessment would judge patients as resectable were 44, 88, 95 and 22%, respectively. A cut-off level of < or =300 kU/l in patients with a bilirubin >35 micromol/l produced values of 30, 94, 94 and 28%, respectively. By using CA19-9 < or =150 kU/l, laparoscopy could have been avoided in 40% of patients, increased to 55% of patients with adjustment for the presence of jaundice; concomitantly, the yield from laparoscopy would have been increased from 15 to 22 and 25%, respectively. CONCLUSION: Use of serum CA19-9 levels would increase the efficiency of laparoscopic staging in patients with suspected pancreatic malignancy.

Early and late complications after pancreatic necrosectomy.

BACKGROUND: Surgery for pancreatic necrosis is associated with a high morbidity and mortality. The aim of this study was to review the incidence of early and late complications after pancreatic necrosectomy in a large contemporary series of patients. METHODS: The clinical outcomes of 88 patients who underwent pancreatic necrosectomy between 1997 and 2003 were reviewed. RESULTS: The median age was 55.5 (range, 18-85) years, 54 (61%) were males, 68 (77%) had primary pancreatic infection, 71 (81%) had >50% necrosis, and the median admission Acute Physiology and Chronic Health Evaluation score was 9 (range, 1-21). Median time to surgery was 31 (range, 1-161) days; 47 patients underwent minimally invasive necrosectomy and 41 open necrosectomy; 81 (92%) of patients had complications postoperatively, and 25 (28%) died. Multiorgan failure (odds ratio = 3.4, P = .05) and hemorrhage (odds ratio = 6.1, P = .03) were the only independent predictors of mortality. During a median follow-up of 28.9 months, 39 (62%) of 63 surviving patients had one or more late complications: biliary stricture in 4 (6%), pseudocyst in 5 (8%), pancreatic fistula in 8 (13%), gastrointestinal fistula in 1 (2%), delayed collections in 3 (5%), and incisional hernia in 1 (2%); intervention was required in 10 (16%) patients. Sixteen (25%) of 63 surviving patients developed exocrine insufficiency, and 19 (33%) of 58 without prior diabetes mellitus developed endocrine insufficiency. CONCLUSIONS: Almost all patients undergoing necrosectomy developed significant early or late complications or both. Multiorgan failure and postoperative hemorrhage were independent predictors of mortality. Long-term follow-up was important because 62% developed complications, and 16% of those with complications required surgical or endoscopic intervention.

T cell lymphoplasmacellular and eosinophilic infiltration of the pancreas with involvement of the gallbladder and duodenum in non-alcoholic duct-destructive chronic pancreatitis.

BACKGROUND: Non-alcoholic duct destructive chronic pancreatitis is a rare entity with specific pathological features. The majority of the patients are from Japan. We report a case with involvement of the distal bile duct, the gallbladder, the duodenum and the ampulla, and present a review of patients from Europe and the USA since 1997. CASE PRESENTATION: A 56-year-old man presented with a 3-month history of mild acute pancreatitis and obstructive jaundice, followed by increasing weight loss, lethargy and epigastric pain. CT showed a mass in the head of the pancreas. ERCP demonstrated a smooth stricture of the intra-pancreatic main bile duct and an irregular, incomplete, stricture in the main pancreatic duct. A pancreatic cancer could not be reliably excluded, and, therefore, he underwent a pylorus-preserving Kausch-Whipple's pancreatoduodenectomy. RESULTS: Histopathology showed typical peri-ductal T cell-rich lymphoplasmacellular and eosinophilic infiltration of the pancreas, with involvement of the distal bile duct but, also, unusual inflammatory infiltration of the gallbladder, the duodenum and the ampulla. CONCLUSION: The inflammatory process in non-alcoholic duct-destructive chronic pancreatitis can affect the entire pancreato-biliary region and mimics pancreatic cancer. Currently, there are no definitive criteria for pre-operative diagnosis, so it is very difficult for one to avoid resection.

Current standards of surgery for pancreatic cancer.

BACKGROUND: Pancreatic cancer carries a dismal prognosis but there has been a vast increase in evidence on its management in the past decade. METHODS: An electronic and manual search was performed for articles on the surgical treatment of pancreatic cancer published in the past 10 years. RESULTS: Six major areas of advancement were identified. Groups at high risk of developing pancreatic cancer, notably those with chronic pancreatitis and hereditary pancreatitis, have been defined, raising the need for secondary screening. Methods of staging pancreatic cancer for resection have greatly improved but accuracy is still only 85-90 per cent. Pylorus-preserving partial pancreatoduodenectomy without extended lymphadenectomy is the simplest procedure; it does not compromise long-term survival. Adjuvant chemotherapy significantly improves long-term survival. Patients who are free from major co-morbidity have better palliation by surgery (with a double bypass) than by endoscopy. High-volume centres improve the results of surgery for all outcome measures including long-term survival. CONCLUSION: The surgical management of pancreatic cancer has undergone a significant change in the past decade. It has moved away from no active treatment. The standard of care can now be defined as potentially curative resection in a specialist centre followed by adjuvant systemic chemotherapy.

Survival of patients with periampullary carcinoma is predicted by lymph node 8a but not by lymph node 16b1 status.

BACKGROUND: The aim of this study was to assess the impact of metastatic disease in lymph nodes 8a and 16b1 (as defined by the Japanese Pancreas Society) on survival in patients with periampullary malignancy. METHODS: Patients undergoing resection for primary pancreatic ductal adenocarcinoma or intrapancreatic bile duct adenocarcinoma were identified from a prospective database (September 1997-May 2003). RESULTS: Thirteen of 54 and ten of 44 evaluable patients had metastatic involvement of lymph nodes 8a and 16b1 respectively. Metastatic involvement of lymph node 8a was associated with a significantly shorter median survival (197 versus 470 days; P = 0.003) but metastatic involvement of lymph node 16b1 did not affect survival (457 versus 503 days; P = 0.185). Multivariate analysis showed lymph node 8a status to be the strongest predictor of outcome (P = 0.006). Median survival of those with metastatic lymph node 8a was not significantly different from that of 81 patients with overt metastatic periampullary cancer at the time of diagnosis (98 days; P = 0.072) CONCLUSION: Lymph node 8a was an independent prognostic factor in patients with periampullary malignancy, but lymph node 16b1 was not. Survival in those with metastatic lymph node 8a was not significantly different from that in patients with metastatic disease at presentation. Preoperative determination of lymph node 8a status may have important implications in selecting patients for treatment.

Fungal infection but not type of bacterial infection is associated with a high mortality in primary and secondary infected pancreatic necrosis.

INTRODUCTION: Knowledge of microbiology in the prognosis of patients with necrotizing pancreatitis is incomplete. AIM: This study compared outcomes based on primary and secondary infection after surgery for pancreatic necrosis. METHOD: From a limited prospective database of pancreatic necrosectomy, a retrospective case note review was performed (October 1996 to April 2003). RESULTS: 55 of 73 patients had infected pancreatic necrosis at the first necrosectomy. 25 of 47 patients had resistant bacteria to prophylactic antibiotics (n = 21) or did not receive prophylactic antibiotics (n = 4), but this was not associated with a higher mortality (9 of 25) compared to those with sensitive organisms (4 of 22). Patients with fungal infection (n = 6) had a higher initial median (95% CI) APACHE II score compared to those without (11 (9-13) verus 8.5 (7-10), p = 0.027). Five of six patients with fungal infection died compared to 13 of 47 who did not (p = 0.014). With the inclusion of secondary infections 21 (32%) of 66 patients had fungal infection with 10 (48%) deaths compared to 11 (24%) of 45 patients without fungal infection (p = 0.047). CONCLUSION: Whether associated with primary or secondary infected pancreatic necrosis, fungal but not bacterial infection was associated with a high mortality.

Influence of opioid use on surgical and long-term outcome after resection for chronic pancreatitis.

BACKGROUND: The outcome of pancreatic resection for chronic pancreatitis in patients with preoperative opioid use is not well described. METHODS: During 1997 to 2003, 112 of 231 patients referred with chronic pancreatitis underwent pancreatic resection. The outcome of patients who had preoperative opioid use (N=46) was compared with those without (N=66). RESULTS: Patients who used opioids presented at a younger age and had a younger age of symptom onset, longer symptom duration, more hospitalizations, a higher frequency of diabetes mellitus, a higher pain score, and more restriction in daily activity (all P<.05). Twenty-one (46%) patients with opioid use had a total pancreatectomy compared with 9 (14%) without opioid use (P=.0002); the 21 patients also had a higher frequency of postoperative bleeding and early reoperation (8 vs 2, P<.02; 11 vs 3, P=.003, respectively). Mortality and overall morbidity was not significantly different between the 2 groups (4 vs 1, 27 vs 34, respectively). Pain scores improved postoperatively in both groups (P=.001) and was not significantly different between the groups from 12 months onward (median follow-up of 12 months, range, 3-60 months). Twenty percent of patients who used preoperative opioids however reverted to morphine use compared with 6% of patients who had not used opioids. CONCLUSIONS: Patients who used opioids had more advanced disease than patients without opioid use, accounting for part of the postoperative morbidity. Although long-term pain relief was comparable between the 2 groups, maintaining opioid withdrawal was more problematic in those with preoperative opioid use. Earlier referral for resection may be warranted in this group of patients.

Major resection for chronic pancreatitis in patients with vascular involvement is associated with increased postoperative mortality.

BACKGROUND: The aim was to evaluate the outcome of major resection for chronic pancreatitis in patients with and without vascular involvement. METHODS: Of 250 patients with severe chronic pancreatitis referred between 1996 and 2003, 112 underwent pancreatic resection. The outcome of 17 patients (15.2 per cent) who had major vascular involvement was compared with that of patients without vascular involvement. RESULTS: The 95 patients without vascular involvement had resections comprising Beger's operation (39 patients), Kausch-Whipple pancreatoduodenectomy (28), total pancreatectomy (25) and left pancreatectomy (three). Twenty-five major vessels were involved in the remaining 17 patients. One or more major veins were occluded and/or compressed producing generalized or segmental portal hypertension, and three patients also had major arterial involvement. Surgery in these patients comprised Beger's operation (eight), total pancreatectomy (five), Kausch-Whipple pancreatoduodenectomy (two) and left pancreatectomy (two). Perioperative mortality rates were significantly different between the groups (two of 95 versus three of 17 respectively; P = 0.024). There were similar and significant improvements in long-term outcomes in both groups. CONCLUSION: Resection for severe chronic pancreatitis in patients with vascular complications is hazardous and is associated with an increased mortality rate. Vascular assessment should be included in the routine follow-up of patients with chronic pancreatitis, to enable early identification of those likely to develop vascular involvement and prompt surgical intervention.

Surgical treatment of pancreatic fistula.

BACKGROUND: Pancreatic fistula remains a significant problem in pancreatic disease, trauma and surgery. Whilst improved diagnostic and treatment techniques, including endoscopic approaches, have resulted in considerably improved outcomes, surgical intervention remains an important aspect of treatment but has been relatively poorly documented. AIMS: The aims were to review the recent world literature on the relative incidence of pancreatic fistula and the results of surgical treatment. RESULTS: The pancreatic fistula rate following partial pancreato-duodenectomy was 421 (12.9%) in 3,268 patients in 13 large series; 80 (13.0%) in 671 patients after left pancreatectomy in 6 large series, and 28 (11.9%) in 243 patients after pancreatic trauma in 4 recent series. The success rate of surgical procedures for external pancreatic fistulae was 101 (90.2%) in 112 patients with an overall mortality of 7 (6.3%) reported in 9 series. For internal pancreatic fistulae the success rate of surgical treatment was 61 (92%) in 66 patients with an overall mortality of 6 (9%) reported in 7 series. CONCLUSIONS: The treatment of established pancreatic fistula remains challenging. Although surgical treatment is reserved for patients who have failed all other treatments, the success rate is 90-92% but with a mortality of 6-9%.

Hereditary pancreatic endocrine tumours.

The two main types of hereditary pancreatic neuroendocrine tumours are found in multiple endocrine neoplasia type 1 (MEN-1) and von Hippel-Lindau disease (VHL), but also in the rarer disorders of neurofibromatosis type 1 and tuberous sclerosis. This review considers the major advances that have been made in genetic diagnosis, tumour localization, medical and surgical treatment and palliation with systemic chemotherapy and radionuclides. With the exception of the insulinoma syndrome, all of the various hormone excess syndromes of MEN-1 can be treated medically. The role of surgery however remains controversial ranging from no intervention (except enucleation for insulinoma), intervening for tumours diagnosed only by biochemical criteria, intervening in those tumours only detected radiologically (1-2 cm in diameter) or intervening only if the tumour diameter is > 3 cm in diameter. The extent of surgery is also controversial, although radical lymphadenectomy is generally recommended. Pancreatic tumours associated with VHL are usually non-functioning and tumours of at least 2 cm in diameter should be resected. Practice guidelines recommend that screening in patients with MEN-1 should commence at the age of 5 years for insulinoma and at the age of 20 years for other pancreatic neuroendocrine tumours and variously at 10-20 years of age for pancreatic tumours in patients with VHL. The evidence is increasing that the life span of patients may be significantly improved with surgical intervention, mandating the widespread use of tumour surveillance and multidisciplinary team management.

Sealed perforated duodenal ulcer discovered during elective laparoscopic cholecystectomy.

During laparoscopic cholecystectomy (LC) there is possibility of discovering concomitant occult intra-abdominal pathology and the surgeon must be prepared to undertake the appropriate procedure. We evaluated the incidence of latent perforated duodenal ulcer in 5539 patients who underwent LC for gallstone disease at our unit between November 1991 and November 2001. Seven (0.13 %) cases of perforated duodenal ulcer were discovered. Following the diagnosis of the perforation, laparoscopic suturing and omental patch repair was carried out in four patients, open repair with selective vagotomy and pyloroplasty in two patients and an open suture and omental patch repair in one patient. In all cases the cholesystectomy was completed successfully.