Clinical Case Summary

A 30-year-old male was firstly evaluated at our gastroenterology outpatient clinic for chronic diarrhea, abdominal pain and weight loss (6kg in 4 months). His medical history included anal condylomatosis. Diagnostic workout for malabsorption syndrome was performed: celiac disease and other infective and noninfective causes of malabsorption were ruled out. Abdominal ultrasonography revealed invagination of the small intestine and mesenteric lymphadenopathy.
After HIV test positivity, a HAART (highly active antiretroviral therapy) with Dolutegravir/Lamivudine was set up. Soon after, the patient was admitted to Infectious Disease Unit for deep vein thrombosis and wasting syndrome.
Blood test showed mild anemia, severe hypoalbuminemia, electrolyte imbalance and slightly elevated inflammatory markers.
Esophagogastroduodenoscopy showed esophageal candidiasis, two precardial Herpetic erosions, hyperemic gastric mucosa, scalloping of duodenal folds with a mosaic pattern (fig.1).
Histological examination showed erosive chronic esophagitis with fungal hyphae and PAS+ spores; focally active chronic inflammation at antral and corpus level; duodenal villous atrophy (with intermediate severity) and crypt hyperplasia, with lymphoplasmacellular infiltrates in lamina propria and slightly increased intraepithelial CD3+ T lymphocytes. HSV, EBV and CMV tested positive at the immunostaining.
Colonoscopy with multiple biopsies showed substantial macroscopic normality, with mild chronic inflammation in every biopsy specimen.
Coproparasitological exam, coprocultures and serologic tests for enteropathogenes showed Candida albicans positivity.
During the hospitalisation, the patient received antiviral and antifungal therapy, HAART, albumin, fluid therapy, enoxaparin, energy-protein supplements.
At follow up, the patient continued HAART regularly, reaching full viral suppression. Weight gain was achieved, with no more episodes of diarrhea. Blood tests showed no more signs of malabsorption.

References

Figure 1. (endoscopic image) Duodenal mucosa (second portion) characterized by a mosaic pattern and flattened Kerckring folds with “scalloping”.

Clinical Case Summary

A 54-year-old man was referred to a Gastroenterology appointment due to weight loss and abnormal pancreatic findings on a CT scan, which revealed massive pancreatic enlargement. He had no relevant medical history. Endoscopic ultrasound demonstrated a globular pancreas with a hypoechoic but highly heterogeneous echotexture, multiple subcentimetric nodular formations and numerous millimetric hyperechoic foci, consistent with calcifications. Chromogranin A level was elevated at 143.2 ng/mL.
A 22G fine-needle aspiration of the pancreatic parenchyma was performed, but the result was inconclusive. Subsequently, PET/CT with 68Ga-peptides revealed a focal area of markedly increased uptake in the pancreatic tail (SUV max 47.8), highly suggestive of a neuroendocrine neoplasm, with increased radiotracer uptake throughout the pancreas. As a result, a percutaneous biopsy of the neoplasm and surrounding parenchyma (tail and body) was performed, confirming a well-differentiated (G1) pancreatic neuroendocrine tumor with amyloid stroma. Treatment was initiated with octreotide, later transitioned to lanreotide.
The patient underwent genetic evaluation, which revealed likely pathogenic variants c.1176+1_1176+7del p.? and c.361T>C p.(Cys121Arg) in the GCGR gene (heterozygous), confirming a diagnosis of Mahvash disease.
Two years later, during follow-up, abnormal liver function tests prompted abdominal magnetic resonance imaging, revealing liver nodules with characteristics of hypervascular metastases and signs of portal hypertension. An upper endoscopy was performed, revealing large esophageal varices with red spots, which were treated with two prophylactic elastic band ligations.
This case illustrates the development of portal hypertension arising from an extremely rare genetic disorder, with only about 15 confirmed cases of Mahvash disease reported to date. It is comprehensively documented through CT, PET imaging, histology, endoscopic ultrasound and upper endoscopy iconography.

References

- Yu, Run. “Plea to radiologists: Please consider Mahvash disease when encountering an enlarged pancreas.” World journal of radiology vol. 16,8 (2024): 371-374. doi:10.4329/wjr.v16.i8.371
- Robbins, Justin et al. “Liver Transplantation in a Woman with Mahvash Disease.” The New England journal of medicine vol. 389,21 (2023): 1972-1978. doi:10.1056/NEJMoa2303226

Clinical Case Summary

An 82-year-old man with Child B alcoholic cirrhosis, complicated by portal hypertension, underwent surveillance upper digestive endoscopy (UDE), revealing large esophageal varices and a 20 mm exophytic lesion, 40 cm from the dental arch, with an infiltrative and ulcerated appearance. Biopsies were inconclusive. Endoscopic ultrasound identified a heterogeneous, amorphous lesion with poorly defined borders and disruption of all wall layers, complicated by numerous large varices, preventing biopsy due to hemorrhagic risk. Computed tomography revealed parietal thickening in the distal esophagus and multiple varices, complicating lesion delineation. An oncology consultation recommended clinical surveillance, considering the patient's age, comorbidities, indeterminate nature of the esophageal lesion, and low suspicion for adenocarcinoma on ultrasound. One year later, surveillance UDE showed regression of the lesion.Histological samples were reviewed, revealing changes consistent with pyogenic granuloma. Pyogenic granuloma is a rapidly growing capillary hemangioma that may occur within the lumen of a pre-existing vessel (intravascular variant). The case is supported by appropriate iconography.

This case is presented with imaging, highlighting the challenge due to the macroscopic appearance suggestive of a neoplastic lesion and the pyogenic granuloma's growth within esophageal varices, limiting diagnostic and therapeutic management.

References

Clinical Case Summary

Background: Enteric duplication cysts are one of the rarest lesions detected in the pancreas. Pancreatic duplication cysts can be challenging to distinguish from neoplastic lesions, particularly in symptomatic patients. Radiologic imaging, such as Computed Topography (CT) and Magnetic Resonance Imaging (MRI), may not always provide a clear differentiation, leading to potential misdiagnosis.
Case presentation: We present the case of a Fifty-seven male patient with a history of long-standing epigastric pain, dyspepsia, and weight loss. Upper and lower endoscopy showed only mild gastritis. Abdominal CECT abdomen showed a well-circumscribed pancreatic lesion of about 20x25 mm with no vascular invasions. Endoscopic ultrasound showed a well-circumscribed pancreatic neck lesion about 20x22 mm, anechoic, negative color flow doppler, soft on elastography, free surrounded vessels with no posterior acoustic enhancement. EUS suggested a cystic lesion versus a vascular NET. EUS fine-needle aspiration (FNA) was done with aspiration of gelatinous material and onsite cytopathology suggested a benign cystic lesion. Further pathological interpretation confirms the diagnosis of a duplication cyst leading to appropriate management without avoidance of major invasive surgical intervention.
Conclusion: EUS and EUS-guided biopsy have a crucial role in differentiating benign pancreatic conditions from malignancies, particularly in patients presenting with nonspecific symptoms and ambiguous radiologic findings. EUS should be considered early in the diagnostic process to avoid misdiagnosis and unnecessary surgical procedures.

Disclosure

Nothing to disclose

Clinical Case Summary

Background: Endoscopic ultrasound (EUS)-guided cystogastrostomy with lumen-apposing metal stents (LAMS) is a widely accepted treatment for symptomatic pancreatic pseudocysts, particularly in cases of walled-off necrosis (WON). While the procedure is generally safe, delayed bleeding can occur. This report presents a novel approach to managing massive upper gastrointestinal bleeding following LAMS placement when interventional radiology (IR) embolization is not feasible.
Case presentation: A 51-year-old male with a history of pancreatitis and a large pancreatic pseudocyst underwent successful EUS-guided placement of an Axios stent. The patient was discharged on the second day of the procedure with no adverse events. Three days later, the patient developed significant melena, pallor, and a dramatic drop in hemoglobin levels, necessitating urgent blood transfusions. Diagnostic upper endoscopy revealed active pulsatile bleeding from the cyst wall behind the stent edge, which is inaccessible to conventional endoscopic methods. Two attempts of angiography by two different IR consultants failed to locate the bleeding source.
Given the critical situation and after resuscitation with IV blood transfusion, a multidisciplinary team proposed a novel technique: injection of Purastat, a hemostatic agent, into the cyst through the stent, followed by stent removal and additional application of glue over the cyst orifice. This approach successfully controlled the bleeding, stabilized the patient’s condition, and eliminated the need for surgery. Follow-up imaging revealed a small splenic artery aneurysm without active bleeding.
Conclusion: This case highlights the potential of Purastat as an effective tool for managing life-threatening gastrointestinal bleeding following LAMS placement. The successful use of this technique in sealing the cyst and stopping the bleeding suggests it could be a definitive management option in similar cases.

References

1. Shamah S, et al. Delayed bleeding after lumen-apposing metal stent placement for pancreatic fluid collections: Incidence, risk factors, and management. *Endoscopy International Open*. 2023;11(2):E158-E163.
2. Varadarajulu S, et al. Hemostasis in gastrointestinal endoscopy: Emerging techniques and devices. *Gastrointestinal Endoscopy Clinics of North America*. 2022;32(3):421-439.

Introduction

Phase Angle (PA) represents a useful bioimpedance analysis-assessable marker of sarcopenia, accurately predicting liver-related hospitalizations in advanced chronic liver disease (ACLD), correlating with C-reactive protein levels. Clinically significant portal hypertension (CSPH) and systemic inflammation constitute the main hepatic decompensation drivers. A relevant proportion of Metabolic dysfunction-associated steatotic liver disease (MASLD) patients present sarcopenia (configuring the paradox of “sarcopenic obesity”), as well as compensated ACLD (cACLD)-MASLD individuals more rapidly progress to decompensation (dACLD) rather than other etiologies. However, the clinical applications of PA have never been specifically investigated in the setting of MASLD.

Aims & Methods

Considering the background, in MASLD, we primarily aimed to evaluate the trend of PA values across the different liver disease progression stages, investigating the relationship with the hepatic decompensation drivers; in dACLD-MASLD, we aimed to estimate the accuracy of the novel PA/C-reactive protein (CRP) index (PAC-I) in predicting 3-month further liver-related events (LREs)-determining hospitalizations.
In this observational study, between December 2023 and January 2025, MASLD patients were consecutively enrolled and stratified according to current LSM and eventual previous LREs in < F3 fibrosis (AF) (n.50), cACLD (n.50), and dACLD (n.50) patients. At baseline, anthropometrical, biochemical, nutritional, and clinical data [including the endoscopic assessment of CSPH and the relative severity (F1, F2, F3) of esophageal varices (OEVs)] were collected. Bioimpedance analysis was adopted to assess body composition parameters. Sarcopenia was diagnosed according to the European Working Group on Sarcopenia (EGWSOP2) criteria. MASLD-dACLD patients were followed up, and further acute decompensation (AD)-LREs-determining hospitalizations [severe ascites, severe hepatic encephalopathy (HE), OEV bleeding, and any acute bacterial infections] in the subsequent 3 months were recorded.

Results

In MASLD patients, PA (°) values inversely correlated with LSM [R: -0.711; p<0.0001], progressively decreasing according to the worsening of liver disease stages, emerging as statistically significantly lower in dACLD compared with cACLD individuals (p<0.0001). In dACLD patients, PA inversely correlated with CRP levels [R: -0.945; p:0.001]. Focusing on CSPH, PA values were significantly lower in > F2-OEV-affected individuals (p<0.0001). Multivariate analysis revealed CSPH (aHR 1.221, p<0.0001), CRP (aHR: 1.112, p:0.002), and PA (aHR: 1.178, p:0.003) as the variables significantly associated with 3-month further LREs’ occurrence in dACLD patients.
ROCy analysis revealed an elevated accuracy and superiority compared to PA alone (AUC PA:0.717; AUC PAC-I: 0.893; p:0.003) of PAC-I in predicting 3-month further LREs’ occurrence, identifying PAC-I < 2.35 as the best cut-off. Consistently, dACLD patients showing baseline PAC-I < 2.35 presented a higher risk [HR: 2.287, p: 0.032] of further LREs.

Conclusion

In MASLD, PA correlates with the main driver of progression, and the PAC-I represents a useful noninvasive tool in predicting short-term liver-related hospitalizations in dACLD patients.

References

1. Espirito Santo Silva DD, Waitzberg DL, Passos de Jesus R, Oliveira LPM, Torrinhas RS, Belarmino G. Phase angle as a marker for sarcopenia in cirrhosis. Clin Nutr ESPEN. 2019 Aug;32:56-60. doi: 10.1016/j.clnesp.2019.05.003. Epub 2019 May 31. PMID: 31221291.
2. Saueressig C, Glasenapp JH, Luft VC, Alves FD, Ferreira PK, Hammes TO, Dall'Alba V. Phase Angle Is an Independent Predictor of 6-Month Mortality in Patients With Decompensated Cirrhosis: A Prospective Cohort Study. Nutr Clin Pract. 2020 Dec;35(6):1061-1069. doi: 10.1002/ncp.10584. Epub 2020 Oct 15. PMID: 33058222.

Introduction

It is recognised that large common bile duct (CBD) stones present a significant challenge in endoscopic practice. A key performance indicator of >80% clearance at the initial ERCP has been recommended to standardise quality of care. Standard techniques such as sphincterotomy +/- balloon sphincteroplasty are usually sufficient; however, in difficult cases, plastic stents are frequently placed to maintain biliary drainage, with elective repeat ERCP planned. When clearance remains unsuccessful, options such as mechanical lithotripsy or cholangioscopy may be employed, though these are often limited to tertiary care centres. Fully covered self-expanding metal stents (FCSEMS) have been proposed as a novel strategy to remodel the distal CBD and facilitate stone retrieval at subsequent procedures. While this approach is not currently included in formal UK guidelines, its use is expanding in complex cases. We report our early experience comparing plastic stents alone with a combination of fully covered metal stents and coaxial plastic stents in a secondary care setting.

Aims & Methods

This was a retrospective observational study conducted at a large secondary care NHS trust. We included patients undergoing ERCP between 2019 and 2024 in whom complete stone clearance could not be achieved at the initial procedure and a stent was placed. Only patients with confirmed choledocholithiasis and naïve papillae at first ERCP were included. All patients had large CBD stones, defined as stones greater than 10 mm in diameter. Patients were categorised according to whether a plastic stent alone or a fully covered metal stent with coaxial plastic stent was used at the index procedure. A 7Fr pigtail plastic stent was used in all cases; for the FCSEMS group, a fully covered metal stent was inserted with a plastic stent placed through it. Data were collected from endoscopy records and electronic patient notes. Outcomes assessed included complete stone clearance at second ERCP, need for mechanical lithotripsy, number of ERCP procedures, and referral to tertiary care for cholangioscopy. Fisher’s exact test and unpaired t-tests were used for statistical analysis.

Results

A total of 63 patients were included, with 46 in the plastic stent group and 17 in the FCSEMS group. Complete stone clearance for large stones (>10 mm) at second ERCP was achieved in 63.0% of patients in the plastic group compared with 76.5% in the FCSEMS group (p=0.380). Mechanical lithotripsy was required in 8.7% of plastic stent cases and 5.9% of FCSEMS cases (p=1.000). Referral to tertiary care for cholangioscopy occurred in 30.4% of the plastic group and 11.8% of the FCSEMS group (p=0.195). The mean number of ERCPs per patient was similar between groups, with an average of 2.50 procedures in the plastic group and 2.41 in the FCSEMS group (p=0.601).

Conclusion

The use of fully covered metal stents with coaxial plastic stents was associated with promising trends, including higher rates of complete stone clearance for large CBD stones and fewer referrals to tertiary centres. Although these findings did not reach statistical significance, they suggest a clinically relevant benefit that merits further investigation. The higher observed clearance rate and reduced need for cholangioscopy may support FCSEMS as a valuable adjunct in managing difficult bile duct stones. These results should be interpreted cautiously given the small size of our dataset, but they provide an encouraging basis for future prospective studies aimed at optimising CBD stone management and potentially reducing the need for tertiary care referral.

Disclosure

Nothing to disclose