Embolisation of Hepatic Artery Aneurysm in Vascular Ehlers-Danlos Syndrome

Background: Vascular Ehlers-Danlos syndrome is a rare connective tissue disorder characterised by arterial fragility, predisposing patients to life-threatening vascular complications. Endovascular aneurysm management in these individuals poses significant challenges due to their delicate vasculature and limited surgical options. This case highlights the novel use of a direct percutaneous transhepatic approach for aneurysm coiling in a patient with a rapidly expanding hepatic artery aneurysm, demonstrating an innovative solution to a complex vascular emergency. Case presentation: A young male with a known diagnosis of vascular Ehlers-Danlos syndrome presented with abdominal pain from a perforated sigmoid colon. After undergoing a midline exploratory laparotomy, imaging revealed a rapidly expanding 3 cm aneurysm in his common hepatic artery. Traditional endovascular coiling was not feasible due to significant stenosis proximal to the aneurysm, which created similar limitations for vascular reconstruction and liver transplantation. After deliberation, the multidisciplinary team opted for a direct transhepatic approach to coil the aneurysm. The interventional radiology team successfully coiled both the Common and left hepatic artery aneurysms using a 6F sheath to deliver 27 microcoils. Ultrasound and fluoroscopy were used to guide direct transhepatic access, with a curvilinear probe employed for real-time ultrasound guidance. Arteries supplying the aneurysm, including the gastroduodenal artery, were embolised using a total of 12 vials of Onyx. The tract was embolised with Spongistan (gelfoam) upon sheath withdrawal to minimise bleeding risk. The patient recovered without complications, and follow-up imaging confirming haemodynamic stability and adequate liver perfusion. Conclusions: This case highlights the importance of an integrative multidisciplinary approach in managing complex vascular emergencies and successfully demonstrates how a direct percutaneous transhepatic approach can serve as a valuable reference for similar cases, expanding the repertoire of endovascular interventional radiology techniques for challenging pathologies.