Coral reef aorta (CRA) is a rare disease, described as hard calcifications involving the arterial wall which protrude into the lumen. It predominantly involves the posterior thoracic and abdominal aorta. CRA luminal lesions can cause significant aortic stenosis.
Patients usually present at around 50 years of age, which is younger than most patients suffering from other arterial occlusive diseases. The frequency of CRA is estimated to be 6 in 1,000 (0.6%) 1.
Patients present with arterial occlusion-like symptoms such as intermittent claudication and visceral ischaemia such as bowel involvement causing diarrhoea, weight loss, and abdominal pain. Renovascular arterial hypertension symptoms are also common 1.
The pathophysiology of CRA is not well understood. It often occurs in patients that have traditional atherosclerotic risk factors: hypertriglyceridaemia, hypercholesterolaemia, tobacco smoking, diabetes, and hypertension 1. There may be a calcification regulation defect secondary to lack of serum fetuin-A (a.k.a. alpha-2-Heremans-Schmid glycoprotein) which acts to inhibit ectopic calcification 1,3.
Non-contrast CT demonstrates dense, serpiginous, exophytic, calcifications of the aortic wall which protrude into the aortic lumen. This is in contrast to the typical appearance of atherosclerosis which follows the curve of the vessel wall. These lesions are located typically at the suprarenal and/or juxtarenal aorta causing significant occlusion. Lesions demonstrate a coral reef shape 2.
Recognition of the extensive endoluminal calcifications can have important implications for planning interventions and treatment, particularly for vascular surgeons and interventional radiologists 2.
Treatment and prognosis
Typically treatment is invasive surgery, most commonly thromboendarterectomy. Other techniques include aortoiliac and aortofemoral bypass. Relative postoperative complications include acute ischaemia of the lower extremities and viscera such as the bowel 1.
- severe aortic atherosclerosis: different from CRA by having calcific plaques involving the aortic wall without luminal projections 2
History and etymology
The term coral reef aorta was coined in 1984 by Qvarfordt et al1.
- 1. Grotemeyer D, Pourhassan S, Rehbein H et-al. The coral reef aorta - a single centre experience in 70 patients. Int. J. Angiol. 2012;16 (3): 98-105. Free text at pubmed - Pubmed citation
- 2. Kopani K, Liao S, Shaffer K. Radiology Case Reports. 2009;4 (1): . doi:10.2484/rcr.v4i1.209
- 3. Schafer C, Heiss A, Schwarz A et-al. The serum protein alpha 2-Heremans-Schmid glycoprotein/fetuin-A is a systemically acting inhibitor of ectopic calcification. J. Clin. Invest. 2003;112 (3): 357-66. doi:10.1172/JCI17202 - Free text at pubmed - Pubmed citation
- 4. Policha A, Moudgill N, Eisenberg J et-al. Coral reef aorta: case report and review of the literature. Vascular. 2013;21 (4): 251-9. doi:10.1177/1708538113478764 - Pubmed citation
- acute aortic syndrome
- thoracic aortic aneurysm
- abdominal aortic aneurysm
- endovascular aneurysm repair (EVAR)
- reporting tips for aortic aneurysms
- aortic coarctation
- aortic pseudocoarctation
- cervical aortic arch
- interrupted aortic arch
- transposition of the great arteries
- variant anatomy of the aortic arch
- traumatic aortic injury