Segmental arterial mediolysis
Citation, DOI & article data
Segmental arterial mediolysis (SAM) is an increasingly recognized vascular disease of the middle-aged and elderly and a leading cause of spontaneous intra-abdominal hemorrhage. It is characterized by fusiform aneurysms, stenoses, dissections and occlusions within splanchnic arterial branches. Imaging is not only the key to diagnosis of this condition, but can also facilitate life-saving endovascular therapy.
Segmental arterial mediolysis was first described as a discrete entity in 1976 1, and was initially thought to be very rare 2. Only 14 cases were reported over 20 years to 1997, but in the last decade reports have become much more frequent 3, and this is thought to be a combination of increased use of thin slice computed tomographic angiography (CTA), and increased awareness in the radiological community. The incidence may be as high as 1 per 100 000 per year 4.
Segmental arterial mediolysis most commonly presents as spontaneous intra-abdominal hemorrhage in patients aged 50 to 80. Bleeding may occur into the mesentery or peritoneum, or less commonly, into the bowel lumen. Abdominal pain, distension, shock, falling hematocrit are typical. Bowel ischemia, hematuria, or hemobilia are also described 3. For middle-aged patients with non traumatic spontaneous mesenteric hemorrhage, segmental arterial mediolysis is the most likely underlying cause. Mortality has been described as high as 50% 5.
Segmental arterial mediolysis is an uncommon arteriopathy, which is not atherosclerotic or inflammatory. The underlying histological process is lysis of the smooth muscle of the outer media of the arterial wall 5, resulting in intramural hemorrhage, saccular or dissecting aneurysms, thrombosis and hemorrhage. It affects the visceral arteries of the abdomen in a skip pattern 6, most commonly affecting the medium size branches of the superior mesenteric artery. The etiology is unknown, but an association with episodes of splanchnic vasoconstriction has been observed (e.g. shock, hypoxia, recent major operation, vasopressor infusion) 5,7. There is some histological similarity to fibromuscular dysplasia, which is a differential diagnosis, but the clinical features and lesion distribution are usually characteristic.
The hallmark of the disorder is multiple abdominal splanchnic artery aneurysms. CT is the modality of choice 6.
Patients presenting with segmental arterial mediolysis typically have CT scans showing mesenteric or intraperitoneal hemorrhage, and CT angiography reveals a range of arteriographic abnormalities of the branches of the visceral arteries 5,6 including:
- fusiform aneurysms
A pattern of aneurysms and stenoses in series is characteristic, the ‘string-of-beads’ appearance. Dissections are typical, and otherwise rare in splanchnic branches, and the distribution tends to spare bifurcations, in contrast to mycotic aneurysms 6.
Treatment and prognosis
Segmental arterial mediolysis is variable in the severity of presenting illness, and conservative therapy may be appropriate. Importantly, immunosupression by steroids or other drugs is thought to be counterproductive, as the arteriopathy is not inflammatory or autoimmune.
For incidentally discovered lesions, the natural history of segmental arterial mediolysis is poorly understood, but most authorities recommend treating aneurysms which have not bled, especially if more than 10 mm in size 8-10.
Significant abdominal hemorrhage often requires urgent treatment. Coil embolization is most frequently advocated, and this is usually straightforward where the affected artery can be sacrificed without causing significant distal ischemia. As usual in the visceral arteries complete exclusion often requires vessels to be occluded both proximal and distal to the bleeding site. Glue embolization has been described 11.
If embolization is not available or contraindicated, surgical exploration, ligation, and resection of affected gut segments and viscera may be necessary.
Cases of segmental arterial mediolysis have been described in which the onset of arteriographic abnormalities is abrupt, and then enter a symptomatic stage, during which hemorrhage carries a significant mortality. After this the arteriopathy has been shown to resolve, to remain in status quo, to fluctuate, and to move from some segments to others. This unpredictable natural history leads most authors to recommend continued surveillance over several years, with prophylactic endovascular treatment for aneurysms which are enlarging 8,9. Generally the prognosis after the presenting episode appears to be good.
Conditions to consider include 12:
- inflammatory arteritides
- different in distribution from SAM, which affects random segments in a ‘skip’ pattern, and does not favor bifurcations
cystic medial necrosis
- rarely affects the visceral segments, and the patient usually has stigmata of Marfan syndrome
- rare in vessels other than the carotid and renal arteries, is not painful, affects a younger age range, and is rarely associated with hemorrhage
- 2. Sakano T, Morita K, Imaki M et-al. Segmental arterial mediolysis studied by repeated angiography. Br J Radiol. 1997;70 (834): 656-8. doi:10.1259/bjr.70.834.9227264 - Pubmed citation
- 3. Slavin RE. Segmental arterial mediolysis: course, sequelae, prognosis, and pathologic-radiologic correlation. Cardiovasc. Pathol. 2009;18 (6): 352-60. doi:10.1016/j.carpath.2008.09.001 - Pubmed citation
- 4. Snow TM, Khorrami N, Strahan A, unpublished observation.
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- 6. Michael M, Widmer U, Wildermuth S et-al. Segmental arterial mediolysis: CTA findings at presentation and follow-up. AJR Am J Roentgenol. 2006;187 (6): 1463-9. doi:10.2214/AJR.05.0281 - Pubmed citation
- 7. Baker-LePain JC, Stone DH, Mattis AN et-al. Clinical diagnosis of segmental arterial mediolysis: differentiation from vasculitis and other mimics. Arthritis Care Res (Hoboken). 2010;62 (11): 1655-60. doi:10.1002/acr.20294 - Free text at pubmed - Pubmed citation
- 8. Ryan J, Suhocki P, Smith T. Journal of Vascular and Interventional Radiology. 2000;11 (7): . doi:10.1016/S1051-0443(07)61802-8
- 9. Davran R, Cinar C, Parildar M et-al. Radiological findings and endovascular management of three cases with segmental arterial mediolysis. Cardiovasc Intervent Radiol. 2010;33 (3): 601-6. doi:10.1007/s00270-009-9651-2 - Pubmed citation
- 10. Hashimoto T, Deguchi J, Endo H et-al. Successful treatment tailored to each splanchnic arterial lesion due to segmental arterial mediolysis (SAM): report of a case. J. Vasc. Surg. 2008;48 (5): 1338-41. doi:10.1016/j.jvs.2008.05.056 - Pubmed citation
- 11. Shimohira M, Ogino H, Sasaki S et-al. Transcatheter arterial embolization for segmental arterial mediolysis. J. Endovasc. Ther. 2008;15 (4): 493-7. doi:10.1583/08-2384.1 - Pubmed citation
- 12. Lie J. Cardiovascular Pathology. 1996;5 (6): . doi:10.1016/S1054-8807(96)00071-3
- 1. Slavin RE, Gonzalez-Vitale JC. Segmental mediolytic arteritis: a clinical pathologic study. Lab. Invest. 1976;35 (1): 23-9. Pubmed citation