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Ulnar Artery Aneurysm as a Late Sequela of Marfan Syndrome
Christopher J Goodenough1, Rana O Afifi2, Siddharth K Prakash3
1Department of Surgery, Division of Plastic Surgery, University of Texas Health Sciences Center at Houston, Houston, TX.
Abstract:
Marfan syndrome (MFS) is a connective tissue disorder caused by mutations of the FBN1 gene encoding fibrillin-1, which leads to overexpression of transforming growth factor-β, increased hyaluronan deposition, and matrix metalloproteinase activity in the media of the aorta and other muscular arteries. Marfan syndrome patients present with connective tissue laxity and aneurysmal changes to muscular arteries. Successful medical and surgical intervention has prolonged the life expectancy of MFS patients, which can allow atypical presentations of the syndrome to manifest. We present a case of a 49-year-old man with MFS who developed an ulnar artery aneurysm that was treated by excision and vein grafting.
Insights
Marfan syndrome, a genetic connective tissue disorder, can lead to arterial aneurysms. This case highlights an ulnar artery aneurysm in a Marfan syndrome patient, successfully treated with surgery.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Connective Tissue Disorders
Background:
- Marfan syndrome (MFS) is an inherited connective tissue disorder caused by FBN1 gene mutations.
- It leads to aortic and arterial aneurysms due to altered extracellular matrix composition.
- Increased life expectancy in MFS patients allows for atypical manifestations.
Observation:
- A 49-year-old male patient with known Marfan syndrome presented with an ulnar artery aneurysm.
- This is an uncommon presentation of vascular involvement in MFS.
Findings:
- The ulnar artery aneurysm was successfully treated by surgical excision.
- Vein grafting was utilized for arterial reconstruction.
Implications:
- This case expands the understanding of vascular manifestations in Marfan syndrome.
- It underscores the importance of vigilance for peripheral arterial aneurysms in MFS patients.
- Surgical management can be effective for these rare presentations.
Related Concept Videos
Aneurysm I: Introduction
Aneurysm II: Clinical Manifestations and Diagnostic Studies
Aneurysm III: Interprofessional Care
Aortic Regurgitation III: Medical Management
Aortic Regurgitation I: Introduction
Mitral Valve Prolapse I: Introduction

