Clinical Presentation, Imaging, and Management of Segmental Arterial Mediolysis: A Rare Vascular Disorder

Objectives Our objective was to analyze the clinical presentation, imaging findings, and the management of segmental arterial mediolysis (SAM) in different case scenarios within our medical institution. Materials and Methods We retrospectively analyzed 13 cases of SAM in our institution from July 2017 to March 2020. The images from the cases were collected from picture archiving and communication system (PACS) along with other pertinent clinical information from the hospital's information system. All the patients we studied underwent contrast-enhanced computed tomography (CT) using a third-generation Siemens SOMATOM Force dual-source CT scanner. Once the dual-phase scanning was completed, the images were analyzed using the workstation's syngo.via software. Results Three out of the 13 cases required stent-grafting of the renal/celiac artery, and the involved branch of the superior mesenteric artery was embolized in one case. The rest of the cases were managed conservatively with antiplatelets/anticoagulants. Subsequent follow-ups of the patients were conducted and showed stabilization/regression of the initial findings without finding any evidence of worsening. Conclusion SAM should be considered when making a differential diagnosis of acute abdominal pain when associated with dissection or aneurysms in splanchnic arteries, and in cases of unexplained intra-abdominal hemorrhaging. The radiologist needs to be aware of this possibility to raise suspicion, alert the clinician, and guide appropriate management.

Segmental Arterial Mediolysis: A Multiguised Vasospastic Arteriopathy with Collateral Mesangial Cell Hyperplasia and Cardiac Toxicity Generated by Norepinephrine and Hyperdense Adrenoceptors Alone or by Crosstalk with Other Pressor Agents

Segmental arterial mediolysis (SAM), an uncommon vasospastic arteriopathy occurring in the muscular arteries innervated by the peripheral sympathetic nervous system, usually presents with catastrophic abdominal and retroperitoneal hemorrhages in elderly patients. SAM is initiated by the coupling of norepinephrine to plastically derived hyperdense foci of alpha-1 adrenergic receptors on the sarcolemma of arterial muscle. This ligand is created by stimuli signaled by iatrogenic sympathomimetic agonists, some beta-2 agonists, or an excessive release of adrenal catecholamines. Coupling of this ligand with cytoplasmic heterotrimeric Gq protein excessively signals a cascade of biochemical events generating two principal lesions of injurious-phase SAM—the shearing of the outer media from the adventitia and an overload of cytoplasmic calcium ions toxic to mitochondria causing mediolysis and/or apoptosis. The massive hemorrhages are caused by ruptured gap aneurysms created by the transmedial loss of the medial muscle. A norepinephrine-directed reparative response rapidly develops either resolving angiographic injurious lesions or creating a body of vascular disorders, the new guises of SAM with ischemic clinical profiles. These present in the epicardial, vertebral, intestinal, and retroperitoneal arteries, often in younger females as fibromuscular dysplasia, dissecting hematomas, and persistent aneurysms. Norepinephrine can crosstalk with other pressor agents to create SAM lesions—serotonin with idiopathic pulmonary hypertension and persistent pulmonary hypertension in the newborn, histamine in spontaneous coronary artery dissections with eosinophilia, and endothelin-1 in a field effect generated by SAM that creates venous fibromuscular dysplasia. Norepinephrine also participates in the collateral development of mesangial hyperplasia with focal segmental glomerulosclerosis and myocardial mediolysis and apoptosis in subjects with markedly elevated heart rates. Conclusion. Norepinephrine coupling with plastically elevated alpha-1 adrenoceptor or other pressor agents generates SAM, a histologically recognizable vasospastic arteriopathy, that with repair is transformed into several different standardized arterial diseases that alter SAM’s clinical profile from a hemorrhagic to an ischemic disorder.

A case of segmental arterial mediolysis with subarachnoid hemorrhage due to anterior cerebral artery dissection followed by internal carotid artery dissection

Background: Segmental arterial mediolysis (SAM) causes subarachnoid hemorrhage (SAH) due to intracranial aneurysm rupture and arterial dissection. We encountered a case of SAM-related SAH due to ruptured dissection of the A1 segment of the anterior cerebral artery concomitant with internal carotid artery (ICA) dissection. Case Description: A 53-year-old man presented with SAH due to a ruptured right A1 dissecting aneurysm. The aneurysm was trapped; however, 7 days after the onset of SAH, he experienced right hemiparesis and aphasia. Angiography showed left ICA dissection; urgent carotid artery stenting was performed, leading to symptom improvement. Abdominal computed tomography angiography showed aneurysms of the celiac and superior mesenteric arteries. He was diagnosed with SAM based on clinical, imaging, and laboratory findings. Conclusion: In the acute phase of SAM-related SAH, cerebral ischemia could occur due to both cerebral vasospasm and intracranial or cervical artery dissection.