Introduction

Early recognition and risk-targeted surveillance of patients with hereditary colorectal cancer (HCRC) and familial CRC (FCRC) reduce CRC incidence, improving in turn the effectiveness of population-based organised screening programmes. However, HCRC/FCRC often remain under-recognized and suboptimally managed and opportunities for CRC prevention are often missed. In 2020, pilot screening programmes for CRC have been started in Romania. In this context, a screening initiative for high-risk groups was started at national level, as an adjunct to population-based screening. Here, we present our experience in setting up an interdisciplinary team as a first step in implementing a screening programme for HCRC/ FCRC with coverage across the country.

Aims & Methods

We used a stepwise approach: first, an evidence-based educational intervention was provided in order to create a national network of clinical specialists; next, implementation of this knowledge in clinical practice and setting-up of a HCRC registry. Between Nov 2022-Jan 2023, we trained clinicians from university hospitals and cancer centers covering the main administrative regions of the country, using an evidence-based online curriculum (weekly, 12 weeks, in total +/-22 hours) for achieving mastery standards (excellence for all) on the recognition and management of HCRC/FCRC. The curriculum was created by an international panel of GI experts and education experts. The training consisted of pre-class preparation (online study of clinical guidelines, instructional videos), virtual classroom (live active learning session with case-based quizzes and targeted feedback) and post-class tutoring sessions (case-based quizzes with instructor/ peer feedback). Pretest and posttest(s) scores were measured. The main outcomes were the proportion of participants reaching the minimum passing standard (MPS) and the learning effect size. Wilcoxon signed-rank test was used to compare the differences between test scores and Cohen’s d coefficients for effect size. Secondary outcome was the course satisfaction.

Results

We enrolled 20 gastroenterologists and 3 non-gastroenterologists (median 37.8 (28-58) years, 61% female, from academic (82.6%) and cancer (17.4%) centers. None received previous training in HCRC/FCRC. At baseline, none of the participants reached the MPS. At the final post-test, all participants reached/ exceeded the MPS. Median test scores significantly increased from pretest to initial posttest and final posttest: 55 (45-62)% vs 84 (82-86)% vs 91 (87-97)%, (P<0.0001). The educational sessions produced a very large learning effect size compared to pretest scores (Cohen’s d=3.91, 95%CI=2.33-5.50). All participants expressed very high satisfaction about course participation. Immediately after the course, they implemented the acquired knowledge into practice, set up local interdisciplinary teams and continued training of their peers/ students. A 2-step approach to genetic testing was adopted in clinical practice: (universal) IHC/ MSI and in case of a dMMR tumor, targeted germline testing.

Conclusion

We launched a national screening initiative for HCRC/ FCRC as an adjunct to population-based CRC screening. Evidence-based education to achieve mastery standards on HCRC recognition was the first critical step in creating an expert working group to coordinate the performance of clinicogenetic diagnosis and targeted risk-reducing interventions. Efforts are currently underway for achieving full rollout of the training program across the country and ultimately set-up a hereditary cancer registry.

Clinical Case Summary

Patient of 71 years old with CP was admitted for painless jaundice with benign common bile duct (CBD) stricture. ERCP failed at her center due to duodenal edema that prevented seeing the papilla so the patient was transferred to our center for assisted EUS drainage.

After after a first attempt with an unsuccessful rendezvous ERCP due to guidewire loss in the exchange of devices, cholecystoduodenostomy was performed with a coaxial double pigtail after confirmation of cystic duct permeability in the cholangiogram (previously performed under EUS guidance with puncture of the CBD to perform de rendezvous). In a second attempt to treat the stricture, a rendezvous through the gallbladder was performed. After papilloplasty and dilating the stricture, a 10 x 60 mm FCMS was placed. The patient improved and the lumen apposing metal stent of the cholecystoduodenostomy was removed. In followup, proximal migration of the stent was observed, impossible to remove by ERCP at her center due to impossibility of accessing the papilla.

In our center, a new endoscopy was performed. CBD was punctured by EUS with a 22 G needle through the biliary stent to perform a rendezvous and a novagold guidewire was advanced to the duodenum. The CBD was accessed with a monorail sphincterotome over the guidewire and rigid guidewire was advanced to the biliary tract. Papilloplasty was performed againd and the migrated stent was removed with rat tooth forceps. A new FCMS was placed successfully.

To conclude, endoscopic management of patients with CP can be very complex and several advanced combined bile duct access techniques are sometimes required. So, these patients should ideally be managed in referral centers.

Introduction

EUS-guided drainage using lumen-apposing metal stents (LAMS) has become standard treatment of symptomatic walled-off necrosis (WON). The development of these fully covered self-expanding metal stents with special design of the flanges and large diameter, improved the drainage of debris and facilitated further direct endoscopic necrosectomy. Delivery systems with integrated electro-cautery (EC) enable direct access and stent deployment whilst the conventional stent insertion technique requires several steps including access using a needle or cystotome, wire insertion and enlargement of the created tract before the stent placement.

Aims & Methods

In this single center study, we aimed to compare the practicality of EUS-guided procedures and their outcome between conventional LAMS (cLAMS) and those with integrated EC (LAMS-EC).
From our prospectively maintained database, we included consecutive patients undergoing EUS-guided LAMS insertion with or without integrated electro-cautery for drainage of walled-off necrosis. Infection of the walled-off pancreatic necrosis, biliary compression with cholestasis or gastric outlet obstruction were accepted indications for intervention. Data on procedure time, sedation, hospital stay, technical and clinical success as well as adverse events were analysed.

Results

We analysed 107 consecutive procedures of transmural EUS-stenting for drainage of walled-off necrosis. 39 cLAMS and 68 LAMS-EC were placed successfully. Mean size of the walled-off necrosis was 12.9 ± 4.2 cm with mean 30% ± 15% solid necrosis.
Procedure times were shorter with LAMS-EC (18 .0 ± 6.6 min vs. 39.7 ± 8.3 min; p<0.05). All EUS-guided procedures with cLAMS drainage were performed under propofol sedation administered by an anaesthetic team. In the LAMS-EC group, 36 patients tolerated the procedure under iv sedation (fentanyl/midazolam) in left lateral position and 17 interventions were performed as daycase procedures. None of the patients developed sedation related adverse events such as aspiration.
Adverse events, technical success and clinical outcome did not differ between both groups.

Conclusion

The findings of this study suggest that cLAMS and EC-LAMS achieve similar rates of technical and clinical success without difference in the rate of adverse events. Compared to cLAMS, deplayment of EC-LAMS is faster and technically less demanding. This might allow to perform the procedure under conventional intravenous sedation and as daycase procedure in carefully selected patients.

Clinical Case Summary

A 90-year-old woman was referred to our endoscopy unit from another hospital for urgent rescue therapy because of impacted Dormia basket during ERCP performed for symptomatic recurrent choledochal lithiasis. In attempt to perform extracanalar lithotripsy Dormia basket was broken near the handle. Another ERCP was performed, and the contrast cholangiography showed dilated common bile duct (up to 13mm) with a constriction in the middle part and impacted Dormia basket with multiple gallstones, confirmed by Spyglass single-operator per-oral cholangioscopy with direct visualization. Electrohydraulics lithotripsy with Autolith EHL system (2437 pulses, medium-high power) was performed with stones fragmentation but Dormia basket entrapped stone fragments. Dormia basket distal margin was caught with a biopsy forceps and passed in duodenoscope working channel. Then biliary clearance was completed with ballon catheter and the following cholangiography confirmed successful biliary drainage and optimal bile outflow without contrast biliary or duodenal spreading. Postprocedural course was regular, and the patient was discharged after three days of hospitalization.

Clinical Case Summary

Introduction: Superior mesenteric artery pseudoaneurysms (SMA) are rare vascular entities, but can potentially result in significant morbidity and mortality (up to 60%) if rupture occurs. Traditionally, SMA pseudoaneurysms are associated with arterial dissection, pancreatitis, infection, trauma, tumor invasion, or arise as post-operative and iatrogenic complication.^1-8
Case report: A 51-year-old man with a medical history of chronic alcohol consumption and chronic pancreatitis presented with multiple episodes of hematemesis and hematochezia, jaundice and severe abdominal pain with a supraumbilical pulsatile mass. The patient was haemodynamically unstable (BP: 79/45 mmHg, HR: 123/min) with inadequate resuscitation despite transfusions of blood products and intravenous fluids. Laboratory tests showed significant reduction in hematocrit (Hct: 23,9%, Hb: 7,7 g/dL) and abnormal liver function tests (TBL: 7,21 mg/dL, DBL: 4,88 mg/dL, SGOT: 156 U/l, SGPT: 92 U/l, γGT: 1822 U/l, ALP: 1577 U/l). An abdominal vascular lesion was suspected and an urgent CT angiography was performed. The CT scan confirmed the presence of a ruptured superior mesenteric artery pseudoaneurysm, 11.5cm in diameter, localized inside a giant pancreatic pseudocyst (Figure A). Ultimately, as the patient was a poor surgical candidate we decided to treat him using endovascular therapy. Arterial catheterization and angiographic evaluation of the superior mesenteric artery pseudoaneurysm was performed, showing active contrast agent extravasation treated by a fully covered stent placement (Figure B). The final angiogram confirmed the the success of the intervention. The postoperative course was uneventful and the patient was discharged on the 8^th postoperative day.
Conclusions: In conclusion, physicians should keep alert to this rare etiology of haemobilia. It is of utmost importance to urgently manage SMA pseudoaneurysms because of the high risk of rupture and potential life-threatening haemorrhage.

References

1. Guirgis M, Xu JH, Kaard A, Mwipatayi BP. Spontaneous Superior Mesenteric Artery Branch Pseudoaneurysm: A Rare Case Report. EJVES Short Rep. 2017 Oct 12;37:1-4.
2. Patelis N, Doukas P, Dodos I, Karampelas T, Kanellopoulos I, Kyriakopoulou K, Bisdas T. Endovascular Repair of a Complex Isolated Dissecting Aneurysm of the Superior Mesenteric Artery. EJVES Short Rep. 2019 Jun 17;44:5-8.
3. Kochar S, Sharparis Y, Banerjee B, Karasek M, Brennan S. Endovascular Coil Embolization of Superior Mesenteric Artery Branch Pseudoaneurysm. Cureus. 2021 Sep 16;13(9):e18014.
4. Camarasa Pérez Á, Soto Sánchez A, Pérez Sánchez E, Hernández Barroso M, Hernández Hernández G, Barrera Gómez M. Obstructive jaundice secondary to a pseudoaneurysm of the superior mesenteric artery. Rev Esp Enferm Dig. 2022 Dec;114(12):756.
5. Kimbara Y, Sato Y, Hasegawa T, Morinaga H, Murata S, Imai Y, Hara K, Okuno N, Inaba Y. Covered stent placement for emergency repair of a ruptured superior mesenteric artery pseudoaneurysm in advanced pancreatic head cancer. Radiol Case Rep. 2018 Oct 29;14(1):133-135.
6. Dávila Arias C, Ruiz-Rodríguez AJ, Cabrera Peña Á. Hematochezia secondary to hemosuccus pancreaticus due to a pseudoaneurysm associated with a pancreatic pseudocyst. Rev Esp Enferm Dig. 2021 Aug;113(8):618.

Clinical Case Summary

Introduction: Pancreaticopleural fistula (PPF) is an abnormal communication between the pancreas and the pleural cavity that represents a rare complication of pancreatitis.¹ Pleural effusions can result that tend to be massive and recurrent, in the context of enduring pancreatic fluid production. As presenting symptoms and investigation are pulmonary-focused, diagnosis is often delayed, ranging from 12 to 49 days with there being no consensus on optimal management.²
Case details: A 62-year-old male presented with worsening abdominal pain, cough, dyspnea and absent breath sounds over the right lung field on examination following a recent episode of acute-on-chronic pancreatitis due to excessive alcohol consumption. Initial work-up showed neutrophilia, transaminitis, and an elevated serum lipase level of 1,720 U/L. Initial chest X-ray and computed tomography (CT) showed a massive right pleural effusion with underlying lung collapse and mediastinal shift. Diagnosis of PPF was confirmed after intercostal catheter drainage for symptomatic relief, with pleural fluid analysis showing an exudative process with elevated amylase and lipase content along with magnetic resonance imaging visualising a fistulous connection between the pleura and pancreas. Endoscopic intervention was considered but not undertaken in the absence of pancreatic ductal obstruction and consideration of procedural/case-specific risks. A solely conservatively approach of intercostal catheter drainage of reaccumulating pleural fluid was successful with serial follow-up confirming complete resolution of the pleural effusion and fistulous tract.
Conclusion: This case exemplifies the success of conservative management of PPF where an advanced endoscopic or surgical intervention is not always required, as well as the importance of tailoring management to the individual in conjunction with a multidisciplinary team.

References

1. Rockey DC, Cello JP. Pancreaticopleural fistula report of 7 patients and review of the literature. Medicine; 69: 332–344.
2. Dhebri AR, Ferran N. Nonsurgical management of pancreaticopleural fistula. Journal of the pancreas; 6(2):152-61.

Images to be included:
Figure 1: Chest radiography showing a massive right pleural effusion.
Figure 2: Magnetic resonance imaging (coronal view) showing a retroperitoneal collection.
Figure 3: Magnetic resonance imaging (axial view) showing a communication between the peripancreatic retroperitoneal fluid collection and the right pleural cavity.

Clinical Case Summary

We present the case of a 51-year-old gentleman with von Hippel-Lindau disease and a complex surgical history involving bilateral adrenalectomy in childhood and a Whipple’s procedure for neuroendocrine tumor (NET) at 36 years of age. Surveillance imaging demonstrated recurrent paraganglioma adjacent to the left aspect of the coeliac axis and a 2 cm NET in the pancreatic tail. The paraganglioma was resected, but attempted resection of the tail was complicated by haemorrhage and had to be abandoned. He was readmitted two weeks later with abdominal pain. CT demonstrated a post operative pancreatic fluid collection which was initially managed by percutaneous drainage. This reduced the collection volume, but resulted in a chronic pancreatic fistula with daily output of 100-150 ml.
Initial attempt to drain this cavity by EUS cyst-gastrostomy proved unsuccessful due to an incomplete cavity wall precluding expansion of the cavity with saline injection. CT with contrast injection through the external drain delineated communication with the dilated main pancreatic duct (PD) and extension of the collection between the liver and gastro-jejunal anastomosis.
Previous Whipple’s procedure made ERCP challenging therefore EUS directed pancreaticogastrostomy was planned.. EUS demonstrated a dilated PD containing significant debris and calculi, particularly centrally towards the pancreatico-jejunal anastomosis. Due to the PD calculi burden it was not possible to insert sufficient wire to facilitate plastic stent insertion. Therefore an 8 mm X 8 mm lumen apposing metal stent (LAMS) was deployed from the stomach into the PD. Injection of contrast through the LAMS opacified the pancreatic duct confirming successful procedure.
The percutaneous drain output reduced to zero within two days and the patient clinically improved. The drain was removed at 4 weeks. Subsequent imaging demonstrated resolution of the collection.

References

1. Preprocedural CT demonstrating communication between the collection and the pancreatic duct.
2. EUS demonstrated dilated PD containing debris and calculi.
3. Fluoroscopic image confirming technically successful pancreaticogastrostomy
4. Follow up CT confirming resolution of percutaneous fistula and associated collection