A giant left internal iliac artery aneurysm in a patient with Loeys–Dietz syndrome

• Published in: Interactive cardiovascular and thoracic surgery 2021-10, Vol.33 (5), p.832-833
• Main Authors: Skopin, Ivan I, Arakelyan, Valerii S, Tchurikov, Nickolai A, Belyaev, Andrei M
• Format: Article
• Language: English
• Subjects: Adolescent; Aneurysm, Dissecting; Angiography; Aorta, Abdominal; Case Reports; Female; Humans; Iliac Aneurysm - diagnostic imaging; Iliac Aneurysm - surgery; Iliac Artery; Loeys-Dietz Syndrome - complications; Loeys-Dietz Syndrome - diagnostic imaging; Loeys-Dietz Syndrome - genetics
• ISSN: 1569-9285; 1569-9293; 1569-9285
• DOI: 10.1093/icvts/ivab157

Abstract A 16-year-old female presented with left iliac fossa pain. In January 2021, she was admitted to her local hospital with severe lower abdominal pain and the pelvic ultrasound demonstrated a 13-cm left internal iliac artery dissecting aneurysm with its partial thrombosis. On examination, she had a high-arched palate, multiple skin stretch marks, flat feet and a soft systolic ejection murmur at the left 5th mid-clavicular line. She had a mildly tender abdomen in the left iliac fossa. Computed tomography angiography demonstrated a 12.2 cm × 10.4 cm × 12.5 cm left internal iliac artery aneurysm. During surgery, the aneurysm was incised and the proximal and distal orifices of the internal iliac artery were ligated. Genetic testing yielded 2 mutations in the SMAD3 gene characteristic for Loeys–Dietz syndrome. Isolated iliac artery aneurysms are rare with an incidence in an autopsy study of 0.03%.