Clinical Case Summary

Introduction: Von Hippel-Lindau (VHL) syndrome is a rare hereditary neurocutaneous disorder distinguished by its propensity for the development of both benign and malignant tumors such as cerebellar hemangioblastomas, retinal angiomas, clear cell renal carcinomas, pancreatic neuroendocrine tumors, and others. Additionally, multiple pancreatic, renal, and urogenital cysts may also develop, potentially giving rise to localized symptoms and the risk of malignant transformations (1).
Case report: This case study centers on a 54-year-old male patient who was flagged for potential tumorous lesions in both kidneys upon undergoing a comprehensive health assessment. Following a multi-sliced computed tomography (MSCT)-guided biopsy, the presence of clear cell renal carcinoma was bilaterally confirmed. An abdominal MSCT delineated an enlarged pancreas featuring multiple cystic formations. Then, an Endoscopic Ultrasound (EUS) examination detailed the presence of multiple cysts in the pancreatic region, forming conglomerates with a collective dimension of up to 80 mm. Following an EUS-FNA (fine needle aspiration) procedure, cuboidal epithelium within the cyst was identified, and a biochemical analysis revealed normal levels of CEA, amylase, and glucose. The combination of these findings, along with the patient's medical history, raised suspicions of serous cystadenoma and prompted consideration of VHL syndrome. Finally, a heterozygous missense variant in the VHL gene was detected by sequencing genes included in the clinical exome panel.
Conclusion: Distinguishing cystic pancreatic lesions, which can give rise to endocrine and/or exocrine insufficiency or be asymptomatic, from potentially malignant lesions is of paramount importance. Therefore, diligent tumor surveillance associated with VHL syndrome is crucial for timely detection and treatment.

References

1. van Asselt SJ, de Vries EG, van Dullemen HM, Brouwers AH, Walenkamp AM, Giles RH, Links TP. Pancreatic cyst development: insights from von Hippel-Lindau disease. Cilia. 2013 Feb 5;2(1):3.

Introduction

EUS-guided Gallbladder Drainage (EUS-GBD) with Lumen Apposing Metal Stent (LAMS) is a standard treatment for management of acute cholecystitis (AC) in unfit-for-surgery patients. Gallbladder Perforation is standardly considered as a contraindication to EUS-GBD.

Aims & Methods

This series aims at exploring outcomes of EUS-GBD in patients with AC and contained perforation (cp-AC).
All consecutive patients undergoing EUS-GBD in a single tertiary institution between 2020 and 2024 were enrolled in a prospective registry (PROTECT, ClinicalTrials.gov NCT04813055). Patients with cp-AC were included in this series, when presenting gallbladder wall rupture with adjacent encapsulated fluid collections. Baseline demographics and technical details were registered together with Technical success, Clinical success, Adverse Events (AEs), Recurrence and Survival evaluated every 60 days.

Results

From a prospective cohort of 48 EUS-GBD, 19 patients presented with cp-AC (median age 71 [62-78], male 42.1%, Charlson Comorbidity Index of 8 [7-9.8]). Acute cholecystitis was iatrogenic in 79% of these cases (ERCP with metal stenting in 14 and TACE in 1). Reason to avoid surgery was underlying malignancy in 89.5% and comorbidities in 15.8%. Amongst underlying malignancies, the most frequent one was Pancreatic Adenocarcinoma.
Ascites was present in 26.3% of cases. All procedures were performed transduodenally, with an operative luminal space of 31 [25 – 36] mm and a median tract thickness of 6 [5-8] mm.
A 10 mm Hot Axios LAMS was used in 95% of cases, and the release was free-hand in 89% and over-the-wire after former distention in 11% of cases. Antibiotic prophylaxis and coaxial double-pigtail plastic stents were adopted in 95% of cases.
Technical success was 100%. Clinical success was 94.7%, with a statistically significant 7-days reduction of white blood cells (p=0.0017 ) and C Reactive Protein (p=0.0067) .
AEs were registered in 21% of cases, severe/fatal in 11%.
Of note, 2 AEs (1 fatal) were related to additional attempts of draining liver abscesses adjacent to the GBD.
Five patients (27%) underwent additional endoscopic procedures for peroral cholecystoscopy, GBD clearance and LAMS removal, while 2 (11%) patients experienced uneventful LAMS migration.
Median hospital stay was 8 [6-13] days and median time to chemotherapy was 26 [19-56] days for those who were candidate to oncological treatment (N=10).
After a median follow-up of 254 [56-454] days, no AC recurrence was registered.

Conclusion

In the context of AC, contained perforation with adjacent collections does not seem to represent a contraindication to EUS-GBD with LAMS, despite the additional complexity related to a thicker interluminal distance. Management of large adjacent abscesses should involve multidisciplinary management and might require percutaneous drainage.

Clinical Case Summary

Drug-induced liver injury is an adverse effect of many pharmaceutical agents. Alverine citrate, an antispasmodic agent found in the fixed-dose combination Meteospasmyl (alverine citrate and simethicone), is widely used in functional gastrointestinal disorders. To date, only three cases are published in the literature. We report a case, with strong evidence, including a rechallenge confirming the causality.
Patient Observation: A 43-year-old woman presented with biliary-type abdominal pain. Her medical history was limited to hypertension treated with bisoprolol with no alcohol use or recent medication. Abdominal ultrasound revealed uncomplicated cholelithiasis; liver tests were normal. Alverine citrate was prescribed for symptomatic relief. Within two weeks, she developed jaundice and fatigue with a normal clinical examination. Blood tests showed severe hepatocellular injury: ALT and AST were 22× and 39× ULN, bilirubin was 274 µmol/L, and GGT/ALP were mildly elevated. Viral and autoimmune panels were negative. Imaging excluded biliary obstruction. Liver biopsy revealed portal inflammation with eosinophilic infiltrates, suggestive of immunoallergic hepatitis. The discontinuation of the drug led to normalization of liver enzymes. 3 years later, the patient was inadvertently re-exposed to alverine citrate, triggering a reproducible episode of acute hepatitis, thereby confirming causality. Diagnostic probability was assessed with the RUCAM score (12, "highly probable") and Naranjo scale (8, "probable"). Only three prior cases of alverine citrate-induced hepatitis, none of which reported a confirmed rechallenge. Although liver biopsy is not routinely required in DILI, it was essential here for diagnostic clarification.
Alverine citrate should be recognized as a rare cause of acute hepatitis. This case confirms its hepatotoxic potential and underscores the importance of early recognition and drug withdrawal to prevent recurrence.

References

1. RUCAM Scale: https://clincasequest.hospital/rucam-scale/
2. Naranjo Scale: https://proeurodilinet.eu/wp-content/uploads/2019/03/Causality-assessment-training-course-15-MARCH-.pdf
3. Couzigou P et al. Hepatitis and Alverine Citrate. J Hepatol. 1997. https://www.sciencedirect.com/science/article/abs/pii/S0168827897801870
4. Turkish case report. Turk J Gastroenterol. 2004. https://pubmed.ncbi.nlm.nih.gov/15259090/
5. Korean case report. Korean J Hepatol. 2010. https://pubmed.ncbi.nlm.nih.gov/20375645/
6. Simethicone safety review. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4033443/
7. Zhang X et al. Histopathologic Manifestations of Drug-induced Hepatotoxicity. Clin Liver Dis. 2013.

Clinical Case Summary

Introduction: Wilson’s disease is a rare inherited liver disorder caused by copper metabolism dysfunction,whereas Crohn’s disease is a chronic inflammatory bowel condition.The co-occurrence of both conditions in an adolescent is extremely rare and becomes particularly noteworthy when associated with severe complications.
Case presentation: We report a 17-year-old male with cirrhotic Wilson’s disease,stable on D-penicillamine, who developed severe anal pain and bloody diarrhea.On proctologic examination,he had hemorrhoids, a s crotal fissure,and a non-draining anal fistula.
Laboratory investigations revealed severe anemia (Hb 4.4 g/dL),leukopenia,and elevated C-reactive protein. Colonoscopy showed multiple ileocolic ulcers on edematous mucosa, suggestive of active Crohn’s disease, confirmed by histopathological examination of biopsies. Pelvic MRI identified a grade II active, intersphincteric, abscessed perianal fistula. Antibiotic therapy was initiated accordingly. The patient was treated with high-dose intravenous corticosteroids for three weeks, followed by oral tapering, leading to significant clinical and laboratory improvement. However, during hospitalization, he developed disseminated intravascular coagulation (DIC) with thrombotic microangiopathy, requiring transfusion of fresh frozen plasma.His condition improved favorably thereafter
Discussion: The coexistence of Wilson’s and Crohn’s diseases is extremely rare. This case stands out for the onset of severe Crohn’s in a patient with chronic liver disease, a complex perianal fistula needing multidisciplinary care,and DIC with thrombotic microangiopathy—likely from systemic inflammation. It highlights the diagnostic and therapeutic challenges in adolescents with multiple chronic illnesses

Conclusion: The association between Wilson’s disease and Crohn’s disease is exceedingly rare. This case emphasizes the need for a multidisciplinary approach in managing complex patients, particularly when faced with severe complications.

Clinical Case Summary

Introduction: Pancreatic involvement in non-Hodgkin lymphomas is rare and often mimics pancreatic adenocarcinoma. We report a case in which endoscopic ultrasound (EUS) was pivotal in establishing the correct diagnosis.
Case Presentation: A 39-year-old male, a chronic smoker, presented with jaundice, anorexia, and fever. Laboratory findings revealed hepatocellular injury and cholestasis (total bilirubin: 15 mg/dL; GGT: 868 U/L; ALP: 679 U/L). CT imaging (Figure 1) demonstrated a large retroperitoneal mass infiltrating both the pancreas and adjacent vascular structures, associated with ascites and omental thickening. EUS (Figure 2) revealed a hypoechoic, poorly defined mass in the head and body of the pancreas (65×55 mm), with evidence of vascular invasion. A fine-needle biopsy (22G) was performed. Endoscopic Retrograde Cholangiopancreatography (ERCP) confirmed malignant duodenal stenosis. Histopathology was consistent with diffuse large B-cell lymphoma (DLBCL). The patient was initiated on R-CHOP chemotherapy (Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone) after baseline staging. Eight months later, follow-up EUS (Figure 3) showed no residual pancreatic mass.
Discussion: In atypical pancreatic masses—especially in younger patients with systemic symptoms—EUS is essential for tissue acquisition and accurate differential diagnosis. Unlike adenocarcinoma, pancreatic lymphomas respond well to chemotherapy.
Conclusion: Early use of EUS in atypical pancreatic masses can prevent misdiagnosis, reduce time to treatment, and avoid unnecessary surgical interventions.

References

Figure 1 - retroperitoneal mass infiltrating pancreas and vascular structures
Figure 2 - hypoechoic, poorly defined mass in the head and body of the pancreas
Figure 3 - no residual pancreatic mass

Clinical Case Summary

Hydatid cyst disease, caused by the Echinococcus parasite, is a zoonotic infection that typically targets the liver, lungs, and nervous system. It commonly occurs in regions such as the Maghreb countries, where it presents a significant health concern. While hydatid cysts in the liver are usually asymptomatic, complications such as cyst rupture, infection, or obstruction can arise, with portal vein involvement being particularly rare. This case report describes a 56-year-old male with a history of surgery for hydatid liver cyst, who presented with portal vein invasion mimicking portal vein thrombosis. Diagnostic imaging, including Color Doppler ultrasound and MRI, revealed a multiloculated cyst in the fourth liver segment with communication to the portal vein, leading to thrombosis and cavernous transformation of the portal vein. Despite the rupture of the hydatid cyst into the portal vein, no anaphylactic shock occurred. Portal vein thrombosis and cavernous transformation due to hydatid cysts represent an exceptional and complex complication of echinococcosis, highlighting the importance of early recognition and appropriate management. Treatment strategies should address both the hydatid liver cyst and associated portal hypertension, with surgical intervention being the cornerstone, including hepatectomies and laparoscopic approaches. This case highlights the necessity of heightened clinical awareness in endemic regions for managing this rare, potentially life-threatening complication.

Introduction

There has been a shift from percutaneous drainage towards endoscopic ultrasound (EUS)-guided drainage for management of post-operative pancreatic fluid collections (POPFC). However, available series have been limited by sample size and little is known about the factors that may influence clinical success.

Aims & Methods

Our aim was to evaluate the outcomes of EUS-guided drainage of POPFC following pancreatic surgery and identify predictors of clinical success.
We performed a retrospective review of patients who underwent EUS-guided drainage of POPFC between 2014 and August 2024 at three academic centers. Primary outcome was clinical success, defined as the complete radiological regression of the collection and resolution of symptoms without the need for additional drainage procedures.

Results

In total, 265 cases were included (mean age 64 [SD±13.9] years, 37% pylorus-resecting pancreatoduodenectomy). Early drainage (≤14 days) was performed in 52% of cases (n=138) and DPPS were used in 209 cases (79%).
Clinical success with a single EUS drainage procedure was achieved in 160 cases (60.4%). Step-up interventions were required in 67 patients (25.3%), 37 of which required endoscopic revision only. Technical success was achieved in 90% (n=238) and adverse events occurred in 43 cases (16%).
Stent type, drainage timing, splenectomy and drainage route did not seem to significantly influence clinical success. Multivariate analysis including stent type and surgical history, showed that distal pancreatectomy was an independent risk factor for clinical failure.

Conclusion

EUS-guided drainage of POPFC was effective in the majority of cases with a single procedure, although distal pancreatectomy was suggested as an independent risk factor for clinical failure. These results further support the use of EUS-guided drainage for the management of postoperative collections.

Disclosure

HH declares no competing interests. MB received trial support from Boston Scientific and holds consultancy agreements with Dekra, Ovesco and Prion Medical. GV received lecture fees from Boston Scientific and travel grants from Pentax Medical and Euromedical. RVW holds a consultancy agreement with Boston Scientific. Schalk van der Merwe holds the Cook chair in Interventional endoscopy, has consultancy agreements with Cook, Pentax and Olympus and co-chairs the Boston-Scientific Chair in Therapeutic Biliopancreatic Endoscopy.