Emergent embolization of a ruptured splenic artery aneurysm complicating Menkes disease

Area of Science:

• Vascular Surgery
• Pediatric Medicine
• Genetics

Background:

• Menkes disease is a rare genetic disorder affecting copper metabolism.
• Vascular abnormalities, including aneurysms, are known complications of Menkes disease.
• Splenic artery aneurysms are rare but can be life-threatening.

Observation:

• A 7-year-old boy with Menkes disease presented with a ruptured splenic artery aneurysm.
• Angiography revealed significant tortuosity in mesenteric and lower extremity vasculature, including bilateral femoral arteries.
• No additional aneurysms were identified in the mesenteric or lower extremity vasculature.

Findings:

• Successful endovascular embolization of the splenic artery aneurysm was achieved using microcoils and n-butyl cyanoacrylate.
• The patient remained free of further vascular complications during a 3-year follow-up period.
• Annual computed tomography angiography confirmed the absence of new vascular events.

Implications:

• This case demonstrates the feasibility and success of endovascular management for arterial aneurysms in patients with Menkes disease.
• It underscores the need for vigilant long-term surveillance of vascular health in individuals with Menkes disease.
• Early detection and intervention for vascular complications can significantly improve patient outcomes.