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47 yr old man presented to ER with severe, increasing epigastric and periumbilical pain, which was "similar to pain of PUD" that he had 10 yrs ago. The pain was not associated with emesis or alteration in bowel habits.
PE: BS were present. Abdomen was soft, non-distended, with mild epigastric tenderness. Otherwise, PE was unremarkable.
Labs: WNL; Collagen vascular disease serology negative.
EGD, performed on admission (r/o PUD), was unremarkable.
CTA revealed dissection of superior mesenteric artery (SMA) in the absence of aortic dissection (i.e., isolated SMA dissection).
Isolated dissection of SMA. A list of possible aetiologies may include the following conditions:
Patient was initially treated with heparin (anticoagulation) and metoprolol (to retard further dissection by decreasing BP and the arterial pulse wave). Heparin was later switched to warfarin. Abdominal pain subsided. Surgical or endovascular interventions were deferred. Patient was discharged on metoprolol and warfarin in stable condition and pain-free.
Full Diagnosis: Superior mesenteric artery dissection, spontaneous, isolated, idiopathic.
Spontaneous SMA dissections are exceptional events. During a period of 52 years, only 35 cases (including this one) have been reported. Nevertheless, they represent the most frequent type of digestive artery dissection, with those of the hepatic artery, splenic artery, left gastric artery, and celiac artery being even less frequent. Patients with SMA dissections are predominantly male (88%) and have an average age of 55 years (between 45 and 87 years). Whereas atherosclerosis, fibromuscular dysplasia, cystic medial necrosis, and connective tissue disorders (Marfan’s, Elhers-Danlos syndroms) are often mentioned as possible causes, in most cases no cause could be found. Other possible aetiologies of isolated SMA dissection include iatrogenic or blunt trauma and segmental mediolytic arteriopathy (uncommon nonatherosclerotic and nonvasculitic arteriopathy, characterized by lytic degeneration of the arterial media, intramural dissection and thrombosed or ruptured aneurysm; mainly involves the intraabdominal arterial system; however, a few cases of involvement of intracranial arteries have been reported).
Two main clinical symptom patterns are described: (1) acute epigastric or nonspecific abdominal pain syndrome combined with nausea and vomiting and an image of pseudoobstruction on the abdominal plain film results, indicating an acute mesenteric ischemia; and (2) chronic intestinal angina (postprandial pain, vomiting, anorexia, weight loss, epigastric murmur). More exceptionally, cases of hemorrhagic shock have been reported as the result of rupture of the dissecting artery or SMA aneurysm into the peritoneal cavity.
Abdominal ultrasound scan, which is often performed as an emergency procedure for a painful abdominal syndrome, may be helpful for diagnosis when an aneurismal dilatation or the intimal flap is observed. However, flap visualization is inconstant. This examination is more sensitive when associated with a Doppler scan examination. The Doppler scan can be used in the assessment of bowel viability in the operating room to decide as to whether vascular surgery is necessary.
CT angiography allows visualization of the false lumen together with the intimal flap during the arterial phase. CT angiography provides signs of acute ischemic bowel (lack of bowel wall enhancement, mesenteric edema) and gives an estimation of the extent of the lesion. The portal phase gives an indication of the severity of mesenteric ischemia by showing mesenteric or portal venous gas that is often associated with bowel necrosis.
Urgent surgery is recommended when such signs are observed.
However, abdominal plain film, abdominal US, and CT angiography findings are inconstant, and arteriography remains the “gold standard” to confirm the diagnosis.
In most cases, the dissection begins within the first 6 cm from the origin, which remains intact, giving an image either of double lumen and dissection flap or an image of eccentric stenosis.
Treatment options include:
Spontaneous SMA dissections are exceptional events. During a period of 52 years, only 35 cases (including this one) have been reported. Nevertheless, they represent the most frequent type of digestive artery dissection, with those of the hepatic artery, splenic artery, left gastric artery, and celiac artery being even less frequent.
Patients with SMA dissections are predominantly male (88%) and have an average age of 55 years (between 45 and 87 years). Whereas atherosclerosis, fibromuscular dysplasia, cystic medial necrosis, and connective tissue disorders (Marfan’s, Elhers-Danlos syndromes) are often mentioned as possible causes. In most cases no cause could be found. Other possible etiologies of isolated SMA dissection include iatrogenic or blunt trauma and segmental mediolytic arteriopathy.
Segmental mediolytic ateriopathy is an uncommon nonatherosclerotic and nonvasculitic disease, characterized by lytic degeneration of the arterial media, intramural dissection, and thrombosis or ruptured aneurysm and mainly involves the intraabdominal arterial system. However, a few cases of involvement of intracranial arteries have been reported.
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