A Delayed Presentation of Superior Mesenteric Artery Syndrome Treated With Robotic-Assisted Strong Procedure.

Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, is a rare condition resulting from compression of the third portion of the duodenum between the aorta and the superior mesenteric artery. When symptomatic, this compression may result in nausea, vomiting, epigastric discomfort, and weight loss, requiring clinical attention and imaging to make the diagnosis. Typically, SMA syndrome presents in young females and is associated with an underlying condition such as anorexia nervosa, cachexia, postoperative development after scoliosis surgery, etc. In this report, we present the case of an atypical delayed presentation of SMA syndrome in an 84-year-old male who had epigastric pain, nausea, intermittent vomiting, and a 30-pound weight loss over two years. SMA syndrome was diagnosed on a computed tomography (CT) scan by a decreased angle between the superior mesenteric artery and abdominal aorta, and treatment with a robotic-assisted strong procedure was performed. The patient was followed postoperatively in the clinic and tolerated the procedure well.

Rare Superior Mesenteric Artery Syndrome in an Eight-Year-Old Girl With Henoch-Schönlein Purpura: A Case Report.

Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by palpable purpura, arthralgia or arthritis, GI symptoms, and renal involvement. Superior mesenteric artery (SMA) syndrome, a rare condition, occurs when the third part of the duodenum is compressed between the aorta and the SMA, leading to upper intestinal obstruction. This case report describes the clinical presentation, diagnostic process, and management of an eight-year-old girl with HSP complicated by SMA syndrome. The patient initially presented with abdominal pain and vomiting, eventually developing the characteristic rash of HSP. While initial management was supportive, her condition deteriorated. Treatment with intravenous methylprednisolone resulted in significant symptom improvement and resolution of both SMA syndrome and HSP manifestations. This case highlights the need to recognize SMA syndrome as a potential complication of HSP and demonstrates the effectiveness of steroid therapy in managing this condition. Further research is needed to develop comprehensive treatment guidelines for HSP patients with SMA syndrome.

Wilkie's syndrome in a patient with Duchenne's muscular dystrophy: A Case Report.

Superior mesenteric artery syndrome, or Wilkie's syndrome, is one of the rarest gastrointestinal disorders known to medical science. It is characterized by the vascular clamp of the third portion of the duodenum, between the superior mesenteric artery and the aorta. It presents as an uncommon cause of upper intestinal obstruction. Imaging is required, preferably with a contrast-enhanced CT or an MRI; conservative management is preferred, leaving surgery for the most complex cases. We present the case of a 34-year-old man with Duchenne's muscular dystrophy and a history of substantial weight loss after hospital admission for aspiration pneumonia. He underwent an abdominal CT scan that showed enlargement of the stomach, the second and third parts of the duodenum; without observing masses, the patient received conservative management with a nasojejunal feeding tube. At the outpatient reevaluation, 1 month postdischarge, he became asymptomatic and had progressive weight gain.

Concurrent Encounter of Superior Mesenteric Artery Syndrome and Nutcracker Syndrome in a Young Female Patient: A Case Report and Literature Review.

Superior mesenteric artery (SMA) syndrome causes duodenal obstruction between the SMA and aorta, which culminates into bowel obstruction. Meanwhile, nutcracker syndrome (NCS) involves left renal vein compression between the aorta and SMA, categorized by the compression site. We present a 15-year-old female with no prior medical or surgical history who displayed early signs of the rarely coexisting SMA and nutcracker phenomena, which were managed symptomatically along with nutritional support to reach her optimal body mass index.

The superior mesenteric artery angle in diagnosis of nutcracker syndrome: a systematic review and meta-analysis.

PURPOSE: Nutcracker syndrome (NCS) can be caused by narrowness of the superior mesenteric artery (SMA) angle. Nevertheless, the cut-off value of the SMA angle is controversial and variable. Therefore, the present study evaluated the optimal SMA angle to maximize diagnostic performance for NCS diagnosis by conducting a meta-analysis. METHODS: We comprehensively searched the English literature related to the diagnosis of NCS from the perspective of SMA (from the date of database inception to June 2022). The accuracy of an SMA angle less than 41° in the diagnosis of NCS was evaluated by calculating the pooled sensitivity (SEN), pooled specificity (SPE), positive likelihood ratio (LR+), negative likelihood ratio (LR-), summary receiver operating characteristic (SROC) curve and area under the curve (AUC) value. The I2 test and meta-regression analysis were used to assess heterogeneity and sources of heterogeneity, respectively. Publication bias was assessed using Deeks' funnel plot asymmetry test. RESULTS: Six studies (526 patients) met the inclusion criteria. SEN and SPE were 0.94 (95% confidence interval (CI) 0.80-0.99) and 0.85 (95% CI 0.65-0.94), respectively. The LR + value was 6.0, and the LR- value was 0.07, revealing that SMA angles less than 41° exhibited an excellent ability to help confirm or exclude NCS. Additionally, SROC curves showed that the AUC of SMA angles less than 41° for the diagnosis of NCS was 0.96, indicating that SMA angles less than 41° have good efficacy for helping to diagnose NCS. CONCLUSION: This study explored the diagnostic efficacy of the cut-off value of the SMA angle by meta-analysis. According to the high SPE and SEN results, SMA angles less than 41° have good efficacy in facilitating NCS diagnosis.

Superior mesenteric artery syndrome-induced pancreatitis: Case report.

Superior mesenteric artery syndrome is an acquired vascular compression disorder resulting from the compression of the third portion of the duodenum, which is the first part of the small intestine, leading to a reduction in the space between the aorta and the superior mesenteric artery. Although rare, superior mesenteric artery syndrome-induced pancreatitis has been documented in the literature. This article presents the case of a 20-year-old female patient with a history of colectomy for acute severe colitis, resulting in significant weight loss. She was admitted to the hospital with symptoms of upper bowel obstruction, and the diagnosis of superior mesenteric artery syndrome complicated by acute pancreatitis was made. The patient underwent a nutritional assistance program along with intravenous fluid therapy, resulting in positive outcomes. Superior mesenteric artery syndrome -induced pancreatitis is rarely reported and can be attributed to an occlusive post-papillary syndrome, which causes retrograde reflux of bile into the pancreatic duct, activating inflammation responsible for pancreatitis.

Navigating Complexities: A Case of Multiple Abdominopelvic Vascular Compression Syndromes in Ehlers-Danlos Syndrome.

Abdominopelvic vascular compression syndromes (VCS) refer to conditions where blood vessels in the abdomen or pelvis are compressed by nearby structures, leading to various symptoms and complications. These conditions include superior mesenteric artery syndrome (SMAS), nutcracker syndrome (NCS), May-Thurner syndrome (MTS), and median arcuate ligament syndrome (MALS). Each syndrome is characterized by specific compressions of blood vessels, resulting in symptoms such as pain, nausea, vomiting, weight loss, leg swelling, and other related issues. Ehlers-Danlos syndrome (EDS), characterized by hyperelasticity, altered collagen, and mobility of the viscera, has been associated with VCS, although the exact prevalence is unknown. We report a case of a patient with EDS who presented with multiple VCS, including NCS, MTS, SMAS, and MALS.

Superior Mesenteric Artery Syndrome: A Report of a Rare Case.

Superior mesenteric artery (SMA) syndrome is a rare disease in which the third part of the duodenum between the SMA and the abdominal aorta is compressed, leading to small bowel obstruction. Treatment is usually conservative, such as parenteral and nasojejunal nutrition. The pathophysiology includes loss of the retroperitoneal fat layer and subsequent duodenal compression. We present a 53-year-old malnourished female patient who came with complaints of vomiting, constipation, abdominal pain, and distension for four days. This article highlights the diagnostic challenges associated with SMA syndrome and emphasizes the importance of early diagnosis and intervention.

Superior mesenteric artery syndrome following spine surgery in idiopathic adolescent scoliosis: a systematic review.

Superior mesenteric artery syndrome (SMAS) is a rare and unpredictable complication after correction spine surgery for adolescent idiopathic scoliosis (AIS). The management of this condition is poorly investigated, with controversial outcomes. This investigation systematically reviewed current evidence on pathogenesis, risk factors, management, and outcomes of SMAS following correction spine surgery for AIS. The present systematic review was conducted according to the 2020 PRISMA statement. All the included investigations reported SMAS presentation following scoliosis correction surgery in AIS. 29 articles with 61 eligible patients were included in this review. The mean age of the patients was 15.8 ± 7.2 years. The mean weight was 45.3 ± 8.0 kg, the mean height 159.6 ± 13.6 cm, and the mean BMI 16.5 ± 2.9 kg/m2. The mean duration of the treatment for SMAS was 21.6 ± 10.3 days. The mean interval between spine surgery and symptoms of SAMS was 69 days, with high between-studies variability (3 days to 4 years). Prompt identification of risk factors and an early diagnosis are necessary to manage SMAS and reduce the risk of complications. Additional investigations are required to establish risk factors and diagnostic criteria.Level of evidence Level IV, systematic review.

Laparoscopic Surgery for Superior Mesenteric Artery Syndrome.

BACKGROUND: Superior mesenteric artery syndrome (SMAS) is a rare condition, for which laparoscopic surgery was successfully performed safely and with long-term efficacy. METHODS: This single center retrospective clinical study comprised 66 patients with SMAS, surgically treated between January 2010 and January 2020, who were allocated to three different surgical groups according to their medical history and symptoms (Laparoscopic duodenojejunostomy, n = 35; Gastrojejunostomy, n = 16; Duodenojejunostomy plus gastrojejunostomy, n = 15). Patient demographics, surgical data and postoperative outcomes were retrieved from the medical records. RESULTS: All operations were successfully completed laparoscopically, and with a median follow-up of 65 months, the overall symptom score was significantly reduced from 32 to 8 (p < 0.0001) and the BMI was increased from 17.2 kg/m2 to 21.8 kg/m2 (p < 0.0001). CONCLUSIONS: When conservative measures failed in the treatment of SMAS, laparoscopic surgery proved to be a safe and effective method. The specific surgical technique was selected according to the history and symptoms of each individual patient. To our knowledge, this study represents the largest number of laparoscopic procedures at a single center for the treatment of superior mesenteric artery syndrome.

A rare cause of upper gastrointestinal system obstruction: Superior mesenteric artery syndrome (Wilkie's syndrome); two different case reports.

Superior mesenteric artery syndrome is defined as a collection of clinical symptoms and findings that result from compression of the third part of the duodenum between the aorta and the superior mesenteric artery. Here, we describe two patients who were diagnosed with superior mesenteric artery syndrome. Two patients, 18 and 38 years old, respectively, presented to our clinic with complaints of nausea, vomiting, and weight loss. Computed tomography scans of both patients supported diagnoses of superior mesenteric artery syndrome. The 18-year-old patient recovered with conservative treatment. However, our 38-year-old patient did not recover with conservative treatment and required two surgeries. In the first operation, duodenal release with Treitz's ligament dissection and pyloroplasty were performed because of concomitant hypertrophic pyloric stenosis. Because the patient exhibited gastroparesis and gastric ptosis after the first operation, subtotal gastrectomy and Roux-n-Y gastrojejunostomy were performed in the second operation. No complications were observed during follow-up after the second operation. Superior mesenteric artery syndrome should be considered in the differential diagnosis of patients with nausea, vomiting, and weight loss of unknown cause. During treatment, weight-gaining conservative approaches should be attempted initially, but surgical treatment should not be excessively delayed in patients who do not respond to medical treatment.

Necrotic mega-stomach from superior mesenteric artery syndrome.

Superior mesenteric artery (SMA) syndrome is a rare cause of proximal bowel obstruction due to duodenal compression by the SMA. The morbidity and mortality associated with delayed diagnosis and its complications make it an important differential cause for bowel obstruction. We report a case of mega-stomach secondary to SMA syndrome requiring total gastrectomy. An 18-year-old male presented with vomiting, abdominal pain and shock after a buffet. Computed tomography (CT) imaging revealed a grossly distended stomach (113 × 187 × 350mm) and a transition point at the third part of the duodenum, along with pneumatosis and portal venous gas. Emergency gastroscopy showed blood and necrotic mucosa. Laparotomy confirmed full thickness necrosis and the patient underwent a total gastrectomy with Roux-en-Y reconstruction. Postoperatively, he had a brief intensive care stay and recovered without complications. This case underscores the importance of considering SMA syndrome during presentations of acute gastric dilatation.

Association between underweight status or low body mass index and the risk of developing superior mesenteric artery syndrome following scoliosis corrective surgery in pediatric patients: a review of the literature.

Superior mesenteric artery (SMA) syndrome is the compression of the third portion of the duodenum between the abdominal aorta and the superior mesenteric artery. Although multifactorial, the most frequent cause of SMA syndrome is significant weight loss and cachexia often induced by catabolic stress. SMA syndrome resulting from scoliosis surgery is caused by a reduction of the aortomesenteric angle and distance. Risk factors include rapid weight loss, malnutrition, and a rapid reduction in the mesenteric fat pad and are the most common causes of a decrease in the aortomesenteric angle and distance. Surgically lengthening the vertebral column can also lead to a reduction of the aortomesenteric distance, therefore, has been identified as a risk factor unique to spinal surgery. Despite a reported decline in SMA syndrome cases due to improved surgical techniques, duodenal compression is still a risk and remains a life-threatening complication of scoliosis surgery. This article is a cumulative review of the evidence of being underweight or having a low body mass index as risk factors for developing SMA syndrome following surgical scoliosis instrumentation and correction.

Understanding Superior Mesenteric Artery Syndrome: Etiology, Symptoms, Diagnosis, and Management.

Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, poses a diagnostic challenge due to its rarity and varied clinical manifestations. This review explores the syndrome's etiology, symptoms, diagnostic challenges, and management strategies. Symptoms range from early satiety to severe abdominal pain, often leading to malnutrition. Diagnosis involves a thorough gastrointestinal evaluation and various imaging modalities. Management includes medical interventions like nasogastric decompression and nutritional support, along with surgical interventions such as duodenojejunostomy. A thorough understanding of SMA syndrome's complexities is crucial for its timely diagnosis and effective management, especially considering its potential overlap with other gastrointestinal disorders or eating disorders. Further research is needed to enhance understanding and improve patient outcomes.

A unique posterior nutcracker syndrome combined with Wilkie syndrome: A singular case.

Left renal vein variants are not commonly observed in the general population. Usually, the renal vein runs in front of the aorta before entering the inferior vena cava, while the most common variants include the presence of a circumaortic or retroaortic renal vein. However, when present, left venal rein variants are important to recognize due to their potential clinical and surgical relevance. In this regard, CE-CT is an instrument with high sensitivity and specificity in detecting vascular anomalies and can certainly help diagnose. In this article, we present a unique case of a left venal rein compressed between the left iliac artery and vertebral bodies associated with the presence of a superior mesenteric artery Syndrome, another rare entity that occurs when the duodenum is compressed between the aorta and the superior mesenteric artery.

Síndrome de la arteria mesentérica superior (Síndrome de Wilkie): A propósito de un caso en una adolescente / Superior mesenteric artery syndrome (Wilkie syndrome): A case report in an adolescent

Introducción. El síndrome de la arteria mesentérica superior, también conocido como síndrome de Wilkie, es una entidad infrecuente en la que una reducción del ángulo o espacio aorto-mesentérico determina una compresión extrínseca con obstrucción del tercio distal del duodeno, demostrando síntomas y signos característicos de obstrucción intestinal. El pilar del tratamiento es el manejo médico conservador con repleción nutricional, con el objetivo de aumentar el grosor de la grasa retroperitoneal y con esto resolver la compresión duodenal. Caso clínico. Paciente de 16 años, con un curso crónico marcado por dolor abdominal, estreñimiento, distensión, pirosis y pérdida de peso. Inicialmente se trató como una enfermedad ácido-péptica, con pobre mejoría, por lo que se realizaron estudios imagenológicos con lo que se documentó una disminución del ángulo aortomesentérico. Resultado. Se instauró un manejo médico con un protocolo de repleción nutricional por vía oral, para lograr ganancia de peso y evitar la cirugía. Conclusión. Con base a los síntomas y hallazgos en imágenes diagnósticas se hizo el diagnóstico de síndrome de la arteria mesentérica superior, que es una causa de obstrucción intestinal con muy baja incidencia. Su presentación clínica incluye gran variedad de síntomas, entre los que destaca el dolor abdominal y la pérdida de peso. Es frecuente la confusión con otras enfermedades más frecuentes, como enfermedad ácido-péptica, reflujo gastrointestinal y malabsorción. Su diagnóstico requiere un alto índice de sospecha, por lo que es importante conocer esta entidad y considerarla en el estudio de pacientes con los síntomas mencionados.

Introduction. Superior mesenteric artery syndrome, also known as Wilkie syndrome, is a rare entity in which a reduction in the aorto-mesenteric angle or space determines extrinsic compression with obstruction of the distal third of the duodenum, demonstrating characteristic symptoms and signs of intestinal obstruction. The mainstay of treatment is conservative medical management with nutritional repletion, with the aim of increasing the thickness of the retroperitoneal fat and thereby resolving duodenal compression. Clinical case. A 16-year-old patient, with a chronic course marked by abdominal pain, constipation, bloating, heartburn and weight loss. Initially was treated as an acid-peptic disease, with poor improvement. Imaging studies were performed, which documented a decrease in the aorto-mesenteric angle. Conclusion. Based on the symptoms and findings on diagnostic images, the diagnosis of superior mesenteric artery syndrome was made, which is a cause of intestinal obstruction with a very low incidence. Its clinical presentation includes a wide variety of symptoms, among which abdominal pain and weight loss stand out. Confusion with other more common diseases is common, such as acid-peptic disease, gastrointestinal reflux and malabsorption. Its diagnosis requires a high index of suspicion, so it is important to know this entity and consider it in the study of patients with the aforementioned symptoms.

Single Anastomosis Sleeve Jejunal Bypass in Acute Mesenteric Artery Syndrome: A Case Report.

Superior mesenteric artery syndrome is a rare vascular compression syndrome in which the duodenum is compressed between the aorta and the overlying superior mesenteric artery. This condition is often chronic and secondary to cachexia. It can trigger further weight loss due to the subsequent proximal intestinal obstruction, causing a positive feedback loop. We report a case of acute presentation of superior mesenteric artery syndrome, complicated by gastric necrosis and treated surgically using the principles of a novel bariatric procedure.

Unusual causes of Small bowel obstruction: a review of the literature and revisited cross-sectional imaging checklist.

Intestinal obstruction is a common surgical emergency with high morbidity and mortality. Patients presenting with features of small bowel obstruction need urgent evaluation to avoid complications such as bowel gangrene, perforation, or peritonitis. Imaging is necessary in most cases of suspected bowel obstruction, to take an appropriate decision, for apt patient management. Among the common causes of small bowel obstruction, adhesions, external herniae, malignancies, and Crohn's disease top the chart. Imaging helps in determining the presence of obstruction, the severity of obstruction, transition point, cause of obstruction, and associated complications such as strangulation, bowel gangrene, and peritonitis. This review is based on the cases with unusual causes of bowel obstruction encountered during our routine practice and also on the extensive literature search through the standard textbooks and electronic databases. Through this review we want our readers to have sound knowledge of the imaging characteristics of the uncommon yet important causes of bowel obstruction. We have also revisited and structured a checklist to simplify the approach while reporting a suspected case of small bowel obstruction. Imaging plays a key role in the diagnosis of small bowel obstruction and in determining the cause and associated complications. Apart from the common causes of small bowel obstruction, we should also be aware of the uncommon causes of small bowel obstruction and their imaging characteristics to make an accurate diagnosis and for apt patient management.

Unusual neonatal case of superior mesenteric artery syndrome with Meckel's diverticulum and literature review.

Superior mesenteric artery syndrome (SMAS) is a rare cause of duodenal obstruction which is characterized by compression of the duodenum due to narrowing of the space between the superior mesenteric artery and aorta. Incomplete duodenal obstruction due to SMAS in neonates is rarely reported in the literature. In this case, it is a full-term 2-day-old male with the complaint of recurrent vomiting starting soon after birth. The patient was diagnosed with SMAS and duodenoduodenostomy was performed. Accompanying Meckel's diverticulum was excised.

El síndrome de la arteria mesentérica superior (SMAS) es una causa rara de obstrucción duodenal que se caracteriza por la compresión del duodeno debido al estrechamiento del espacio entre la arteria mesentérica superior y la aorta. La obstrucción duodenal incompleta por SMAS en recién nacidos rara vez se informa en la literatura. En este caso se trata de un varón de 2 días nacido a término que presenta vómitos recurrentes desde poco después del nacimiento. El paciente fue diagnosticado de SMAS y se le realizó duodenoduodenostomía. Se extirpó el divertículo de Meckel que lo acompañaba.

Challenges and pitfalls in diagnosing superior mesenteric artery syndrome: A case report.

Superior mesenteric artery syndrome is a rare condition that causes duodenal obstruction due to compression by the superior mesenteric artery and the aorta. It can be related to congenital or acquired factors that reduce the aortomesenteric angle and distance. The clinical presentation is nonspecific and often mimics other causes of upper gastrointestinal obstruction. The diagnosis requires a high index of suspicion and a correlation of clinical symptoms with radiographic findings. We report a case of a 17-year-old female patient who presented with chronic abdominal pain, vomiting, and weight loss. She was diagnosed with superior mesenteric artery syndrome based on an abdominal computed tomography scan that showed a reduced aortomesenteric angle of 30° and a reduced aortomesenteric distance of 7 mm. She was treated conservatively with anticoagulation therapy, dietary modification, and physical activity. She reported symptom improvement and weight gain after 2 weeks of treatment. This case illustrates the challenges and pitfalls in diagnosing superior mesenteric artery syndrome.