Images in Hepatology - Answer

Giant Hemangioma Causing Budd-Chiari Syndrome

This prospective study evaluated the safety and effectiveness of percutaneous sclerotherapy in the treatment of secondary Budd-Chiari syndrome due to hepatic venous malformations (HVMs). Four patients (mean age, 40 years; 3 women) with 5 HVMs underwent 7 sessions of percutaneous sclerotherapy with a mixture of bleomycin and lipiodol. All patients had chronic Budd-Chiari syndrome, determined based on imaging findings, with the main symptom being abdominal discomfort and distention. On physical examination, 2 patients had ascites and the other 2 had an epigastric mass. The indication for treatment was intractable abdominal symptoms due to hepatic and/or inferior vena cava (IVC) outflow compression. All procedures were technically successful, with no major complications. Three patients underwent a second session because of incomplete IVC decompression. The patients’ symptoms completely resolved at 6 and 12 months of follow-up. There was a significant reduction in lesion volume (P = .007) and an increase in IVC luminal area (P = .018) at 12 months of follow-up.

Hepatic hemangiomas (HHs) are benign liver lesions often discovered incidentally on imaging for various unrelated pathologies. We herein review the etiology, classification, diagnostic imaging, and management of HHs.

A comprehensive systematic review was performed utilizing MEDLINE/PubMed and Web of Science databases, with the end of search date being March 1, 2022, regarding HH diagnosis, imaging, and management.

HHs can be broadly classified as capillary hemangiomas or cavernous hemangiomas. While the exact pathophysiology related to the development of HHs remains largely unknown, hormone exposure has been postulated to cause HH growth. HHs appear homogenously hyperechoic on US with distinct margins and posterior acoustic enhancement. While cavernous hemangiomas appear as well-defined hypodense lesions on pre-contrast CT images with the same density as the vasculature, one of the most reliable imaging features for diagnosing cavernous hemangiomas is high signal intensity on T2 weighted images. While most HHs are asymptomatic, some patients can present with pain or compressive symptoms with bleeding/rupture being very rare. Kasabach-Merritt syndrome is a rare but life-threatening condition associated with thrombocytopenia and microangiopathic hemolytic anemia. When HHs are symptomatic or in the setting of Kasabach Merritt syndrome, surgery is indicated. Enucleation is an attractive surgical option for HH as it spares normal liver tissue. Most patients experience symptom relief following surgical resection.

HHs are very common benign liver lesions. High-quality imaging is imperative to distinguish HHs from other liver lesions. Surgery is generally reserved for patients who present with symptoms such as pain, obstruction, or rarely Kasabach-Merritt syndrome. Surgery can involve either formal resection or, in most instances, simple enucleation. Patients generally have good outcomes following surgery with resolution of their symptoms.