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

Author:

Skopin Ivan I1,Arakelyan Valerii S2,Tchurikov Nickolai A3,Belyaev Andrei M4ORCID

Affiliation:

1. Cardiac Surgery Department, Bakoulev's Scientific Center of Cardiac Surgery, Moscow, Russia

2. Cardiovascular Surgery Department, Bakoulev's Scientific Center of Cardiac Surgery, Moscow, Russia

3. Department of Epigenetic Mechanisms of Gene Expression Regulation, Engelhardt Institute of Molecular Biology, Moscow, Russia

4. Cardiac Surgery Department, Bakoulev's Scientific Center of  Cardiac Surgery, Moscow, Russia

Abstract

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.

Publisher

Oxford University Press (OUP)

Subject

Cardiology and Cardiovascular Medicine,Pulmonary and Respiratory Medicine,Surgery

Reference1 articles.

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