Ayusha Poudel, MD1, Anurag Adhikari, MD2, Anas Almoghrabi, MD3 1Cook County Health, Chicago, IL; 2NYC Health + Hospitals/Jacobi, New York, NY; 3Cook County Health and Hospital Systems, Chicago, IL
Introduction: The Superior Mesenteric Artery (SMA) is defined as the compression of the third part of the duodenum between the SMA and the aorta. Nutcracker syndrome is defined as the compression of the left renal vein between the aorta and superior mesenteric artery. Their coexistence is rare and diagnosis is challenging due to vague and overlapping syndrome.
Case Description/Methods: A 64-year-old male with a history of diabetes mellitus, major depressive disorder, seronegative spondyloarthropathy, severe protein energy malnutrition, and a recent diagnosis of duodenal neuroendocrine tumor (NET) presented with complaints of substernal non-exertional chest pain and dyspnea. He had a significant weight loss due to loss of appetite, and frequent vomiting with coffee-ground emesis. He had been having weakness in his lower extremities for the past six months and was wheelchair-bound. Physical examination revealed cachexia and tachycardia.
He had elevated liver enzymes and red blood cells in his urine. X-ray evaluation demonstrated a compressed third portion of the duodenum with proximal dilation. Esophagogastroduodenoscopy noted an extrinsic compression at the junction between the third and fourth portions of the duodenum, esophagitis and multiple small duodenal ulcers but an enteroscopy showed no strictures or mass. Computed tomography (CT) detected the aortomesenteric angle to be < 20° and SMA was diagnosed. The upper pole of the left kidney had an infarct with anatomy consistent with nutcracker syndrome.
He underwent laparoscopic duodenojejunostomy which was complicated by hypotension with sustained ventricular tachycardia and he passed away.
Discussion: SMA syndrome with a prevalence of 0.1-0.3% prevalence poses a mortality risk from recurrent vomiting and aspiration pneumonia which makes early diagnosis essential. Weight loss exacerbates the symptoms and perpetuates a vicious cycle. CT scans and magnetic resonance imaging facilitate the diagnosis with gastric decompression and duodenojejunostomy yielding an 80-90% success rate. Our patient also had a concurrent nutcracker syndrome. This also manifests as nausea and loss of appetite. While conservative management is recommended for minimal symptoms, surgery including renal auto-transplantation is indicated for intractable pain.
The coexistence of SMA and nutcracker syndrome is possible due to similar anatomic pathology but is rare. The diagnosis is difficult due to vague and overlapping symptoms though imaging can aid in diagnosis.
Figure: Figure 1: 1a Compression of the duodenum between superior mesenteric artery and aorta with massive stomach and duodenal dilation;1b: Sagittal sectional showing left renal vein compression by superior mesenteric artery (SMA) with SMA angle<20 degrees 1c: Coronal plane demonstrating renal vein compression (nutcracker syndrome)
Disclosures:
Ayusha Poudel indicated no relevant financial relationships.
Anurag Adhikari indicated no relevant financial relationships.
Anas Almoghrabi indicated no relevant financial relationships.
Ayusha Poudel, MD1, Anurag Adhikari, MD2, Anas Almoghrabi, MD3. P1570 - Coexistence of Superior Mesenteric Artery Syndrome With Nutcracker Syndrome: A Rare Combination, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.