Clinical Case Summary

We report a rare and fatal case of diffuse hepatic hemangiomatosis (DHH) in a 50-year-old male presented with acute painless jaundice of 10 days’ duration, dark urine, and hepatosplenomegaly. Laboratory workup revealed normocytic anemia, indirect hyperbilirubinemia, and elevated LDH levels, suggesting hemolysis, though the direct Coombs test was negative. Liver function tests showed cholestatic and hepatocellular injury patterns, while creatinine and coagulation profiles were initially normal. Abdominal ultrasound and triphasic computed tomography demonstrated hepatosplenomegaly and multiple hepatic focal lesions with peripheral nodular enhancement and centripetal fill-in. Liver biopsy revealed markedly dilated sinusoids without malignant infiltration. Unlikely, the patient developed acute kidney injury, hematemesis, and hepatic encephalopathy, culminating in death from liver failure within one month from the onset of his illness.

Clinical Case Summary

In advanced chronic liver disease (ACLD) patients, the endoscopic evidence of esophageal and/or gastric varices denotes the presence of clinically significant portal hypertension (CPSH), representing the main clinical consequence of portal hypertension (PH).
In this setting, although infrequently, besides typical sites (i.e., esophagus and stomach), PH-related varices can manifest even in other atypical portosystemic-shunt districts, including the vagina. CSPH-related vaginal varices bleeding can occur as a rare complication documented by limited case reports.
A 62-year-old female patient with viral (eradicated Hepatitis C Virus infection)-related- decompensated (grade 2 ascites)-ACLD, complicated by CSPH (grade F2 esophageal varices), was admitted to our Department in February 2025 for microcitic anemia. Anamnesis revealed a previous gastric cancer managed with total gastrectomy resection in 2021.
On admission, abdominal imaging showed radiological signs of ACLD/PH (coarse pattern, splenomegaly, dilated splenic-portal axis). Esophagogastroduodenoscopy ruled out active sources of bleeding, whereas colonoscopy revealed anal congestive not-bleeding varices. During the hospitalization, an episode of metrorrhagia imposed gynecologic evaluation with vaginal speculum examination. This assessment documented vaginal varices with the relative rupture as the current cause of the bleeding, suggesting this site is the further district for (CS)PH manifestation consequently to potentially occurred total gastrectomy-determined changes in abdominal vascular anatomy.
In ACLD patients receiving surgery altering abdominal vascularization, variceal bleeding represents a possible CSPH-related complication occurring even in atypical sites, such as the vagina. This scenario imposes an adequate tailored management, monitoring unusual bleeding signs, and adopting appropriate preventive strategies by including gynecologic evaluation in follow-up.

References

1. McHugh PP, Jeon H, Gedaly R, Johnston TD, Depriest PD, Ranjan D. Vaginal varices with massive hemorrhage in a patient with nonalcoholic steatohepatitis and portal hypertension: Successful treatment with liver transplantation. Liver Transpl. 2008 Oct;14(10):1538-40. doi: 10.1002/lt.21506. PMID: 18825698.
2. Kreek MJ, Raziano JV, Hardy RE, Jeffries GH. Portal hypertension with bleeding vaginal varices. Ann Intern Med. 1967 Apr;66(4):756-9. doi: 10.7326/0003-4819-66-4-756. PMID: 6067181.
3. Chan G, Taqi A, Drage M, Quan D. A case report of massive vaginal variceal hemorrhage in a patient with cirrhosis secondary to nonalcoholic steatohepatitis unresolved even after successful liver transplantation. Transplant Proc. 2013 Mar;45(2):835-7. doi: 10.1016/j.transproceed.2012.04.033. Epub 2012 Sep 19. PMID: 23267803.
4. Lebrec D, Benhamou JP. Ectopic varices in portal hypertension. Clin Gastroenterol. 1985 Jan;14(1):105-21. PMID: 3872747.
5. Nagata J, et al. Vaginal bleeding in a patient with type C liver cirrhosis without a past history of laparotomy: successful treatment with partial splenic artery embolization. Clin J Gastroenterol. 2012 Aug;5(4):275-81. doi: 10.1007/s12328-012-0316-3. Epub 2012 Jun 30. PMID: 26182393.

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

Although liver involvement is common in leukemia, acute hepatitis as its initial presentation is rare. This case highlights the diagnostic challenge of distinguishing reactive hematological changes from underlying malignancies in patients with unexplained hepatitis.
A 35-year-old woman presented in February 2024 with myalgias, fever with chills (38°C), jaundice, and right upper quadrant discomfort. Relevant history included recent intake of herbal supplements (vitamin C, zinc, echinacea, elderberry).
Initial laboratory findings showed leukocytosis, lymphocytosis (25%), monocytosis (12%), blasts (3%), thrombocytopenia (136×10⁹/L), and abnormal liver tests (AST 448 U/L, ALT 686 U/L, ALP 242 U/L, GGT 147 U/L, total bilirubin 4.14 mg/dL, direct bilirubin 2.9 mg/dL), with normal INR. Peripheral blood smear revealed mononuclear pleomorphism and lymphocytes with prominent nucleoli. Abdominal computed tomography showed moderate hepatomegaly without biliary or vascular abnormalities. Comprehensive testing for infeccious, autoimmune and metabolic causes was negative.
Liver tests improved and leukocytosis turned into leukopenia. Repeat blood smear was deemed reactive by Hematology. She was discharged and in early March re-evaluation, liver parameters normalized and leukopenia improved.
Later that month, she returned with fever, night sweats, and abdominal pain. Laboratory findings showed recurrent cytopenias and cytocholestasis. Blood smear revealed increasing blast count, and immunophenotyping suggested monoclonality. Bone marrow and liver biopsy confirmed B-cell acute lymphoblastic leukemia (ALL) with hepatic infiltration.
This case illustrates an unusual presentation of ALL as relapsing acute hepatitis. It reinforces the need to consider hematological malignancy in cryptogenic hepatitis, particularly when associated with evolving hematological changes. Serial blood smears, immunophenotyping, and timely bone marrow evaluation are essential to avoid diagnostic delay.

References

1. Hepatic biopsy: clusters of intermediate to large cells expressing TdT, CD20, PAX5, CD10, BCL6, CD43.
2. Bone marrow aspirate: 64% blasts, CD10+, CD20+ (strong homogeneous expression).
3. Bone marrow biopsy: increased cellularity due to diffuse interstitial infiltration by intermediate cells with blast-like morphology and lymphoid immunophenotype (TdT+, CD34+, MPO-, CD20+).