S2819 Evaluating All Angles: Wilkie's Syndrome—A Rare Cause of Intestinal Obstruction

Abstract

INTRODUCTION: Superior mesenteric artery (SMA) syndrome is a rare cause of duodenal obstruction. The prevalence of SMA syndrome is estimated at less than 0.3%. Normally, the angle between the SMA and aorta is 38-65 degrees maintained by mesenteric fat padding. The mechanism of intestinal obstruction is due to exaggerated acute angularity of the SMA caused by excessive fat loss, leading to compression of the third part of the duodenum as it travels between the SMA and aorta. There are multiple predisposing factors that can increase the angularity, such as anorexia nervosa, kyphoscoliosis, and bariatric surgery. However, there are limited reports of SMA syndrome secondary weight loss exacerbated by chemotherapy. CASE DESCRIPTION/METHODS: We present a 22-year old female with a history of aplastic anemia, post allogenic bone-marrow transplant with cyclophosphamide/fludarabine/anti-thymocyte globulin conditioning therapy, who presented with nausea and vomiting for 4 days and a 9 kg loss in weight since conditioning therapy. Physical examination revealed a severely dehydrated patient with peri-umbilical tenderness. The patient had presented with similar symptoms 1 month prior, with a normal EGD, and resolution of symptoms with supportive care. On readmission CT-Abdomen revealed a massively distended stomach, first, and second portions of the duodenum, and complete collapse of the duodenum at the level of the SMA. CT-Angiography revealed acute angulation of the SMA-aortic takeoff at 20 degrees, establishing the diagnosis of SMA syndrome. The patient underwent nasogastric decompression and laparoscopic duodenojejunostomy resulting in a successful bypass. DISCUSSION: Physicians should be wary of this rare etiology of duodenal obstruction and its clinical presentation. A high index of suspicion should be employed for young patients presenting with signs and symptoms of intestinal obstruction with a history of weight loss. Delayed diagnosis can lead to significant morbidity, due to electrolyte abnormalities or perforation. CT-Angiography is the imaging modality of choice to establish the diagnosis. However, the diagnosis may be overlooked, as radiological findings do not always correlate with clinical findings. Additionally, some patients may experience intermittent symptomatic compression of the duodenum, leading to a delay in diagnosis, as illustrated with our patient. Depending on initial management, it is recommended that once symptom free and patency of the duodenum is achieved, patients begin a dietary regimen.Figure 1.: 3D Reconstructed CT-Angiography revealing severely distended stomach, first, and second portion of the duodenum.Figure 2.: CT Angiography revealing severe acute angularity of the SMA-aortic takeoff. Black arrow—Celiac artery, Maroon arrow—Aorta, Blue arrow—Superior Mesenteric Artery.