Enhanced recovery after surgery: Current status and future progress.

Enhanced Recovery After Surgery (ERAS) pathways were first introduced almost a quarter of a century ago and represent a paradigm shift in perioperative care that reduced postoperative complications and hospital length of stay, improved postoperative quality of life, and reduced overall healthcare costs. Gradual recognition of the generalizability of the interventions and transferable improvements in postoperative outcomes, led them to become standard of care for several surgical procedures. In this article, we critically review the current status of ERAS pathways, address related controversies, and propose measures for future progress.

Postoperative arginine-enriched immune modulating nutrition: Long-term survival results from a randomised clinical trial in patients with oesophagogastric and pancreaticobiliary cancer.

BACKGROUND & AIMS: Immune modulating nutrition (IMN) has been shown to reduce postoperative infectious complications and length of stay in patients with gastrointestinal cancer. Two studies of IMN in patients undergoing surgery for head and neck cancer also suggested that this treatment might improve long-term survival and progression-free survival. In the present study, we analysed follow-up data from our previous randomised controlled trial of IMN, in patients undergoing surgery for oesophagogastric and pancreaticobiliary cancer, in order to evaluate the long-term impact on survival of postoperative IMN versus an isocaloric, isonitrogenous control feed. METHODS: This study included patients undergoing surgery for cancers of the pancreas, oesophagus and stomach, who had been randomised in a double-blind manner to receive postoperative jejunostomy feeding with IMN (Stresson, Nutricia Ltd.) or an isonitrogenous, isocaloric feed (Nutrison High Protein, Nutricia) for 10-15 days. The primary outcome was long-term overall survival. RESULTS: There was complete follow-up for all 108 patients, with 54 patients randomised to each group. There were no statistically significant differences between groups by demographics [(age, p = 0.63), sex (p = 0.49) or site of cancer (p = 0.25)]. 30-day mortality was 11.1% in both groups. Mortality in the intervention group was 13%, 31.5%, 70.4%, 85.2%, 88.9%, and 96.3% at 90 days, and 1, 5, 10, 15 and 20 years respectively. Corresponding mortality in the control group was 14.8%, 35.2%, 68.6%, 79.6%, 85.2% and 98.1% (p > 0.05 for all comparisons). CONCLUSION: Early postoperative feeding with arginine-enriched IMN had no impact on long-term survival in patients undergoing surgery for oesophagogastric and pancreaticobiliary cancer.

Perioperative nutrition: Recommendations from the ESPEN expert group.

BACKGROUND & AIMS: Malnutrition has been recognized as a major risk factor for adverse postoperative outcomes. The ESPEN Symposium on perioperative nutrition was held in Nottingham, UK, on 14-15 October 2018 and the aims of this document were to highlight the scientific basis for the nutritional and metabolic management of surgical patients. METHODS: This paper represents the opinion of experts in this multidisciplinary field and those of a patient and caregiver, based on current evidence. It highlights the current state of the art. RESULTS: Surgical patients may present with varying degrees of malnutrition, sarcopenia, cachexia, obesity and myosteatosis. Preoperative optimization can help improve outcomes. Perioperative fluid therapy should aim at keeping the patient in as near zero fluid and electrolyte balance as possible. Similarly, glycemic control is especially important in those patients with poorly controlled diabetes, with a stepwise increase in the risk of infectious complications and mortality per increasing HbA1c. Immobilization can induce a decline in basal energy expenditure, reduced insulin sensitivity, anabolic resistance to protein nutrition and muscle strength, all of which impair clinical outcomes. There is a role for pharmaconutrition, pre-, pro- and syn-biotics, with the evidence being stronger in those undergoing surgery for gastrointestinal cancer. CONCLUSIONS: Nutritional assessment of the surgical patient together with the appropriate interventions to restore the energy deficit, avoid weight loss, preserve the gut microbiome and improve functional performance are all necessary components of the nutritional, metabolic and functional conditioning of the surgical patient.

The impact of peri-operative intravenous lidocaine on postoperative outcome after elective colorectal surgery: A meta-analysis of randomised controlled trials.

BACKGROUND: There has recently been increasing interest in the use of peri-operative intravenous lidocaine (IVL) due to its analgesic, anti-inflammatory and opioid-sparing effects. However, these potential benefits are not well established in elective colorectal surgery. OBJECTIVES: To examine the effect of peri-operative IVL infusion on postoperative outcome in patients undergoing elective colorectal surgery. DESIGN: A meta-analysis of randomised controlled trials (RCTs) comparing peri-operative IVL with placebo infusion in elective colorectal surgery. The primary outcome measure was postoperative pain scores up to 48 h. The secondary outcome measures included time to return of gastrointestinal function, postoperative morphine requirement, anastomotic leak, local anaesthetic toxicity and hospital length of stay. DATA SOURCES: PubMed, Scopus and the Cochrane Library databases were searched on 5 November 2018. ELIGIBILITY CRITERIA: Studies were included if they were RCTs evaluating the role of peri-operative IVL vs. placebo in adult patients undergoing elective colorectal surgery. Exclusion criteria were paediatric patients, noncolorectal or emergency procedures, non-RCT methodology or lack of relevant outcome measures. RESULTS: A total of 10 studies were included (n = 508 patients; 265 who had undergone IVL infusion, 243 who had undergone placebo infusion). IVL infusion was associated with a significant reduction in time to defecation (mean difference -12.06 h, 95% CI -17.83 to -6.29, I = 93%, P = 0.0001), hospital length of stay (mean difference -0.76 days, 95% CI -1.32 to -0.19, I = 45%, P = 0.009) and postoperative pain scores at early time points, although this difference does not meet the threshold for a clinically relevant difference. There was no difference in time to pass flatus (mean difference -5.33 h, 95% CI -11.53 to 0.88, I = 90%, P = 0.09), nor in rates of surgical site infection or anastomotic leakage. CONCLUSION: This meta-analysis provides some support for the administration of peri-operative IVL infusion in elective colorectal surgery. However, further evidence is necessary to fully elucidate its potential benefits in light of the high levels of study heterogeneity and mixed quality of methodology.

Computed tomography-based psoas skeletal muscle area and radiodensity are poor sentinels for whole L3 skeletal muscle values.

BACKGROUND AND AIMS: Computed tomography (CT)-based measurement of skeletal muscle cross-sectional area (CSA) and Hounsfield unit (HU) radiodensity are used to assess the presence of sarcopenia and myosteatosis, respectively. The validated CT-based technique involves analysis of skeletal muscle at the third lumbar vertebral (L3) level. Recently there has been increasing interest in the use of psoas muscle alone as a sentinel. However, this technique has not been extensively investigated or compared with the previous validated standard approach. METHODS: Portovenous phase CT images at the L3 level were identified retrospectively from a single institution in 150 patients who had non-emergency scans and were analysed by a single assessor using SliceOmatic software v5.0 (TomoVision, Canada). Manual segmentation based upon validated HU thresholds for skeletal muscle density was performed for all skeletal muscle, as well as the individual muscle groups. The muscle CSA and mean radiodensity of each group were compared against the whole L3 slice values. RESULTS: When compared with whole L3 slice CSA, anterior abdominal wall CSA had the strongest correlation (r = 0.9315, p < 0.0001) followed by paravertebral (r = 0.8948, p < 0.0001), then psoas muscle (r = 0.7041, p < 0.0001). The mean ± SD density of the psoas muscle (42 ± 8.4 HU) was significantly higher than the whole slice radiodensity (32.3 ± 9.5 HU, p < 0.0001), with paravertebral radiodensity being a more accurate estimation (34.5 ± 10.8 HU). There was a significant difference in the prevalence of myosteatosis when the density measured from the psoas was compared with that of the whole L3 skeletal muscle (27.7% vs. 66.0%, p < 0.0001). CONCLUSION: Whole L3 slice CSA correlated positively with psoas muscle CSA but was subject to wide variability in results. Psoas muscle radiodensity was significantly greater than whole L3 slice density and resulted in underestimation of the prevalence of myosteatosis. Given the lack of equivalence from individual muscle groups, we recommend that further work be undertaken to investigate which muscle group, or indeed whether the gold standard of whole L3 skeletal muscle, provides the best correlation with clinical outcomes.

A comparison of two different software packages for analysis of body composition using computed tomography images.

OBJECTIVES: The analysis of body composition from computed tomography (CT) imaging has become widespread. However, the methodology used is far from established. Two main software packages are commonly used for body composition analysis, with results used interchangeably. However, the equivalence of these has not been well established. The aim of this study was to compare the results of body composition analysis performed using the two software packages to assess their equivalence. METHODS: Triphasic abdominal CT scans from 50 patients were analyzed for a range of body composition measures at the third lumbar vertebral level using OsiriX (v7.5.1, Pixmeo, Switzerland) and SliceOmatic (v5.0, TomoVision, Montreal, Canada) software packages. Measures analyzed were skeletal muscle index (SMI), fat mass (FM), fat-free mass (FFM), and mean skeletal muscle Hounsfield Units (SMHU). RESULTS: The overall mean SMI calculated using the two software packages was significantly different (SliceOmatic 51.33 versus OsiriX 53.77, P < 0.0001), and this difference remained significant for non-contrast and arterial scans. When FM and FFM were considered, again the results were significantly different (SliceOmatic 33.7 versus OsiriX 33.1 kg, P < 0.0001; SliceOmatic 52.1 versus OsiriX 54.2 kg, P < 0.0001, respectively), and this difference remained for all phases of CT. Finally, when analyzed, mean SMHU was also significantly different (SliceOmatic 32.7 versus OsiriX 33.1 HU, P = 0.046). CONCLUSIONS: All four body composition measures were statistically significantly different by the software package used for analysis; however, the clinical significance of these differences is doubtful. Nevertheless, the same software package should be used if serial measurements are being performed.

The Role of Oral Antibiotic Preparation in Elective Colorectal Surgery: A Meta-analysis.

OBJECTIVES: To compare the impact of the use of oral antibiotics (OAB) with or without mechanical bowel preparation (MBP) on outcome in elective colorectal surgery. SUMMARY BACKGROUND DATA: Meta-analyses have demonstrated that MBP does not impact upon postoperative morbidity or mortality, and as such it should not be prescribed routinely. However, recent evidence from large retrospective cohort and database studies has suggested that there may be a role for combined OAB and MBP, or OAB alone in the prevention of surgical site infection (SSI). METHODS: A meta-analysis of randomized controlled trials and cohort studies including adult patients undergoing elective colorectal surgery, receiving OAB with or without MBP was performed. The outcome measures examined were SSI, anastomotic leak, 30-day mortality, overall morbidity, development of ileus, reoperation and Clostridium difficile infection. RESULTS: A total of 40 studies with 69,517 patients (28 randomized controlled trials, n = 6437 and 12 cohort studies, n = 63,080) were included. The combination of MBP+OAB versus MBP alone was associated with a significant reduction in SSI [risk ratio (RR) 0.51, 95% confidence interval (CI) 0.46-0.56, P < 0.00001, I = 13%], anastomotic leak (RR 0.62, 95% CI 0.55-0.70, P < 0.00001, I = 0%), 30-day mortality (RR 0.58, 95% CI 0.44-0.76, P < 0.0001, I = 0%), overall morbidity (RR 0.67, 95% CI 0.63-0.71, P < 0.00001, I = 0%), and development of ileus (RR 0.72, 95% CI 0.52-0.98, P = 0.04, I = 36%), with no difference in Clostridium difficile infection rates. When a combination of MBP+OAB was compared with OAB alone, no significant difference was seen in SSI or anastomotic leak rates, but there was a significant reduction in 30-day mortality, and incidence of postoperative ileus with the combination. There is minimal literature available on the comparison between combined MBP+OAB versus no preparation, OAB alone versus no preparation, and OAB versus MBP. CONCLUSIONS: Current evidence suggests a potentially significant role for OAB preparation, either in combination with MBP or alone, in the prevention of postoperative complications in elective colorectal surgery. Further high-quality evidence is required to differentiate between the benefits of combined MBP+OAB or OAB alone.

Intestinal failure in adults: Recommendations from the ESPEN expert groups.

BACKGROUND & AIMS: Intestinal failure (IF) is defined as "the reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that intravenous supplementation is required to maintain health and/or growth". Functionally, it may be classified as type I acute intestinal failure (AIF), type II prolonged AIF and type III chronic intestinal failure (CIF) The ESPEN Workshop on IF was held in Bologna, Italy, on 15-16 October 2017 and the aims of this document were to highlight the current state of the art and future directions for research in IF. METHODS: This paper represents the opinion of experts in the field, based on current evidence. It is not a formal review, but encompasses the current evidence, with emphasis on epidemiology, classification, diagnosis and management. RESULTS: IF is the rarest form of organ failure and can result from a variety of conditions that affect gastrointestinal anatomy and function adversely. Assessment, diagnosis, and short and long-term management involves a multidisciplinary team with diverse expertise in the field that aims to reduce complications, increase life expectancy and improve quality of life in patients. CONCLUSIONS: Both AIF and CIF are relatively rare conditions and most of the published work presents evidence from small, single-centre studies. Much remains to be investigated to improve the diagnosis and management of IF and future studies should rely on multidisciplinary, multicentre and multinational collaborations that gather data from large cohorts of patients. Emphasis should also be placed on partnership with patients, carers and government agencies in order to improve the quality of research that focuses on patient-centred outcomes that will help to improve both outcomes and quality of life in patients with this devastating condition.

Impact of mechanical bowel preparation in elective colorectal surgery: A meta-analysis.

AIM: To analyse the effect of mechanical bowel preparation vs no mechanical bowel preparation on outcome in patients undergoing elective colorectal surgery. METHODS: Meta-analysis of randomised controlled trials and observational studies comparing adult patients receiving mechanical bowel preparation with those receiving no mechanical bowel preparation, subdivided into those receiving a single rectal enema and those who received no preparation at all prior to elective colorectal surgery. RESULTS: A total of 36 studies (23 randomised controlled trials and 13 observational studies) including 21568 patients undergoing elective colorectal surgery were included. When all studies were considered, mechanical bowel preparation was not associated with any significant difference in anastomotic leak rates (OR = 0.90, 95%CI: 0.74 to 1.10, P = 0.32), surgical site infection (OR = 0.99, 95%CI: 0.80 to 1.24, P = 0.96), intra-abdominal collection (OR = 0.86, 95%CI: 0.63 to 1.17, P = 0.34), mortality (OR = 0.85, 95%CI: 0.57 to 1.27, P = 0.43), reoperation (OR = 0.91, 95%CI: 0.75 to 1.12, P = 0.38) or hospital length of stay (overall mean difference 0.11 d, 95%CI: -0.51 to 0.73, P = 0.72), when compared with no mechanical bowel preparation, nor when evidence from just randomized controlled trials was analysed. A sub-analysis of mechanical bowel preparation vs absolutely no preparation or a single rectal enema similarly revealed no differences in clinical outcome measures. CONCLUSION: In the most comprehensive meta-analysis of mechanical bowel preparation in elective colorectal surgery to date, this study has suggested that the use of mechanical bowel preparation does not affect the incidence of postoperative complications when compared with no preparation. Hence, mechanical bowel preparation should not be administered routinely prior to elective colorectal surgery.

Body composition measurement using computed tomography: Does the phase of the scan matter?

OBJECTIVES: The aim of this study was to determine, from the methodologic standpoint, the effect of the presence or absence of intravenous contrast on body composition variables obtained by analysis of computed tomography (CT) images. METHODS: Triphasic abdominal (noncontrast, arterial phase, and portovenous phase contrast) CT scans from 111 patients were analyzed by two independent assessors at the third lumbar vertebral level using SliceOmatic software (version 5.0, TomoVision, Montreal, Canada). Variables included skeletal muscle index (SMI), fat and fat-free mass (FM and FFM, respectively), and mean skeletal muscle Hounsfield units (SMHU). RESULTS: Mean SMHU was lowest in the noncontrast phase (29.4, standard deviation [SD] 8.9 HU), followed by arterial (32.4, SD 9.3 HU) then portovenous phases (34.9, SD 9.4 HU). The mean skeletal muscle attenuation was significantly different depending on the phase of the scan in which the images were obtained. Calculated FM was significantly lower in both arterial (28.6, SD 8.8 kg, P < 0.0001) and portovenous phase scans (28.5, SD 8.9 kg, P < 0.0001) when compared with noncontrast (29.2, SD 8.9 kg). The mean FFM was not significantly different as measured on noncontrast, arterial, or portovenous phase CT scans (48, SD 11.2; 48.1, SD 9.8; and 48.6, SD 10.2 kg, respectively). No difference was seen in SMI. Interobserver reliability was high. CONCLUSIONS: The definition of myosteatosis should include a standardized phase of CT for analysis and this should be incorporated within its definition. However, as the magnitudes of the differences were relatively small, the effect of the phase of the scan on predicting outcome needs to be determined.

Antibiotics Versus Appendicectomy for the Treatment of Uncomplicated Acute Appendicitis: An Updated Meta-Analysis of Randomised Controlled Trials.

BACKGROUND: Uncomplicated acute appendicitis has been managed traditionally by early appendicectomy. However, recently, there has been increasing interest in the potential for primary treatment with antibiotics, with studies finding this to be associated with fewer complications than appendicectomy. The aim of this study was to compare outcomes of antibiotic therapy with appendicectomy for uncomplicated acute appendicitis. METHOD: This meta-analysis of randomised controlled trials included adult patients presenting with uncomplicated acute appendicitis treated with antibiotics or appendicectomy. The primary outcome measure was complications. Secondary outcomes included treatment efficacy, hospital length of stay (LOS), readmission rate and incidence of complicated appendicitis. RESULTS: Five randomised controlled trials with a total of 1430 participants (727 undergoing antibiotic therapy and 703 undergoing appendicectomy) were included. There was a 39 % risk reduction in overall complication rates in those treated with antibiotics compared with those undergoing appendicectomy (RR 0.61, 95 % CI 0.44-0.83, p = 0.002). There was no significant difference in hospital LOS (mean difference 0.25 days, 95 % CI -0.05 to 0.56, p = 0.10). In the antibiotic cohort, 123 of 587 patients initially treated successfully with antibiotics were readmitted with symptoms suspicious of recurrent appendicitis. The incidence of complicated appendicitis was not increased in patients who underwent appendicectomy after "failed" antibiotic treatment (10.8 %) versus those who underwent primary appendicectomy (17.9 %). CONCLUSION: Increasing evidence supports the primary treatment of acute uncomplicated appendicitis with antibiotics, in terms of complications, hospital LOS and risk of complicated appendicitis. Antibiotics should be prescribed once a diagnosis of acute appendicitis is made or considered.

The impact of sarcopenia and myosteatosis on outcomes of unresectable pancreatic cancer or distal cholangiocarcinoma.

BACKGROUND & AIMS: Patients with pancreatic cancer have a poor prognosis, are often cachectic, and frequently demonstrate features of systemic inflammation, which may contribute to the phenomenon of myosteatosis. Analysis of body composition from CT scans has been used to study sarcopenia and its association with prognosis in a number of types of cancer, particular in combination with obesity. It has also been suggested that myosteatosis, defined as attenuated mean skeletal muscle Hounsfield units (HU), is associated with reduced survival in cancer. This study aimed to assess the association between body composition (sarcopenia and myosteatosis) and outcome in patients with unresectable pancreatic cancer. METHODS: All patients diagnosed with unresectable pancreatic cancer at Nottingham University Hospitals NHS Trust between 2006 and 2013 were considered for the study. A total of 228 patients were included retrospectively. Body composition was assessed using cross-sectional CT analysis to calculate a skeletal muscle index (SMI) for sarcopenia and use mean skeletal muscle HU for myosteatosis. RESULTS: The prevalence of sarcopenia in the whole patient group at baseline was 60.5% (138/228). Overall, patients who were sarcopenic had no significant difference in overall survival versus those who were not (p = 0.779). However, patients who were overweight/obese and sarcopenic had a significantly lower survival (p = 0.013). Of the 58 patients who were overweight or obese and sarcopenic, 32 were also myosteatotic. The prevalence of myosteatosis overall at baseline was 55.3% (126/228) and this was associated with significant reduction in overall survival (p = 0.049). Univariate Cox regression revealed myosteatosis but not sarcopenia to be predictive of reduced survival, however this relationship was lost on multivariate testing. Myosteatosis was associated with significantly greater levels of systemic inflammation (white cell count, neutrophil-lymphocyte ratio and C-reactive protein), anaemia and worsening of baseline blood urea. This relationship was not seen with sarcopenia. CONCLUSIONS: This is the largest study on the association between body composition and survival in patients with unresectable pancreatic cancer and has shown that although sarcopenia alone did not have a bearing on survival, the presence of myosteatosis was associated significantly with the presence of systemic inflammation and reduced survival.

Systematic review of the impact of HbA1c on outcomes following surgery in patients with diabetes mellitus.

BACKGROUND & AIMS: Diabetes is a significant risk factor for surgical complications and also increases the prevalence of comorbidities, thereby increasing surgical risk. The aim of this systematic review was to establish the relationship between long-term preoperative glycemic control as measured by HbA1c and postoperative complications. METHODS: A systematic search was conducted to source articles published between 1980 and 2014 pertinent to the review. Full-text articles were included if they met the pre-determined criteria as determined by two reviewers. Studies reporting the impact of preoperative HbA1c levels on postoperative outcomes in all disciplines of surgery were included. RESULTS: Twenty studies, including a total of 19,514 patients with diabetes mellitus from a range of surgical specialties, were suitable for inclusion. Preoperative glycemic control did not have a bearing on 30-day mortality. There were no significant differences in the incidence of stroke, venous thromboembolic disease, hospital readmission and ITU length of stay based on glycemic control. The majority of studies suggested no link between preoperative HbA1c levels and acute kidney injury or need for postoperative dialysis, dysrhythmia, infection not related to the surgical site and total hospital length of stay. The literature was highly variable with regards to myocardial events, surgical site infection and reoperation rates. CONCLUSIONS: Elevated preoperative HbA1c was not definitively associated with increased postoperative morbidity or mortality in patients with diabetes mellitus. The studies included in this review were relatively heterogeneous, predominantly retrospective, and often contained small patient numbers, suggesting that good quality evidence is necessary.

Intraoperative Goal-directed Fluid Therapy in Elective Major Abdominal Surgery: A Meta-analysis of Randomized Controlled Trials.

OBJECTIVES: To compare the effects of intraoperative goal-directed fluid therapy (GDFT) with conventional fluid therapy, and determine whether there was a difference in outcome between studies that did and did not use Enhanced Recovery After Surgery (ERAS) protocols. METHODS: Meta-analysis of randomized controlled trials of adult patients undergoing elective major abdominal surgery comparing intraoperative GDFT versus conventional fluid therapy. The outcome measures were postoperative morbidity, length of stay, gastrointestinal function and 30-day mortality. RESULTS: A total of 23 studies were included with 2099 patients: 1040 who underwent GDFT and 1059 who received conventional fluid therapy. GDFT was associated with a significant reduction in morbidity (risk ratio [RR] 0.76, 95% confidence interval [CI] 0.66-0.89, P = 0.0007), hospital length of stay (LOS; mean difference -1.55 days, 95% CI -2.73 to -0.36, P = 0.01), intensive care LOS (mean difference -0.63 days, 95% CI -1.18 to -0.09, P = 0.02), and time to passage of feces (mean difference -0.90 days, 95% CI -1.48 to -0.32 days, P = 0.002). However, no difference was seen in mortality, return of flatus, or risk of paralytic ileus. If patients were managed in an ERAS pathway, the only significant reductions were in intensive care LOS (mean difference -0.63 days, 95% CI -0.94 to -0.32, P < 0.0001) and time to passage of feces (mean difference -1.09 days, 95% CI -2.03 to -0.15, P = 0.02). If managed in a traditional care setting, a significant reduction was seen in both overall morbidity (RR 0.69, 95% CI 0.57 to -0.84, P = 0.0002) and total hospital LOS (mean difference -2.14, 95% CI -4.15 to -0.13, P = 0.04). CONCLUSIONS: GDFT may not be of benefit to all elective patients undergoing major abdominal surgery, particularly those managed in an ERAS setting.

Mixed periampullary adenocarcinoma and somatostatinoma with small bowel gastrointestinal stromal tumour in neurofibromatosis type 1.

CONTEXT: Gastrointestinal (GI) involvement is present in about one quarter of cases of neurofibromatosis type 1 (NF1). Adenocarcinomas have been reported in several organs. Gastrointestinal stromal tumors are the most common GI lesion seen in NFI. GISTs in combination with ampullary neuroendocrine tumors in NF-1 have been reported rarely. CASE REPORT: We present the case of a 44-year-old man who presented with a history of obstructive jaundice and weight loss. Investigations revealed a pancreatic tumor associated with a common bile duct (CBD) stricture. At operation, an ampullary adenocarcinoma that infiltrated into the head of pancreas with an adjacent somatostatinoma was found. In addition, a small bowel GIST was present. CONCLUSIONS: Mixed periampullary adenocarcinoma and somatostatinoma in a patient with NF1 has only been previously reported once. The current case highlights the spectrum of associated tumor types which can be seen in association with NF1. Patients with NF1 who present with jaundice and weight loss should be investigated in the usual manner with increased suspicion for duodenal and ampullary tumors.