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Superior Mesenteric Artery Syndrome: a Prospective Study in a Single Institution

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Superior Mesenteric Artery Syndrome: a Prospective Study in a Single Institution Journal of Gastrointestinal Surgery (2019) 23:997–1005 https://doi.org/10.1007/s11605-018-3984-6 ORIGINAL ARTICLE Superior Mesenteric Artery Syndrome: a Prospective Study in a Single Institution Angelica Ganss1 & Sabrina Rampado1 & Edoardo Savarino2 & Romeo Bardini1 Received: 19 May 2018 /Accepted: 17 September 2018 /Published online: 5 October 2018 # 2018 The Society for Surgery of the Alimentary Tract Abstract Background Superior mesenteric artery syndrome (SMAS) is a rare cause of duodenal obstruction, resulting from the compres- sion of the duodenum between superior mesenteric artery and aorta. This prospective registry aims to describe demographic, clinical, and outcome features of patients suffering from SMAS and to point out the indications for surgery. Methods Between 2008 and 2016, patients with chronic gastrointestinal symptoms and diagnosis of SMAS were included. Demographics, clinical presentation, diagnosis, and surgical outcome were recorded. Symptoms were investigated with a standardized questionnaire. The diagnosis was achieved through barium swallow, CT/MR angiography (aortomesenteric angle ≤ 22°, distance ≤ 8 mm), endoscopy. All patients underwent duodenojejunostomy ± distal duodenum resection. At follow-up, symptom score and barium swallow were re-evaluated. Results Thirty-nine patients (11 M/28 F, median age 38 years, median BMI 17.8 kg/m2) were included. Barium swallow showed a gastroduodenal dilation in 57% of patients, and a delayed gastroduodenal emptying in 38%. Median aortomesenteric angle was 11° and distance was 5 mm. All patients underwent duodenojejunostomy, and in 32 patients, a distal duodenum resection was also performed. At a median follow-up of 47 months, the overall symptom score significantly dropped (10 vs. 32, p < 0.0001) and BMI increased (19.5 vs. 17.8, p < 0.0001). Barium swallow at 2 months postoperatively showed an improvement in terms of gastroduodenal dilation and emptying in 38% of patients with preoperative pathological findings. Conclusions SMAS is a rare condition that should be suspected in cases of chronic, refractory upper digestive symptoms, particularly in females with low BMIs. Surgical treatment may improve symptoms and quality of life, although it is not curative in all cases. ClinicalTrials.gov Identifier: NCT03416647 Keywords Superior mesenteric artery syndrome . Chronic duodenal obstruction . Duodenojejunostomy . Bile reflux Introduction teric artery and the aorta. Over the years, it has been described by various names, i.e., Cast Syndrome, Wilkie’ssyndrome, Superior mesenteric artery syndrome (SMAS) is a rare cause of arteriomesenteric duodenal compression, and duodenal vascu- chronic duodenal obstruction, resulting from the compression lar compression. These multiple terminologies have made it of the third portion of the duodenum between superior mesen- difficult to estimate the true frequency in the general population. The prevalence has been estimated at around 0.0024–0.3%, and the syndrome has been diagnosed more often in women in early * Angelica Ganss adulthood.1–3 Risk factors include significant weight loss (eat- [email protected] ing disorders, burn injury, bariatric surgery), abdominal or spi- nal surgery (proctocolectomy with ileoanal pouch for ulcerative 1 Department of Surgery, Oncology and Gastroenterology, General colitis, spinal elongation for the treatment of scoliosis) and an- Surgery Unit, School of Medicine, University of Padua, Padua, Italy atomic and congenital abnormalities (intestinal malrotation, low 2 Department of Surgery, Oncology and Gastroenterology, origin of the superior mesenteric artery, high insertion of the Gastroenterology Unit, School of Medicine, University of Padua, ligament of Treitz).4–8 However,accordingtoAkinetal.,upto Padua, Italy 40.4% of SMAS cases have no apparent cause.9 998 J Gastrointest Surg (2019) 23:997–1005 Clinical presentation consists of chronic upper gastrointes- Thirty-nine enrolled patients underwent duodenal sur- tinal symptoms, such as postprandial epigastric pain, nausea, gery due to clinical and radiological criteria. They present- vomiting, dyspepsia, abdominal bloating and weight loss sec- ed with one or more of the following clinical features: (a) ondary to decreased oral intake. A specific symptom score for severe and frequent upper digestive symptoms (occurring patients with SMAS has not yet been developed. The differ- at least once a week), associated with poor quality of life ential diagnosis includes motility disorders, gastroesophageal and refractory response to medical treatment; (b) a condi- reflux disease, pancreatitis, peptic ulcer disease, biliary colic, tion of underweight (BMI < 18.5 kg/m2) associated with and mesenteric ischemia.3,10 Given the poor specificity of the difficulty eating; (c) severe complications of SMAS (e.g., symptoms reported, the diagnosis remains challenging and is gastric perforation, acute pancreatitis, aspiration pneumo- often rendered by excluding competing diagnoses. nia). In all patients, the following features were recognized Indeed, once the syndrome is suspected clinically, it must at imaging studies: (a) suggestive findings of SMAS at be confirmed radiologically by a barium swallow and a CT barium swallow; (b) diagnostic aortomesenteric angle and and/or MR angiography that represent the Bgold standard^. distance at CT/MR angiography. Esophagogastroduodenoscopy (EGD) can also provide addi- This study was performed according to the Declaration of tional information, such as the presence of esophagitis and/or Helsinki Principles. It was reviewed and approved by the gastritis caused by duodenogastric reflux. EGD may also help Internal Review Board in Padua, Italy. Written consent was exclude differential diagnoses. obtained from all patients prior to study enrolment. Therapeutic options for SMAS range from conservative management – including gastroduodenal decompression, cor- rection of electrolyte imbalances and adequate nutritional Preoperative Assessment support11—and surgical strategies to bypass or remove the obstruction. Surgery is recommended only when initial con- The following demographic and clinical data were prospec- servative treatment fails, or in severe cases.12 tively collected: age, weight, height, body mass index (BMI), We prospectively investigated 39 patients with a history of symptom duration, past and current medical therapy, and pre- chronic refractory upper gastrointestinal disorders, who vious surgical and endoscopic procedures. underwent surgical correction for SMAS, in order to discuss A symptom score was collected before and after surgery. clinical presentation, diagnostic workup and surgical outcome Symptoms were assessed at baseline and during follow-up by at a long-term follow-up. The following knowledge gaps were using a detailed Lickert-scale based questionnaire for epigas- investigated and addressed: (a) a standardized diagnostic tric pain, nausea, vomiting, abdominal bloating and regurgita- workup for superior mesenteric artery syndrome; (b) a specific tion. For each symptom, a score was calculated by combining symptom score, in order to standardize the evaluation of pre/ severity with frequency, as shown in Table 1. The total symp- postoperative symptoms; (c) the duration of conservative tom score was obtained from the sum of the scores of all the treatment before considering surgical correction of the syn- symptoms, a maximum value of 55, and a minimum value of drome; (d) thorough indications for surgery; (e) a theoretical 25 was judged necessary to suspect a diagnosis of SMAS. comparison between different surgical techniques; (f) a pre- The clinical diagnosis required confirmation by radiologic cise knowledge of long-term outcomes after surgical correc- investigations, such as barium swallow, CT and/or MR angi- tion of SMAS. ography with multiplanar 3-dimensional reconstructions, and EGD.13 Typicalfindingsatbariumswallowaregastrectasy, proximal duodenal dilation, an abrupt vertical or oblique com- pression of the third portion of the duodenum, duodenogastric Patients and Methods reflux, and a delayed transit (Fig. 1). Radiological criteria at CT/MR angiography are a reduction of the aortomesenteric Between October 2008 and March 2016, we prospectively angle to 22° or less (normal 25°–60°) and a decrease of the screened 254 consecutive patients with aspecific upper diges- aortomesenteric distance to 8 mm or less (normal 10– tive symptoms, lasting from at least 5 months, who were re- 28 mm).14 The aortomesenteric distance was defined as the ferred to the Department of Surgery, Oncology and maximum distance at the level where the duodenum passes Gastroenterology at the University-Hospital of Padua (Italy), between the vessels (Fig. 2a); the aortomesenteric angle was a referral center for complex upper gastrointestinal surgery. measured at the same level on sagittal images (Fig. 2b). Both We excluded 215 (85%) patients based on the following values were blindly reviewed and calculated by two expert criteria: inability to provide the informed consent (2.8%); ma- radiologists in order to standardize the method. EGD cannot lignancies (7.4%); bowel motility disorders (57.2%); severe rule out the diagnosis, but it can show the presence of bile psychiatric illness (9.3%); pregnancy (2.3%); impossibility to reflux, suggestive for duodenal obstruction, and exclude ma- perform the required diagnostic workup (20.9%). lignant diseases. J Gastrointest Surg (2019) 23:997–1005 999 Table 1 Symptom score. The following questionnaire
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