Superior mesenteric artery (SMA) syndrome (also known as Wilkie's syndrome, cast syndrome, or aorto-mesenteric compass syndrome) is an obstruction of the duodenum caused by extrinsic compression between the SMA and the aorta. The median age of patients is 23 years old (range 0-91 years old) and predominant in females over males with a ratio of 3:2. The symptoms are variable, consisting of postprandial abdominal pain, nausea and vomiting, early satiety, anorexia, and weight loss and can mimic anorexia nervosa or functional dyspepsia. Because recurrent vomiting leads to aspiration pneumonia or respiratory depression via metabolic alkalosis, early diagnosis is required. The useful diagnostic modalities are computed tomography as a standard tool and ultrasonography, which has advantages in safety and capability of real-time assessments of SMA mobility and duodenum passage. The initial treatment is usually conservative, including postural change, gastroduodenal decompression, and nutrient management (success rates: 70%-80%). If conservative therapy fails, surgical treatment (i.e., laparoscopic duodenojejunostomy) is recommended (success rates: 80%-100%).

The duodenum and the left renal vein occupy the vascular angle made by the superior mesenteric artery and the aorta. When the angle becomes too acute, compression of either structure can occur. Each type of compression is associated with specific clinical symptoms that constitute a rare disorder. If clinical symptoms are mild, conservative treatment is implemented. However, surgery is often the only solution that can improve quality of life and/or avoid life-threatening complications. This report describes a case of a patient with both types of aortomesenteric compression that required two separate surgeries to alleviate all symptoms.

A 20-year-old white woman presented to the Emergency Room complaining of sudden onset severe left flank and lower left quadrant abdominal pain, nausea, and vomiting. A clinical work-up revealed elevated white blood cells and hematuria. She was discharged with a diagnosis of urinary tract infection. Symptoms continued to worsen over the subsequent 2 months. Repeated and extensive clinical work-ups failed to suggest evidence of serious pathology. Ultimately, an endoscopy revealed obstruction of her duodenum, and barium swallow identified compression by the superior mesenteric artery, leading to the diagnosis of superior mesenteric artery syndrome. She underwent a Roux-en-Y duodenojejunostomy. Six weeks later she continued to have severe left-sided pain and intermittent hematuria. Venography revealed compression of the left renal vein, extensive pelvic varices, and significant engorgement of her left ovarian vein. A diagnosis of nutcracker syndrome was made and a left renal vein transposition was performed. Significant improvement was seen after 8 weeks.

The disorders associated with aortomesenteric compression can lead to serious symptoms and sometimes death. Diagnosis is challenging not only because of the lack of awareness of these rare disorders, but also because they are associated with symptoms that are similar to those seen in less serious diseases. Guidance for health care professionals with respect to relevant radiological and clinical markers needs to be reconsidered in order to clarify the etiology of the diseases and create better diagnostic protocols.

Superior mesenteric artery (SMA) syndrome is an uncommon and potentially fatal cause of small bowel obstruction where the third portion of the duodenum is compressed between the abdominal aorta and the superior mesenteric artery. This is most frequently seen after sudden and significant weight loss, but other etiologies can also cause this duodenal compression. This syndrome can lead to food aversion, poor intake, and weight loss that exacerbate symptoms in a vicious cycle. SMA syndrome is often a diagnosis of exclusion due to nonspecific symptoms, including abdominal pain and distention, feelings of fullness after meals, and bilious emesis. Diagnosis may be assisted with radiography, tomography, endoscopy, and ultrasound imaging. Once SMA syndrome is identified, treatment is directed toward symptom management and nutritional support. If conservative measures fail, symptoms are severe, or the duodenum is compromised, several effective surgical procedures are routinely considered. This article provides an overview of SMA syndrome including history, pathophysiology, signs and symptoms, diagnostic testing, medical and surgical treatment, and implications for nursing staff.

Superior mesenteric artery syndrome (SMAS) has an incidence of 0.1-0.3%. It is caused by a narrow angle between the aorta and the superior mesenteric artery, resulting in the clamping of the third portion of the duodenum. It is more common in females (2:1), with no racial differences. It is also more frequent in patients under 30 years and may have a congenital or acquired aetiology. There is only one case of the familial form described in the medical literature. We present a case of a 20-year-old woman, with recurrent episodes of epigastric discomfort and vomiting since she was 10 years old. After clinical suspicion, diagnosis was achieved after digestive endoscopy, upper gastrointestinal transit and abdominal MR. The patient was kept under parenteral nutrition for 17 days before surgery. She was discharged 8 days after surgery and the postoperative course was uneventful.

Background Superior mesenteric artery syndrome (SMAS) results from the compression of the third part of the duodenum between the aorta and the proximal part of the superior mesenteric artery (SMA). Clinical presentation of SMAS is characterized by the dilatation of the proximal part of the third part of the duodenum. SMAS is a rare cause of the upper gastrointestinal system (UGS) obstruction. In this study, we aimed to present our clinical experience in the treatment of five patients with SMAS, which is a rare clinical condition requiring surgery. Patients and methods The retrospective study included five patients who were treated due to SMAS at our clinic between January 2010 and January 2014. Results All the patients were underweight, with a mean BMI of 15.73 (14-16). The clinical symptoms included epigastric pain after food intake, large volume bilious emesis, early satiety, failure to gain weight, indigestion, esophageal reflux, sense of fullness, and persistent weight loss. SMAS was diagnosed using barium meal studies, upper gastrointestinal endoscopy, abdominal ultrasonography, and CT angiography. Four patients underwent duodenojejunostomy and one patient was managed with gastrojejunostomy. No complication was observed during the postoperative period, and all the patients achieved significant improvement in symptoms. Conclusion SMAS is a rare cause of UGS obstruction, and the diagnosis of SMAS is often delayed. SMAS should be suspected in the differential diagnosis of the patients with unsubstantiated symptoms of persistent nausea, emesis, and significant weight loss.

The superior mesenteric artery (SMA) syndrome is a rare but potentially life-threatening gastrointestinal condition. Over the years, it has been referenced by several names, the most common of which is Wilkie's syndrome. These numerous terminologies have made it difficult to estimate its true frequency in the general population. Common symptoms associated with this syndrome include intermittent postprandial abdominal pain, nausea, and bilious vomiting. Our review revealed that although it is currently well-defined in the literature, the diagnosis of SMA syndrome remains challenging as other disorders can mimic its presentation. However, CT angiography is currently favored in the literature for diagnosis as it can not only show the narrowed aorto-mesenteric angle and distance, but also the extent of duodenal obstruction. In addition, we found no consensus on the preferred mode of therapy once SMA syndrome is diagnosed. The agreement among authors is that the treatment options should be based on severity of the disease, using conservative measures as the first line of therapy in mild SMA syndrome. Duodenojejunostomy is the preferred surgical approach when conservative management fails, or in severe cases.

Celiac artery compression syndrome (CACS) and superior mesenteric artery syndrome (SMAS) are 2 rare diseases, widely described in literature. Their association has not been specifically investigated; in fact, few cases have been reported. For this reason we reviewed our experience from January 1974 to June 2004. We report 59 patients affected by CACS and 28 by SMAS. Coexistence of both syndromes in 8 patients was observed. These 8 patients were successfully treated with duodenojejunal bypass and decompression of the celiac trunk. In this paper, we analyze the pathogenesis, clinical presentation, diagnosis, and treatment of these syndromes, emphasizing their common aspects. The misdiagnosis of this association may justify in some cases the controversial results reported regarding the surgical treatment of these syndrome.

A case of newborn with incomplete duodenal obstruction caused by superior mesenteric artery syndrome has been presented with this report. A full term, 1-day-old baby girl was referred to our hospital because of recurrent bilious vomiting since birth and upper gastrointestinal barium study revealed the incomplete obstruction at the 3rd part of the duodenum with a vertical abrupt cutoff. The diagnosis of superior mesenteric artery syndrome was made with ultrasonography and duodenojejunostomy was carried out. Although it is extremely rare, superior mesenteric artery syndrome should also be considered as one of the rare cause of incomplete duodenal obstruction in newborn period.

Duodenal obstruction by Superior Mesenteric Artery (SMA) is a misdiagnosed vomiting syndrome in children. Several factors are involved, including rapid weight loss, rapid statural growth without weight augmentation. Diagnosis is suspected when an improvement is achieved by ventral decubitus and it is confirmed by plain films of the abdomen, GI study with barium and echography, measuring the aortomesenteric angle (inferior to 25-30 degrees ). Patients must at first be treated conservatively. Surgery is indicated for occlusive episodes with unsuccessful conservative therapy. The authors report 3 cases with different clinical presentation. However, all the patients presented important weight loss and vomiting.

Two cases of gastroduodenal outlet obstruction caused by arteriomesenteric compression in children who have cerebral palsy are reported. Clinical symptoms of gastrointestinal obstruction include recurrent postprandial nausea and vomiting, upper abdominal distension, and pain. In such patients, multiple predisposing factors can contribute to the development of arteriomesenteric compression, including marked weight loss, supine position, and severe scoliosis. Upper gastrointestinal x-rays using barium contrast allow diagnostic confirmation. In our experience, this cause of acute gastroduodenal outlet obstruction may usually resolve after conservative treatment using a jejunal feeding tube passed beyond the compression, left lateral positioning, and renutrition.

Early diagnosis of superior mesenteric artery syndrome, less frequent in children than in young adults, is improved by the use of ultrasonography and CT scan.

An eleven year-old boy was admitted because he suffered from bilious vomiting for 24 hours. A diagnosis of superior mesenteric artery syndrome was made from the results of plain films of the abdomen, ultrasonography and roentgenograms after instillation of dilute barium solution. Diameter of the left renal vein appeared increased. The patient was given fractionated meals and placed in left lateral position. The CT scan performed 5 days later confirmed the diagnosis but did not show dilatation of the left renal vein. The patient is well one year later without treatment. Ultrasonography performed 3 hours after a meal showed similar findings to those seen in the acute phase.

Abdominal scanning and ultrasound are valuable tools for diagnosis of such a syndrome. The diagnostic value of dilatation of the left renal vein remains to be determined.