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.

Superior mesenteric artery syndrome managed laparoscopically: a case report.

BACKGROUND: Superior mesenteric artery syndrome is a rare condition that has only around 400 reported cases so far. Typically, the superior mesenteric artery branches off the abdominal aorta at 45° to create an aortomesenteric distance of 10-28 mm, with the duodenum passing through. However, if this aortomesenteric angle reduces to less than 25°, the third portion of the duodenum becomes compressed between the SMA and aorta, causing mechanical obstruction. CASE PRESENTATION: This case report aims to demonstrate the diagnostic difficulties and the laparoscopic management of a 52-year-old Indian male presenting with abdominal pain and vomiting, with associated weight loss. Imaging was further suggestive of high intestinal obstruction, and he was later found to have superior mesenteric artery syndrome. CONCLUSION: Taking into account a significant reduction in morbidity, we propose laparoscopic duodenojejunostomy to be the new procedure of choice for superior mesenteric artery syndrome.

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.

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.

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.

[Wilkie syndrome as a diagnostic challenge in intestinal obstruction: case report]. / Síndrome de Wilkie como reto diagnóstico en obstrucción intestinal: reporte de caso.

Wilkie syndrome is a rare pathology that generates intestinal obstruction due to a decrease of the aortomesenteric angle compromising the third portion of the duodenum. We describe a case of an 18-year-old female patient, diagnosed with Wilkie syndrome, with clinical symptoms of intestinal obstruction and weight loss. The diagnosis was made with abdominal CT. Wilkie syndrome is a rare pathology, which becomes a diagnostic challenge because it presents a similar picture to other more common pathologies. We recommend that it should be suspected in the presence of duodenal obstruction.

Vascular compression syndromes in the abdomen and pelvis: a concise pictorial review.

Vascular compression syndromes are a diverse group of pathologies that can manifest asymptomatically and incidentally in otherwise healthy individuals or symptomatically with a spectrum of presentations. Due to their relative rarity, these syndromes are often poorly understood and overlooked. Early identification of these syndromes can have a significant impact on subsequent clinical management. This pictorial review provides a concise summary of seven vascular compression syndromes within the abdomen and pelvis including median arcuate ligament (MAL) syndrome, superior mesenteric artery (SMA) syndrome, nutcracker syndrome (NCS), May-Thurner syndrome (MTS), ureteropelvic junction obstruction (UPJO), vascular compression of the ureter, and portal biliopathy. The demographics, pathophysiology, predisposing factors, and expected treatment for each compression syndrome are reviewed. Salient imaging features of each entity are illustrated through imaging examples using multiple modalities including ultrasound, fluoroscopy, CT, and MRI.

Delayed presentation of Wilkie's syndrome after scoliotic curve correction surgery: a case report.

BACKGROUND: Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, is a rare but serious complication following scoliosis correction surgery. It occurs as a result of mechanical compression of third part of duodenum between the SMA and aorta. This condition occurs most commonly in significantly underweight patients with deformities, and usually during the first week following spinal deformity corrective surgeries. The angle between the abdominal aorta and the SMA gets reduced following spinal lengthening during deformity correction surgery causing compression of third part of duodenum resulting in development of SMA syndrome. CASE PRESENTATION: We present a case of 17-year-old male with congenital scoliosis with a 70-degree scoliotic curve who underwent spinal deformity correction surgery with posterior instrumented fusion. Post-operative course was uneventful and the patient was discharged after suture removal on post-operative day 15. The patient presented after 21-days of symptom onset on post-operative-day 51, with a 3 week history of post-prandial vomiting, abdominal pain and distension which resulted in rapid weight loss of 11 kg. A CT-angiogram showed obstruction at third part of duodenum. After reviewing clinical and radiological profile of the patient, a diagnosis of SMA syndrome was made. Conservative management was tried, but due to rapid deterioration of patient condition and symptoms of complete intestinal obstruction, the patient was treated surgically by gastro-jejunostomy and side-to-side jejuno-jejunostomy, which improved his condition. CONCLUSION: SMA syndrome can occur much later than previously reported cases and with potentially life-threatening symptoms following scoliosis correction. Having a high index of suspicion, early recognition of condition and institution of appropriate treatment are essential to prevent occurrence of severe complications including risk of intestinal perforation and mortality. This case highlights management of delayed onset of SMA syndrome, with presentation further delayed after symptom onset, as is common in developing parts of the world, due to limited availability and accessibility of resources, and low socio-economic status of large segments of the population.

Acute superior mesenteric artery syndrome complicated by severe gastric, pancreatic and renal ischaemia.

Superior mesenteric artery syndrome (SMAS) is a rare and potentially life-threatening cause of small bowel obstruction in which the superior mesenteric artery impinges on the third portion of the duodenum. SMAS is typically encountered in patients with low body fat and a history of rapid weight loss and is often diagnosed as a chronic or subacute condition. Here, we describe a case of a healthy adolescent boy without typical SMAS prodromal symptoms presenting with a severe, hyperacute proximal small bowel obstruction due to SMAS. Complications arising from massive gastric and duodenal distension, including gastric, pancreatic and renal ischaemia, necessitated emergent surgical intervention consisting of the duodenojejunostomy bypass with partial gastric resection. The patient recovered without significant lasting consequences.

Superior Mesenteric Artery Syndrome in Anorexia Nervosa: A Case Report and a Systematic Revision of the Literature.

BACKGROUND: Superior mesenteric artery syndrome (SMAS) is a rare condition caused by the compression of the duodenum, which may occur in the case of fast weight loss. Currently, the relationship between superior mesenteric artery syndrome and anorexia nervosa is still unclear. The aim of this study is to identify the precocious clinical signs and symptoms of SMAS in patients affected by anorexia nervosa so as not to delay the diagnosis. METHODS: We present the clinical case of a young female patient with anorexia nervosa complicated by SMAS. We performed a literature review of SMAS in children affected by anorexia nervosa between 1962 and 2023, according to the PRISMA Extension Guide for Scoping Reviews. RESULTS: Reviewing the literature, 11 clinical cases were described for the pediatric age. The median age at diagnosis was 17 years (ranging from 13 to 18 years). The diagnosis of SMAS may be challenging as symptoms overlap those of anorexia, but it should be kept in mind mostly in cases of post-prandial abdominal pain, anxiety or depression, nausea, vomiting, and weight loss. CONCLUSION: Even specific clinical symptoms may act as flag tags to drive attention to this rare but potentially fatal condition.

Dynamic ultrasonography for optimizing treatment position in superior mesenteric artery syndrome: Two case reports and review of literature.

BACKGROUND: Superior mesenteric artery (SMA) syndrome is a rare cause of duodenal obstruction by extrinsic compression between the SMA and the aorta (SMA-Ao). Although the left lateral recumbent position is considered effective in the treatment of SMA syndrome, individual variations in the optimal patient position have been noted. In this report, we present two elderly cases of SMA syndrome that exhibited rapid recovery due to ultrasonographic dynamic evaluation of the optimal position for each patient. CASE SUMMARY: Case 1: A 90-year-old man with nausea and vomiting. Following diagnosis of SMA syndrome by computed tomography (CT), ultrasonography (US) revealed the SMA-Ao distance in the supine position (4 mm), which slightly improved in the lateral position (5.7-7.0 mm) without the passage of duodenal contents. However, in the sitting position, the SMA-Ao distance was increased to 15 mm accompanied by improved content passage. Additionally, US indicated enhanced passage upon abdominal massage on the right side. By day 2, the patient could eat comfortably with the optimal position and massage. Case 2: An 87-year-old woman with vomiting. After the diagnosis of SMA syndrome and aspiration pneumonia by CT, dynamic US confirmed the optimal position (SMA-Ao distance was improved to 7 mm in forward-bent position, whereas it remained at 5 mm in the supine position). By day 7 when her pneumonia recovered, she could eat with the optimal position. CONCLUSION: The optimal position for SMA syndrome varies among individuals. Dynamic US appears to be a valuable tool in improving patient outcomes.

Laparoscopic lateral duodenojejunostomy for pediatric superior mesenteric artery compression syndrome: a cohort retrospective study.

PURPOSE: There are only a few case reports of laparoscopic lateral duodenojejunostomy (LLDJ) in children with Wilkie's syndrome, also known as superior mesenteric artery compression syndrome (SMAS). We aimed to describe our laparoscopic technique and evaluate its outcomes for SMAS in children. METHODS: From January 2013 to May 2021, SMAS children who received LLDJ were included. The procedure was carried out utilizing the four-trocar technique. The elevation of the transverse colon allows good exposure of the dilated and bulging second and third sections of the duodenum. Using a linear stapler, we established a lateral anastomosis connecting the proximal jejunum with the third part of the duodenum. Following that, a running suture was used to intracorporeally close the common enterotomy. Clinical data on patients was collected for analysis. The demographics, diagnostic findings, and postoperative outcomes were analyzed retrospectively. RESULTS: We retrospectively analyzed 9 SMAS patients (6 females and 3 males) who underwent LLDJ, aged between 7 and 17 years old. The mean operative time was 118.4 ± 16.5 min and the mean estimated blood loss was 5.6 ± 1.4 ml. There were no conversion, intraoperative complications or immediate postoperative complications. The mean postoperative hospital stay was 6.8 ± 1.9 days and the mean follow-up time was 5.4 ± 3.0 years. During follow-up, seven patients (77.8%) experienced complete recovery of symptoms prior to surgery. One patient (11.1%) still had mild vomiting, which resolved with medication. Another patient (11.1%) developed psychological-induced nausea, which significantly improved after treatment with education, training and diet management. CONCLUSIONS: LLDJ represents a feasible and safe treatment option for SMAS in well-selected children. Further evaluation with more cases and case-control studies is required for the real benefits.

Superior mesenteric artery syndrome and anorexia nervosa: a case report.

BACKGROUND: Superior mesenteric artery (SMA) syndrome is an underdiagnosed complication in anorexia nervosa (AN) patients, which results from weight loss-induced atrophy of the mesenteric fat pad, causing compression of the third part of the duodenum. SMA syndrome can be life-threatening as its nonspecific symptomatology often results in a delayed diagnosis. It is a rare condition, but its true prevalence may be higher than the reported numbers. A history of persistent nausea and vomiting after oral intake and weight loss in AN should raise suspicion about this diagnosis, as weight loss is the most significant factor in this diagnosis. Other high-risk factors include rapid, extreme weight loss, anatomical abnormalities, or a history of prior abdominal or spinal surgeries. CASE PRESENTATION: The patient presented in this report was a 26-year-old Caucasian female with a history of severe enduring anorexia nervosa. This patient suffered from an insidious case of SMA syndrome secondary to AN. This patient presented with vague symptoms of nausea and vomiting, persistent abdominal pain, and rapid weight loss. The patient was successfully treated but could have had a much different outcome if the diagnosis had been further delayed. CONCLUSIONS: An awareness of SMA syndrome and its clinical presentation within similar populations can prevent complications and even fatalities that come with it.

Understanding the diagnosis of superior mesenteric artery syndrome: analysis of the location of duodenal impression on upper gastrointestinal studies.

BACKGROUND: Upper gastrointestinal (GI) contrast studies are frequently requested to aid superior mesenteric artery syndrome diagnosis, a rare entity. Compression of the third duodenal part is expected to be mid-to-left of the midline where the superior mesenteric artery arises from the aorta; however, a duodenal impression to the right of the midline due to normal anatomic impression by the inferior vena cava (IVC) is often encountered and frequently misdiagnosed. OBJECTIVE: The purpose of this study was to determine the frequencies of (1) normal right-of-midline duodenal impressions and (2) mid-to-left of midline compressions in upper GI studies in a tertiary pediatric referral center. MATERIALS AND METHODS: All upper GI studies performed at our institution over 2 years were retrospectively evaluated to determine whether the duodenum had vertical duodenal impression to the right of the vertebral midline, mid-to-left of the vertebral midline, or no identifiable duodenal impression at all. RESULTS: In total, 538 upper GI studies were included in this analysis. A total of 275 male and 247 female patients between 0 and 17 years of age (median: 6 years, range: 1 month-17 years) were included. Of 538 total upper GI studies, there were 240 studies (44.6%) with a right-of-midline impression. There were only 10 studies (1.9%) with a mid-to-left of midline compression, and 9/10 also showed a concurrent right-sided impression sign. CONCLUSION: Right-of-midline duodenal impression is a normal anatomic finding caused by the IVC and should not be confused with superior mesenteric artery syndrome. In the presence of an appropriate clinical context, proximal duodenal dilation, "to-and-fro" motion of contrast, and duodenal impression at mid-to-left of midline, a diagnosis of superior mesenteric artery syndrome should be considered.

Incidence and Potential Risk Factors of Superior Mesenteric Artery Syndrome After Spinal Corrective Surgery in Patients with Adult Spinal Deformity.

OBJECTIVE: This study aimed to determine the incidence and potential risk factors of superior mesenteric artery syndrome (SMAS) after corrective spinal surgery in patients with adult spinal deformity (ASD). METHODS: In total, 102 patients (67.6 ± 8.4 years; 8 male/94 female; body mass index (BMI); 22.4 ± 3.6 kg/m2) with ASD treated by spinal correction surgery were enrolled. Preoperative and postoperative spinal parameters, including thoracolumbar kyphosis (TLK: T10-L2) and upper lumbar lordosis (ULL: L1-L4) were measured. To evaluate the potential risk factors of SMAS, the angle and the distance between the superior mesenteric artery and aorta, the aortomesenteric angle (AMA) and aortomesenteric distance (AMD), were evaluated pre- and postoperatively. Based on the postoperative AMA, AMD, and abdominal symptoms, the patients were diagnosed with SMAS. Correlations between demographic data or spinal parameters and AMA and AMD were assessed. RESULTS: Two (2.0%) patients were diagnosed with SMAS. Postoperative TLK significantly correlated with postoperative AMA (P = 0.013, 0.046). Postoperative ULL was significantly correlated with postoperative AMD (ß = -0.27; P = 0.014). CONCLUSION: The incidence of SMAS after corrective spinal surgery in patients with ASD was 2.0%. Postoperative smaller TLK and greater ULL can be risk factors for developing SMAS. Spine surgeons should avoid overcorrection of the upper lumbar spine in the sagittal plane to prevent SMAS.

Massive Gastric Dilatation and Multi-Organ Ischemia Due to Superior Mesenteric Artery Syndrome: A Rare Case Report.

BACKGROUND Superior mesenteric artery (SMA) syndrome, a rare condition in which the SMA and aorta occlude the third duodenal portion, can cause serious complications. We present the case of an 83-year-old Japanese man who presented with shock because of massive gastric dilatation due to SMA syndrome and developed multi-organ ischemia. CASE REPORT The day before admission, the patient had visited the emergency department with abdominal pain and was sent home following spontaneous symptom resolution, but experienced abdominal pain flare-up. His history included diabetes mellitus, hypertension, gastric ulcer, prostatic hypertrophy, esophageal hiatal hernia, and esophageal cancer. Plain computed tomography showed gastric dilatation and obstruction of the duodenal third portion by the SMA, leading to SMA syndrome diagnosis. Since a nasogastric tube could not be manually inserted into the stomach and the gastric dilatation could not be decompressed, the tube was inserted endoscopically. Endoscopy revealed mechanical obstruction of the gastric cardia and gastric mucosal ischemia. He was admitted to intensive care, and blood pressure was maintained with vasopressors and blood transfusion. The next day, contrast-enhanced computed tomography performed for persistently elevated lactate levels revealed extensive ischemia affecting multiple gastrointestinal organs. Surgery and other treatments were considered too risky due to the patient's advanced age and condition. Best supportive care was administered after discussion with the family, and he died on the second day of hospitalization. CONCLUSIONS SMA syndrome with extensive ischemia and infarction is rare. Given this, clinicians should remain vigilant for its potential complications.