Clinical Case Summary

Intraductal papillary mucinous neoplasms (IPMN) are premalignant or malignant tumors of the pancreas that may progress to invasive carcinoma. The management of IPMNs is still challenging and oriented on the accurate assessment of the risk of malignant transformation [1-3]. Because definitive diagnosis of Main Duct IPMN could be challenging in advanced chronic pancreatitis cases, we scheduled a peroral pancreatoscopy (POPS). Pancreatoscopy allowed direct high-definition visualization of the pancreatic ducts and targeted biopsy on abnormal-appearing mucosal lesions [4,6]. Herein, we present a case of a 68-years old male patient with EUS evidence of chronic pancreatitis and main duct dilation, a single enhancing mural nodule of 6 mm and no evidence of malignancy after FNB. After multidisciplinary discussion, we performed a pancreatoscopy to better evaluate the extension of the suspected Main Duct-IPMN. POPS showed MPD segments with mucus, papillary fronds, villous projections with a “fish-egg” like, and MPD segments with vegetative-villous protrusions and abnormal vascular images appearance, staged accordingly to the visual Hara classification [5]. Histological examination found intestinal and gastric type IPMN with coexistence of both low-grade and high grade dysplasia with patch distribution. The patient was then referred to surgical treatment with total pancreasectomy, with no recurrence of disease after one year follow-up. Preoperative POPS showed to be reliable for identifying IPMN and may provide useful information for the surgeon when there is high suspicion of MD-IPMN, and previous techniques such as EUS-FNA and FNB are not conclusive [7,8].

References

1. Tanaka M, et al. Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas. Pancreatology. 2017 Sep-Oct;17(5):738-753.
2. Ohtsuka T, et al. International evidence-based Kyoto guidelines for the management of intraductal papillary mucinous neoplasm of the pancreas. Pancreatology. 2024 Mar;24(2):255-270.
3. European Study Group on Cystic Tumours of the Pancreas. European evidence-based guidelines on pancreatic cystic neoplasms. Gut. 2018 May;67(5):789-804.
4. Nagayoshi Y, et al. Peroral pancreatoscopy using the SpyGlass system for the assessment of intraductal papillary mucinous neoplasm of the pancreas. J Hepatobiliary Pancreat Sci. 2014 Jun;21(6):410-7
5. Hara T, et al. Diagnosis and patient management of intraductal papillary-mucinous tumor of the pancreas by using peroral pancreatoscopy and intraductal ultrasonography. Gastroenterology. 2002 Jan;122(1):34-43.
6. Arnelo U, et al. Single-operator pancreatoscopy is helpful in the evaluation of suspected intraductal papillary mucinous neoplasms (IPMN). Pancreatology. 2014 Nov-Dec;14(6):510-4.
7. De Jong DM, et al. European Cholangioscopy study group. The role of pancreatoscopy in the diagnostic work-up of intraductal papillary mucinous neoplasms: a systematic review and meta-analysis. Endoscopy. 2023 Jan;55(1):25-35.
8. Parbhu SK, et al. Efficacy, Safety, and Outcomes of Endoscopic Retrograde Cholangiopancreatography With Per-Oral Pancreatoscopy: A Multicenter Experience. J Clin Gastroenterol. 2017 Nov/Dec;51(10):e101-e105.

Disclosure

Claudio Zulli is lecturer for Pentax and Boston Scientific.
Mariano Sica is lecturer for Pentax.
Attilio Maurano is lecturer for Boston Scientific.

Clinical Case Summary

Introduction: Locally advanced pancreatic tumors can lead to both biliary and duodenal obstruction by invasion or compression. Quality of life is severely affected if obstruction occurs: jaundice, emesis and pain can become invalidating and patients become too frail for palliative chemotherapy and are sometimes declared unfit even for derivative surgery. Recently, endoscopic techniques under EUS or ERCP guidance are being used to create biliodigestive and enteral anastomoses.
Clinical case: A 75-year-old female was diagnosed with locally advanced pancreatic head adenocarcinoma in January 2024. The tumor was declared unresectable and chemotherapy was initiated. A plastic stent was inserted into the main biliary duct (MBD) to ensure biliary drainage. In May 2024, she presented with cholangitis and emesis. EGD revealed duodenal stenosis and ERCP failed due to tumor infiltration. A combined endoscopic approach was performed: EUS-guided hepatico-gastrostomy (EHG) for biliary drainage and EUS-guided gastro-enterostomy using a lumen-apposing metal stent (LAMS). In July 2024, the plastic stent was removed, and in August 2024, two fully covered 60 x 10 mm SEMS were inserted antegrade through the EHG into the MBD to improve patency. At 11 months post-intervention, the patient returned for routine reassessment, declaring good clinical performance: she remained asymptomatic, with routine blood tests, including bilirubin levels within normal range and with all stents patent and correctly positioned (confirmed radiologically and endoscopically). The patient was able to maintain oral intake and continue chemotherapy. No late adverse events were noted.
Conclusion: This case demonstrates the long-term clinical and technical success of a staged endoscopic triple bypass for the management of complex biliary and duodenal obstruction due to unresectable pancreatic cancer. The approach was minimally invasive, well tolerated and remarkably improved the patient's quality of life.

References

1. Adler, Douglas G MD; Baron, Todd H MD, FACP. Endoscopic Palliation of Malignant Gastric Outlet Obstruction Using Self-Expanding Metal Stents: Experience in 36 Patients. American Journal of Gastroenterology 97(1):p 72-78, January 2002. | DOI: 10.1111/j.1572-0241.2002.05423.x
2. Giovannini, Marc. (2019). EUS-guided hepaticogastrostomy. Endoscopic Ultrasound. 8. 35. 10.4103/eus.eus_47_19.
3. Dumonceau, Jean-Marc & Tringali, Andrea & Blero, Daniel & Devière, J & Laugiers, R & Heresbach, D & Costamagna, Guido. (2012). Biliary stenting: Indications, choice of stents and results: European Society of Gastrointestinal Endoscopy (ESGE) clinical guideline. Endoscopy. 44. 277-98. 10.1055/s-0031-1291633.
4. Van Der Merwe, S. W., Van Wanrooij, R. L., Bronswijk, M., Everett, S., Lakhtakia, S., Rimbas, M., ... & Van Hooft, J. E. (2022). Therapeutic endoscopic ultrasound: European society of gastrointestinal endoscopy (ESGE) guideline. Endoscopy, 54(02), 185-205. 10.1055/a-1717-1391

Introduction

A common finding in patients undergoing endoscopic retrograde cholangiopancreatography (ERCP) is periampullary diverticulum (PAD). It is more prevalent in females and elderly patients but is also linked to cholangitis and common bile duct stones. The anatomy may be distorted by PAD, possibly making papilla cannulation challenging. Whether PAD increases the difficulty of cannulation and whether it is linked to a higher incidence of adverse events has been debated. To provide further insights into this scenario, we conducted a nationwide registry-based study.

Aims & Methods

Our objective was to determine the impact of PAD on the cannulation rate, adverse events, and ERCP duration. We collected data on ERCP procedures performed between 2006 and 2021 from the Swedish Registry for Gallstone Surgery and ERCP (GallRiks). The 125,558 ERCPs that were registered in the registry during the study period were reduced to 73,937 after we excluded rendezvous procedures, previous sphincterotomies, previous stenting, and procedures with missing follow-up data.

Results

PAD was discovered in 12,782 (10.2%) ERCPs, with data recorded in the registry, and was present in 8,589 (11.6%) ERCPs in the study population. For 2,796 (3.8%) of the patients in the study group had the papilla inside the PAD (Boix Type 1), while 5,793 (7.8%) of the patients had a papilla on the edge of the PAD (Boix Type 2) or immediately next to it (Boix Type 3). The probability of successful cannulation was greater in non-PAD patients (89.7% vs. 88.6%; P=.002), whereas successful cannulation was reduced to only 81.1% if the papilla was located within the PAD (Type 1) (P<.001). The procedure time did not differ between the PAD (37.4 min) and non-PAD (37.5 min) groups (P=.73). There were no differences in intraprocedural complications (3.1%) or adverse events (16.4% vs. 15.5%; P=.05), whether PAD was present or not. In the PAD group, the risk of bleeding increased (1.8% vs. 1.3; P=.001). The perforation risk did not differ across the groups (PAD 0.8% vs. 0.7% non-PAD; P=.22), however PAD had a reduced incidence of cholangitis (1.8% vs. 2.3%; P=.01) and pancreatitis (4.5% vs. 5.2%; P=.008).

Conclusion

The cannulation rate is slightly reduced in the presence of PAD, and this effect is more pronounced and particularly apparent in patients with Type 1 PAD. The safety of ERCP is unaffected by the presence of PAD.

References

[1] Boix J, Lorenzo-Zúñiga V, Añaños F, et al. Impact of periampullary duodenal diverticula at endoscopic retrograde cholangiopancreatography: a proposed classification of periampullary duodenal diverticula. Surg Laparosc Endosc Percutan Tech 2006; 16: 208–211.
[2] Tham TCK, Kelly M. Association of periampullary duodenal diverticula with bile duct stones and with technical success of endoscopic retrograde cholangiopancreatography. Endoscopy 2004; 36: 1050–1053.
[3] Loffeld RJLF, Dekkers PEP. The Impact of Duodenal Diverticuli and the Execution of Endoscopic Retrograde Cholangiopancreaticography. Int Sch Res Not 2016; 2016: 5026289.
[4] Sun Z, Bo W, Jiang P, et al. Different Types of Periampullary Duodenal Diverticula Are Associated with Occurrence and Recurrence of Bile Duct Stones: A Case-Control Study from a Chinese Center. Gastroenterol Res Pract 2016; 2016: 9381759.
[5] Corral JE, Mousa OY, Kröner PT, et al. Impact of Periampullary Diverticulum on ERCP Performance: A Matched Case-Control Study. Clin Endosc 2019; 52: 65–71.
[6] Tabak F, Ji G-Z, Miao L. Impact of periampullary diverticulum on biliary cannulation and ERCP outcomes: a single-center experience. Surg Endosc 2021; 35: 5953–5961.

Clinical Case Summary

Introduction: Acute pancreatitis (AP) is an acute inflammatory disease characterized by severe abdominal pain and the development of local-systemic complications. In its etiology, drugs, especially gallstones and alcohol, can also cause AP (1). Drug-related acute pancreatitis constitutes 2-5% of the etiology of all acute pancreatitis (2). Here, we planned to present a patient diagnosed with Multiple Myeloma who developed AP after daratumumab.
Case: A 75-year-old male patient diagnosed with multiple myeloma complained of abdominal pain in the epigastric region radiating to the back on the 13th day of chemotherapy after starting the daratumumab, bortezomib and dexamethasone (DVd) protocol. There was no history of jaundice, abdominal pain, alcoholism, trauma, hyperparathyroidism. The patient, who did not use any medication other than DVd, increased serum amylase to 1601 U/L (28-100) and lipase to 1807 U/L (13-66) within seven days after the second dose of daratumumab treatment and was diagnosed with acute pancreatitis. The patient's abdominal computed tomography was compatible with AP and no stones or sludge were observed in the gallbladder. Other causes of pancreatitis were excluded. Since the patient who had previously received bortezomib treatment, had no complaints of abdominal pain, daratumamab was suspected as the etiology. The drug was discontinued and the patient received symptomatic medical treatment. After the treatment, the symptoms regressed and serum amylase and lipase returned to normal.
Conclusion: In order to say that any drug causes pancreatitis, other causes that may cause pancreatitis must be excluded, there must be findings such as regression of pancreatitis after discontinuation of the accused drug and recurrence of the pancreatitis attack when the drug is used again. We aimed to draw attention to drug use, which is a rare cause in the etiology of pancreatitis, by presenting a case of acute pancreatitis due to daratumumab use.

References

1. Jin DX, Lacson R, et al. A clinical model for early diagnosis of acute pancreatitis in the emergency department. Pancreas. 2018;47:871-79.
2. Lowe ME. Pancreatitis. In: Wyllie R, Hyams JS, Kay M, eds. 5th ed. Pediatric Gastrointestinal and Liver Disease. Philadelphia: Elsevier; 2016. p.1016-26

Clinical Case Summary

Approximately 20% of patients with Ulcerative Colitis (UC) develop Acute Severe UC (ASUC), for which intravenous systemic steroid therapy and possibly Infliximab-based rescue therapy is generally imposed. However, there are no significant guideline recommendations on ASUC regarding Vedolizumab as an alternative to Infliximab. A 56-year-old male, non-obese, past smoker, with steroid-dependent UC on therapy with mesalazine and therapeutic-targeted azathioprine developed ASUC induced by second-line chemotherapy (for development of colitis pembrolizumab-induced by immunotherapy set in the first line) with gemcitabine plus carboplatin for treatment of recurrence of right pleomorphic lung carcinoma with lower lobe involvement as well as lymph node to hilar and paratracheal sites (primary tumor, G3, arising on the left lung and treated with left pneumectomy). Treatment with intravenous methylprednisolone was imposed; however, there was no reduction in bowel movements in the days following admission. Rescue therapy with Infliximab was contraindicated because of the oncologic history. Surgical consultation contraindicated colectomy and abdomen computerized tomography showed no megacolon. Administration of Vedolizumab 300 mg was initiated. After infusion with Vedolizumab, there was a significant reduction in bowel movements starting the day after infusion until normalization of bowel movements within three days and the concomitant normalization of inflammatory indices. Enteral feeding was reintroduced since parenteral nutrition was set up in the previous days under clinical nutritionist consultation with customized nutritional bags. The patient is currently in clinical remission and on therapy with Vedolizumab 108 mg subcutaneously every two weeks and is in oncologic follow-up for pulmonary neoplasm. This case highlights a novel potential of Vedolizumab as a possible rescue therapy in ASUC, especially in special populations, where it may offer a better safety profile.

References

- Sedano R, Quera R, Simian D, et al. An approach to acute severe ulcerative colitis. Expert Rev Gastroenterol Hepatol. 2019;13:943–955.
- Spinelli A, Bonovas S, Burisch J, Kucharzik T, Adamina M, Annese V, Bachmann O, Bettenworth D, Chaparro M, Czuber-Dochan W, Eder P, Ellul P, Fidalgo C, Fiorino G, Gionchetti P, Gisbert JP, Gordon H, Hedin C, Holubar S, Iacucci M, Karmiris K, Katsanos K, Kopylov U, Lakatos PL, Lytras T, Lyutakov I, Noor N, Pellino G, Piovani D, Savarino E, Selvaggi F, Verstockt B, Doherty G, Raine T, Panis Y. ECCO Guidelines on Therapeutics in Ulcerative Colitis: Surgical Treatment. J Crohns Colitis. 2022 Feb 23;16(2):179-189.
- Choy MC, Seah D, Faleck DM, et al. Systematic Review and Meta-analysis: Optimal Salvage Therapy in Acute Severe Ulcerative Colitis. Inflamm Bowel Dis. 2019;25:1169–1186.
- Bonovas S, Fiorino G, Allocca M, et al. Biologic Therapies and Risk of Infection and Malignancy in Patients With Inflammatory Bowel Disease: A Systematic Review and Network Meta-analysis. Clin Gastroenterol Hepatol. 2016;14:1385-1397.e10.

Introduction

While understanding the cause is pivotal for managing acute pancreatitis (AP), there is currently no established protocol for determining its aetiology. Ongoing research aims to validate diagnostic protocols and integrate advanced tests for cases of idiopathic acute pancreatitis (IAP). This study evaluated current international opinion about diagnosis and follow-up of IAP.

Aims & Methods

An international survey was conducted through an online survey platform; the link was shared through social media, e-mails and conference presentations. Responses were collected anonymously. The 37 questions were developed by an international multidisciplinary team, part of a Pancreas2000 collaborative project.

Results

Respondents (n=247) were from over 35 countries; 39% of them had over 10 years of clinical experience; 73% were working in tertiary referral centres; 56% were gastroenterologists, 34% surgeons. An equal number of respondents (n=85, 34%) chose magnetic resonance cholangiopancreatography (MRCP) and endoscopic ultrasound (EUS) as the first test ideally performed in case of no suspected aetiology for AP after basic tests. When both investigations were available in the respondents’ centre, 44% chose other tests, 30% MRCP and 27% EUS. If there is no evidence of biliary aetiology, 79% of respondents would not perform cholecystectomy; the rest would choose recurrent AP and a history of elevated liver enzymes as main indications. No correlation was found when comparing tertiary and non-tertiary centres in performing cholecystectomies, similarly when comparing centres with or without EUS availability. Most respondents (90%) followed up on moderate and severe AP cases without an aetiology at index admission; 53% of centres followed up on all the IAP cases after first discharge. The follow-up was mostly done by gastroenterologists (62%); 33% of specialists would conduct a follow-up visit every 6 months, while 17% wouldn’t recommend follow-up visits.

Conclusion

This international survey highlights the lack of standardisation for diagnosis and follow-up of IAP. Although MRCP and EUS were ideally chosen as first options in IAP diagnosis, respondents chose other investigations in their practice even when both were available. Only half of the centres schedule visits, showing a lack of consensus regarding follow-up. There is a need for international standardisation for diagnosis and follow-up of IAP.

Clinical Case Summary

A 74-year-old man with a history of distal gastrectomy was hospitalized because of left lower extremity edema. Occlusion of left common iliac vein (CIV) and severe stenosis of left external iliac vein (EIV) were found (Video.1). Stents were used to release the edema. In order to investigate the pathogenesis, contrast-enhanced computed tomography was then carried out. Diffuse enlargement and continued enhancement of pancreas were found (Figure.1). Soft-tissue mass with low density grew around inferior mesenteric artery, left common iliac artery (CIA), left internal and external iliac arteries (Figure.2). Left CIV and EIV were compressed by the soft-tissue mass. Elevated serum immunoglobulin G4 (IgG4) was 598mg/dl. Therefore, the diagnosis of autoimmune pancreatitis (AIP) was confirmed. The soft-tissue mass was considered as AIP-associated retroperitoneal fibrosis (RF). The left lower extremity edema was actually attributed to AIP. Glucocorticoid was initially administered at 60mg/day for 2 weeks, and tapered by 5mg every week. Until to 20mg/day, glucocorticoid was tapered by 2.5mg every week. And it was finally maintained at a dose of 5mg/day.
Sausage-like shape is a typical sign of AIP¹. That serum IgG4 is more than 135mg/dl can be used for differential diagnosis from pancreatic cancer². According to the guideline², AIP was definitely confirmed based on diffuse enlargement of pancreas and elevated serum IgG4. Patients sometimes suffer from extra-pancreatic lesions, which are so called other organ involvement², such as RF. In this patient, the space between left CIA and vertebra became much narrow due to AIP-associated RF. Its compression contributed to the occlusion of left CIV that ran through the gap, resulting in the clinical symptoms of left lower extremity venous reflux disorder. This is the first AIP patient who primarily presented with left lower extremity edema. And no patient has been reported to suffer from left CIV occlusion caused by AIP-associated RF.

References

1. Masamune AA-O, Kikuta K, Hamada S, et al. Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2016. Journal of gastroenterology 2020;55(4):462-470.
2. Okazaki K, Kawa S, Kamisawa T, et al. Amendment of the Japanese consensus guidelines for autoimmune pancreatitis, 2020. J Gastroenterol 2022;57:225-245.