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A man with the rare simultaneous combination of three abdominal vascular compression syndromes: median arcuate ligament syndrome, superior mesenteric artery syndrome, and nutcracker syndrome.

ABSTRACT: Median arcuate ligament syndrome and superior mesenteric artery syndrome are well-known abdominal compression syndromes, the coexistence of which is rarely described in literature. In addition, due to the common pathogenesis, anterior nutcracker syndrome may occur simultaneously to superior mesenteric artery syndrome. To our knowledge, this is the first case reporting combination of these 3 syndromes detected with ultrasound, Computed Tomography and upper gastrointestinal fluoroscopic exam. A 69-year-old man came to our attention for rapid weight loss, postprandial epigastric pain and recurrent vomiting for at least 6 months. Doppler ultrasound showed both celiac artery and left renal vein stenosis with simultaneous left varicocele. Computed tomography showed a reduction of aortomesenteric space causing both left renal vein and duodenal stenosis, this latter confirmed by upper gastrointestinal fluoroscopic exam. The diagnosis of these three vascular compression syndromes (MALS, SMAS, and anterior NCS) has been formulated, based on clinical and imaging findings. We assumed that the postprandial crises caused by median arcuate ligament syndrome may induce a reduction of meals consumption and progressive weight loss which can be a cause of anterior nutcracker syndrome and superior mesenteric artery syndrome onset. Doppler ultrasound, in expert hands, allows to accurately diagnosing these syndromes which are often underestimated. Failure to recognize it and inadequate treatment could have serious consequences for patients' health.

SUBMITTER: Farina R

PROVIDER: S-EPMC8026914 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:OBJECTIVE:Optimal diagnosis and management of median arcuate ligament (MAL) syndrome (MALS) remains unclear in contemporary practice. The advent and evolution of laparoscopic and endovascular techniques has redirected management toward a less invasive therapeutic algorithm. This study examined our contemporary outcomes of patients treated for MALS. METHODS:All patients treated for MALS at Dartmouth-Hitchcock Medical Center from 2000 to 2013 were retrospectively reviewed. Demographics and comorbidities were recorded. Freedom from symptoms and freedom from reintervention were the primary end points. Return to work or school was assessed. Follow-up by clinic visits and telephone allowed quantitative comparisons among the patients. RESULTS:During the study interval, 21 patients (24% male), with a median age of 42 years, were treated for MALS. All patients complained of abdominal pain in the presence of a celiac stenosis, 16 (76%) also reported weight loss at the time of presentation, and 57% had a concomitant psychiatric history. Diagnostic imaging most commonly used included duplex ultrasound (81%), computed tomography angiography (66%), angiography (57%), and magnetic resonance angiography (5%). Fourteen patients (67%) underwent multiple diagnostic studies. All patients underwent initial laparoscopic MAL release. Seven patients (33%) underwent subsequent celiac stent placement in the setting of recurrent or unresolved symptoms with persistent celiac stenosis at a mean interval of 49 days. Two patients required surgical bypass after an endovascular intervention failed. The 6-month freedom from symptoms was 75% and freedom from reintervention was 64%. Eighteen patients (81%) reported early symptom improvement and weight gain, and 66% were able to return to work. CONCLUSIONS:A multidisciplinary treatment approach using initial laparoscopic release and subsequent stent placement and bypass surgery provides symptom improvement in most patients treated for MALS. The potential placebo effect, however, remains uncertain. A significant minority of patients will require reintervention, justifying longitudinal surveillance and prudent patient selection. Patients can anticipate functional recovery, weight gain, and return to work with treatment.

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A man with the rare simultaneous combination of three abdominal vascular compression syndromes: median arcuate ligament syndrome, superior mesenteric artery syndrome, and nutcracker syndrome.

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