D Westaby

Variceal hemorrhage and cystic fibrosis: outcomes and implications for liver transplantation.

Autopsy and imaging studies show that liver involvement is common in cystic fibrosis. However, complications of chronic liver disease including portal hypertension and variceal bleeding are infrequently encountered, and the degree to which variceal hemorrhage affects prognosis in cystic fibrosis is unclear. This uncertainty has lead to debate as to whether liver transplantation is indicated in these patients. We describe a case series of 18 patients and compare their survival with a control group of cystic fibrosis patients without liver disease. The median age at first bleed was 20.0 years (range 9.7‐30.9). The median survival after first bleed was 8.4 years, compared to 13.0 years in the control group (P = 0.15). A total of 14 patients have died, 9 from respiratory disease with no discernable contribution from their liver disease. Liver disease contributed to 4 deaths. Only 1 patient suffered a fatal hemorrhage, which may have been either variceal or bronchial in origin. Long‐term survival is a frequent occurrence in patients with cystic fibrosis who suffer variceal hemorrhage, and age at death is comparable to the general cystic fibrosis population. In conclusion, this suggests that liver transplantation is not indicated in these patients without additional features of liver decompensation. (Liver Transpl 2005.)

Emergency colonoscopy for distal intestinal obstruction syndrome in cystic fibrosis patients

Recent advances in the treatment of cardiorespiratory complications in patients with cystic fibrosis (CF) have improved median survival and resulted in gastrointestinal complications becoming more evident.1,2 The distal intestinal obstruction syndrome (DIOS) develops due to the accumulation of viscid muco-faeculent material in the terminal ileum and right hemicolon.3 Reduced intestinal water content, lower luminal acidity of the foregut, and accumulation of intraluminal macromolecules contribute to the development of DIOS.4 Typically, patients develop progressive symptoms and signs of small bowel obstruction. A plain abdominal x ray will reveal dilated loops of centrally placed small bowel, with faecal loading at the terminal ileum and right hemicolon (see fig 1). Most cases will respond to the aggressive use of laxative agents administered orally and rectally, intravenous hydration, pancreatic supplements, and N -acetylcysteine (Parvolex) and gastrografin (meglumine diatrizoate) orally or per rectum.5–8 Surgical decompression has been reserved for refractory cases but carries a …

The safety and utility of prophylactic pancreatic duct stents in the prevention of post-ERCP pancreatitis: an analysis of practice in a single UK tertiary referral center

BackgroundThe use of temporary prophylactic pancreatic duct (PD) stents in the prevention of post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis in high-risk patients has been shown to be effective in multiple trials. However, there are limited data on the clinical implications of PD stents and their impact on practice outside of the trial setting.MethodsThe utility of prophylactic pancreatic stenting was evaluated in a retrospective analysis of 1,000 consecutive ERCPs performed in a single tertiary referral pancreatobiliary center over a 24-month period, based upon a predetermined protocol to identify patients at high risk of postprocedure pancreatitis.ResultsOne thousand procedures performed in 688 patients were studied. Sixty-one patients were considered for stent placement and stents were successfully placed in 58 cases. The overall rate of post-ERCP pancreatitis in our study population was 3.6%. The rate of pancreatitis in the stented patients was considered high at 22.4%, but the majority (69%) were classified as mild and there were no reported severe episodes. This compares to pancreatitis in the nonstented group, in whom the majority (73.9%) experienced either moderate or severe episodes.ConclusionA strategy of prophylactic PD stents in this study has eliminated severe post-ERCP pancreatitis in high-risk patients. However, the high pancreatitis rate in stented patients may represent the cost to achieve this, while stent type and size employed are likely contributing factors. To maximize the benefits of PD stenting, there is a need to identify and treat all those considered at high risk.

Short and long-term post-operative outcomes of duodenum preserving pancreatic head resection for chronic pancreatitis affecting the head of pancreas: a systematic review and meta-analysis.

BACKGROUNDnTo evaluate the short and long term outcomes of duodenum preserving pancreatic head resection (DPPHR) procedures in the treatment of painful chronic pancreatitis.nnnMETHODSnA systematic literature search was performed to identify all comparative studies evaluating long and short term postoperative outcomes (pain relief, morbidity and mortality, pancreatic exocrine and endocrine function).nnnRESULTSnFive published studies fulfilled the inclusion criteria including 1 randomized controlled trial comparing the Beger and Frey procedure. In total, 323 patients underwent surgical procedures for chronic pancreatitis, including Beger (n = 138) and Frey (n = 99), minimal Frey (n = 32), modified Frey (n = 25) and Bernes modification (n = 29). Two studies comparing the Beger and Frey procedure were entered into a meta-analysis and showed no difference in post-operative pain (RD = -0.06; CI -0.21 to 0.09), mortality (RD = 0.01; CI -0.03 to 0.05), morbidity (RD = 0.12; CI -0.00 to 0.24), exocrine insufficiency (RD = 0.04; CI -0.10 to 0.18) and endocrine insufficiency (RD = -0.14 CI -0.28 to 0.01).nnnCONCLUSIONnAll procedures are equally effective for the management of pain for chronic pancreatitis. The choice of procedure should be determined by other factors including the presence of secondary complications of pancreatitis and intra-operative findings. Registration number CRD42015019275. Centre for Reviews and Dissemination, University of York, 2009.

Symptomatic Pancreatitis in Patients With Cystic Fibrosis

OBJECTIVES:A small group of cystic fibrosis (CF) patients develop symptomatic pancreatitis. The clinical characteristics of these cases are not well documented in the literature. Most cases are in pancreatic sufficient (PS) patients, but it is not clear whether pancreatitis does occur in pancreatic insufficient (PI) patients. There is no information on how the group with PS and pancreatitis differs from the group with PS that does not develop pancreatitis.METHODS:The Royal Brompton Hospital database of adult CF patients was searched to identify all patients with symptomatic pancreatitis. Clinical details were taken from the case notes. PS pancreatitis patients were then compared with an age- and sex-matched PS control group drawn from the database.RESULTS:Sixteen patients (9 males) had suffered symptomatic pancreatitis, representing 1.6% of the total database. The mean age at CF diagnosis was 18.7 years, and at presentation with pancreatitis it was 28.8 years. Twelve were PS at diagnosis of CF. At presentation with pancreatitis, seven patients were PS and at the most recent follow-up or death, two remained PS. There was a median of three hospital admissions with pancreatitis. Eight cases developed pancreatic or hepatobiliary complications. In the comparison of pancreatitis patients with controls, there was no difference in survival but pancreatitis patients were significantly more likely to develop PI status. Mild CF transmembrane conductance regulator mutations in general, and R117H in particular, were found more often in pancreatitis patients.CONCLUSIONS:Symptomatic pancreatitis is a significant problem in 1–2% of patients with CF. These patients are PS at birth but are more likely to develop late PI status than PS patients without pancreatitis. R117H may be associated with this phenotype.

Surgery remains the best option for the management of pain in patients with chronic pancreatitis: A systematic review and meta-analysis

Controversy related to endoscopic or surgical management of pain in patients with chronic pancreatitis remains. Despite improvement in endoscopic treatments, surgery remains the best option for pain management in these patients.

OC-079 Evaluation of Tertiary Centre Management of Type 2 Sphincter of Oddi Dysfunction Supports Manometry Defined Endoscopic Intervention

Introduction There is still doubt about the role of manometry and endoscopic intervention in Type 2 sphincter of Oddi dysfunction (T2SOD). We aimed to examine the efficacy of a manometry guided approach in the evolving management of T2SOD in our tertiary clinical practice at Hammersmith Hospital, London, UK. Methods We retrospectively evaluated all T2SOD patients referred between 2010 and 2014. Baseline characteristics and procedural outcomes were extracted including manometry readings, type of endoscopic intervention (sphincterotomy or Botulinum toxin injection), complications and pain improvement at 3 and 12 months. Results 74 T2SOD patients were identified, 17 of whom were excluded due to prior sphincterotomy or follow up elsewhere. Botulinum toxin injection was performed in 11 patients with normal manometry; 27% of whom reported short term but unsustained benefit. 46 patients were managed with dual sphincterotomy. Sustained benefit at 12 months was seen significant more often in those with abnormal (72%) than normal (21%) sphincter pressure (p = 0.046, OR = 4.6, CI: 1.2–17.5). Complications occurred in 19.2% (11/57) of patient’s post-sphincterotomy, but interestingly were confined only to those with abnormal manometry. Initial pain relief after cholecystectomy (p = 0.037, OR = 11.7, CI = 1.227–110.953) predicted better outcome while those with prior hysterectomy (p = 0.039, OR = 0.039, CI = 0.006-0.849) had worse outcome. Daily opiate users were more likely to suffer complications (p = 0.072, OR = 6.333, CI = 1.114–35.997). Finally, biliary and pancreatic sphincter pressures correlated highly (R = 0.586, p < 0.001). Conclusion We found abnormal manometry predicted both sustained pain improvement post-sphincterotomy and complications. The increased complication risk seems attributable to the underlying disease highlighting the safety of manometry itself. The correlation between biliary and pancreatic sphincter pressure suggests measurement of both may not be necessary before dual sphincterotomy after confirmation of sphincter hypertension. Our study of all-comers advocates a strategy of manometry-defined endoscopic intervention in T2SOD. Disclosure of Interest None Declared

OC-074 Novel Point of Care Test for Detection of Human Phospholipase A2 to Predict Acute Pancreatitis Post Endoscopic Retrograde Cholangiopancreatography (ERCP) and Aid Same Day Discharge

Introduction ERCP is a therapeutic procedure which carries a 5% risk of pancreatitis. Most patients who have undergone ERCP are admitted overnight for observation as it can take up to 12 hours for post-ERCP pancreatitis to manifest. Phospholipase A2 Group IB (PLA2GIB) is a sensitive marker of pancreatitis. A rapid, quantitative, point-of-care (POC) test for PLA2GIB has been developed.1 We aimed to apply this test in a clinical setting, and determine its ability to detect clinically significant episodes of post-ERCP pancreatitis. Methods Ethical approval for the study and informed patient consent were obtained. Patients undergoing ERCP were randomly selected within a tertiary referral HPB centre. Blood amylase levels were obtained before and 3u2009hrs after ERCP. At the same times, 20µL of blood was taken, added to a running buffer and applied to the POC kit. Movement of the diluent was observed along the lateral flow strip. The lateral flow strip was then read by a micro device, calculating the PLA2GIB concentration from a standard curve. Cases of post ERCP pancreatitis were idenitified on the basis of characteristic pain, examination findings and elevation in serum amylase. Results 46 patients undergoing ERCP were recruited (26u2009M). Indication for ERCP included biliary stones (21), biliary strictures (12), biliary stent removal (6), pancreatic duct intervention (2) and other (5). The median pre-ERCP amylase level was 52u2009IU/L (range 19–316). The median post ERCP amylase was 78u2009IU/L (32–1720). Median pre-ERCP PLA2GIB concentration was 4u2009ng/mL (0.5–45). Median post-ERCP PLA2GIB was 6u2009ng/mL (0.6–418). There was a median 0.3 fold (-0.7-29) increase in PLA2GIB concentration after ERCP. 3 patients developed post-ERCP pancreatitis. These patients all had corresponding elevations in PLA2GIB above 70u2009ng/mL. A further 3 patients had elevation of amylase >3 x the upper limit of normal but without clinical symptoms of pancreatitis and with a PLA2GIB level <70u2009ng/mL. With a cut off level of 70u2009ng/mL, PLA2GIB had a sensitivity of 100% and specificity of 98%. The PPV was 75% and NPV 100%. Conclusion This is the first clinical trial of a POC test for PLA2GIB activity. The test appears to be highly sensitive for post-ERCP pancreatitis. In our cohort of 46 patients undergoing ERCP, a negative test at 3 hours would have reliably excluded all cases of post-ERCP pancreatitis. This low cost, easy to use POC test could be used to support same day discharge of patients undergoing ERCP. Reference 1 Chapman R, et al. Multivalent nanoparticle networks enable point-of-care detection of human phospholipase-A2 in serum. ACS Nano 2015;2565–2573. Disclosure of Interest None Declared

PWE-031 Modelling of suspicious and high risk endosonographic morphology, cytopathology and cyst biochemistry highlights the accuracy of endosonography to predict operative histological outcome in pancreatic cystic tumours

Introduction Distinguishing benign from potentially malignant pancreatic cystic lesions has important prognostic and therapeutic implications. Diagnosis is achieved through a combination of history, imaging, biochemical, endoultrasonographic (EUS) and cyst aspirate (CA) analysis. We evaluated the accuracy of suspicious and high risk variables identified at EUS and CA analysis to predict premalignant or malignant histopathology confirmed at surgery. Method Patients who underwent EUS prior to surgery were selected. Data on age, sex, cyst location, suspicious (S) or high risk (HR) EUS or CA features, diagnosis (grouped as benign vs premalignant/malignant) at EUS (EUS-D), post CA analysis (CA-D) and combined EUS and CA analysis (EUS&CA-D) were collected. These were compared with final histological diagnosis (H-D) from surgery. High-risk (HR) variables were defined as C4/C5 diagnosis or carcinoma embryonic antigen (CEA) >192 ng/ml on CA, and cyst size >30 mm, PD dilatation >10 mm, mural nodules or mixed solid/cystic components at EUS Suspicious (S) variables included HR features or mucin, C3 diagnosis or amylase >1000 U/L on CA, and pancreatic duct (PD) dilatation or cyst wall thickening on EUS. Variables were assigned a score of 1 when present, and combined with either EUS-D (EUS-D-S or EUS-D-HR) or CA-D (CA-DS or CS-D-HR). A single positive variable defined positivity in groups. EUS and CA outcomes were then evaluated to identify the sensitivity, specificity, area under the ROC curve (AUC) and likelihood ratio (LR) to predict H-D. Results 38 patients were identified (mean age 60.4 years, range 19–81; 52.6% female). Histology identified benign (18.5%; retention cysts, accessory spleen, chronic pancreatitis, hydatid, serous cystic neoplasm), premalignant (60.5%; intraductal papillary mucinous neoplasm (IPMN), mucinous cystadenoma, solid pseudopapillary tumour) or malignant disease (21.1%; adenocarcinoma, adenosquamous carcinoma, neuroendocrine tumour). Analysis (AUC and LR) ranked by AUC confirmed EUS-D-S significantly predicted H-D (0.81, p = 0.022; 5.87) with a sensitivity and specificity of 83.7% and 85.7% respectively. This was followed by EUS-D-HR (0.68; 4.29; p = 0.187), CS-D-HR (0.64; 1.83; p = 0.306), EUS&CA-D-HR (0.62; 1.81; p = 0.374) and CS-D-S (0.52; 1.04; p = 0.89). EUS&CA-D-S did not predict H-D (0.5; 1.16; p = 1.0). Conclusion EUS can accurately predict final H-D in patients cystic pancreatic lesions with excellent sensitivity and specificity when modelled appropriately. Interestingly, the accuracy of this method diminished when combined with CA variables. Disclosure of interest None Declared.

PWE-212 Genotype-phenotype correlations in cystic fibrosis patients with pancreatitis highlights an increased likelihood of pancreatic insufficiency and identifies unique mutation pairs that potentially predispose to and protect from pancreatitis

Introduction 1–2% of cystic fibrosis (CF) patients develop symptomatic pancreatitis. Studies have shown these patients present later in life, are pancreatic sufficient (PS) and have a less severe CF phenotype. This study aimed to evaluate the genotype:phenotype relationship of CF patients with pancreatitis in a tertiary referral centre for CF. Method CF patients with a history of pancreatitis were identified from 1216 patients in The Royal Brompton Hospital CF database. Each case was age and sex matched with controls. Clinical information on age, sex, PS status, age when CF diagnosed, CF genotype and mortality were collected. To compare pancreatitis patients with controls, a two tailed t-test (age of CF diagnosis, R117H mutation rate) and Kaplan-Meir survival curves (rate of PI and mortality rates) were utilised. Results 37 patients (51% females) with pancreatitis were matched to 135 controls (3:1/4:1). Mean age of CF diagnosis was significantly higher in the pancreatitis group (19yrs) than controls (10.6yrs; p = 0.046). Median time between CF diagnosis and pancreatitis was 17 yrs (range 8–36 yrs). 65% (n = 24) of pancreatitis sufferers were PS at presentation to adult services; of these 63% (n = 15) remained PS until their most recent review or death. Furthermore, 67% (n = 16) suffered with recurrent or chronic pancreatitis. Using Log-rank (Mantal-Cox), there was no difference in survival between pancreatitis patients and controls (p = 0.97). However, pancreatitis patients were significantly more likely to develop PI (p = 0.012). The ^F508:R117H mutation pair was noted more frequently in pancreatitis patients (18.9%;n = 7) than controls (3.8%;n = 5) but not significancantly so (p = 0.56). Interestingly in 43% (n = 16) of the remaining pancreatitis patients, 15 mutation pairs unique to this group were identified. Similarly in controls, 50% (n = 65) had mutation pairs unique to this group.Abstract PWE-212 Figure 1 Rate of pancreatic insufficiency in CF pancreatitis patients vs CF controls Conclusion CF patients who suffer with pancreatitis are significantly more likely to develop PI than those who do not. They present with CF at an older age but this does not affect their survival. The R117H mutation is seen more frequently in the pancreatitis group but not significantly so for this phenotype. We have identified a number of mutation pairs that seem to either predispose or protect CF patients from pancreatitis. Disclosure of interest None Declared.