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An isolated aneurysm of the abdominal aorta in a patient with Marfan's syndrome - a case report.

07:00 EST 11th February 2019 | BioPortfolio

Summary of "An isolated aneurysm of the abdominal aorta in a patient with Marfan's syndrome - a case report."

We present a case of successfully treated abdominal aortic aneurysm in a 24-year old patient with Marfan's syndrome. Following initial physical and ultrasound examination, the multislice computed tomography scan revealed infrarenal aortic aneurysm of 6 cm in diameter, 10 cm long, along with slightly dilated iliac arteries. However, dimensions of aortic root, aortic arch and descending suprarenal aorta were within normal limits. Further on, since the patient presented with signs of impending rupture, an urgent surgical intervention was performed. The patient was discharged in good general medical condition 7 days following surgery. After 6 months of follow-up, the patient's condition was satisfying and no MSCT-signs of further aortic dissection/aneurysm were identified. To the best of our knowledge a case of successful management of a patient with Marfan's syndrome and truly isolated infrarenal and symptomatic abdominal aortic aneurysm has not been described in the literature before.

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This article was published in the following journal.

Name: Annals of vascular surgery
ISSN: 1615-5947
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Medical and Biotech [MESH] Definitions

An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE, dilation of the AORTA, and aortic dissection. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome is associated with mutations in the gene encoding fibrillin, a major element of extracellular microfibrils of connective tissue.

An abnormal balloon- or sac-like dilatation in the wall of the ABDOMINAL AORTA which gives rise to the visceral, the parietal, and the terminal (iliac) branches below the aortic hiatus at the diaphragm.

An autosomal dominant aneurysm with multisystem abnormalities caused by increased TGF-BETA signaling due to mutations in type I or II of TGF-BETA RECEPTOR. Additional craniofacial features include CLEFT PALATE; CRANIOSYNOSTOSIS; HYPERTELORISM; or bifid uvula. Phenotypes closely resemble MARFAN SYNDROME; Marfanoid craniosynostosis syndrome (Shprintzen-Goldberg syndrome); and EHLERS-DANLOS SYNDROME.

The portion of the descending aorta proceeding from the arch of the aorta and extending to the DIAPHRAGM, eventually connecting to the ABDOMINAL AORTA.

A condition caused by occlusion of terminal aorta, the primary branches of the ABDOMINAL AORTA, as in aortoiliac obstruction. Leriche syndrome usually occurs in males and is characterized by IMPOTENCE, absence of a pulse in the femoral arteries, weakness and numbness in the lower back, buttocks, hips, and lower limbs.

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