Median arcuate ligament compression syndrome in monozygotic twins,☆☆,

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Abstract

Twin 27-year-old women had symptomatic mesenteric ischemia caused by median arcuate ligament compression. Arteriography demonstrated severe celiac artery stenosis in one twin, celiac artery occlusion in the other, and proximal superior mesenteric artery narrowing with retrograde filling from a meandering mesenteric artery in both. Division of the ligament and direct celiac artery revascularization completely relieved symptoms in both patients. Median arcuate ligament compression of the celiac and superior mesenteric arteries can result in mesenteric ischemia. Documentation of this unusual syndrome in monozygotic twins suggests that the responsible anatomic relationships are congenital. (J VASC SURG 1994;19:934-8.)

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Case 1

A 27-year-old white woman was admitted with a progressive 3-month history of postprandial abdominal cramping, bloating, and occasional nausea and vomiting. An 8-pound weight loss was noted. Medical history was insignificant; there was no previous abdominal surgery, nor were there prior gastrointestinal complaints. She took no medications and did not smoke. There was no history of psychiatric illness. The physical examination result was unremarkable except for an epigastric bruit, which did not

DISCUSSION

Since its first descriptions in the early 1960s, 1 controversy has surrounded the diagnosis of MAL compression syndrome, also known as CA compression syndrome. Its existence has been questioned on several grounds: theoretically, that isolated obstruction of the CA from any cause should not result in visceral ischemia because of the presence of collateral channels; epidemiologically, that CA narrowing from extrinsic compression is a common arteriographic finding in asymptomatic individuals 2;

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From the Departments of Surgery (Drs. Bech and Gewertz), Radiology (Drs. Loesberg and Rosenblum), and Pathology (Dr. Glagov), Pritzker School of Medicine, The University of Chicago.

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Reprint requests: Bruce L. Gewertz, MD, Department of Surgery, The University of Chicago, MC 5029, 5841 Maryland Ave., Chicago, IL 60637.

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