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.

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.

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.

[A Case of Primary Jejunum Cancer Mimicking Superior Mesenteric Artery Syndrome].

In this report, we described a case of jejunum cancer that presented with abdominal pain and vomiting as chief complaints, which mimicked superior mesenteric artery syndrome. An elderly woman in her 70s was referred to our department for prolonged abdominal discomfort. Findings from CT and abdominal echo indicated that superior mesenteric artery syndrome may be responsible for jejunum cancer. Upper gastrointestinal endoscopy revealed a peripheral type 2 lesion in the upper jejunum. Upon biopsy, the patient was diagnosed with adenocarcinoma(papillary type). Surgical resection of the small intestine was performed. Although small intestinal cancer is a fairly rare disease, it should be considered a differential diagnosis. Comprehensive evaluations including medical history and imaging should be considered.

Surgical Intervention Could Relieve Obstruction-Related Symptoms of Refractory Superior Mesenteric Artery Syndrome: Long-Term Follow-up Results.

PURPOSE: Superior mesenteric artery syndrome (SMAS) is one of the rare causes of chronic duodenal obstruction. The aim of our study was to evaluate the outcomes of surgical management of refractory SMAS (Re-SMAS) in our institution during the last decade. MATERIALS AND METHODS: Consecutive patients diagnosed as Re-SMAS and underwent surgical intervention were retrospectively enrolled. A modified Likert-scale-based questionnaire was used to quantify the symptoms of SMAS. The primary outcome was the increase of BMI and symptomatic relief after surgery. RESULTS: From January 2010 to January 2020, 22 patients diagnosed with Re-SMAS and underwent surgery were included. Age distribution of included patients were < 19 (10/22, 45%), 19-45 (11/22, 50%), and > 45 (1/22, 5%). A significant BMI increase was recorded, [16.1 (14.6-23.7) kg/m2 vs 21.9 (15.6-29.5) kg/m2 before and after surgery, respectively, p < 0.001]. Up to 20 patients (20/22, 91%) reported symptomatic relief. The obstruction-related symptom score decreasing significantly (p < 0.001), with reported nausea, vomiting, and regurgitation incidences dropping from 77 to 41% (p = 0.031), 68 to 23% (p = 0.006), and 32 to 5% (p = 0.046), respectively. CONCLUSION: Surgical interventions could relieve obstruction-related symptoms of Re-SMAS and promote weight gain. For patients who suffered from Re-SMAS and referred to surgeons mainly for obstruction-related symptoms, surgical intervention is recommended.

Surgical therapy of celiac axis and superior mesenteric artery syndrome.

INTRODUCTION: Compression syndromes of the celiac artery (CAS) or superior mesenteric artery (SMAS) are rare conditions that are difficult to diagnose; optimal treatment remains complex, and symptoms often persist after surgery. We aim to review the literature on surgical treatment and postoperative outcome in CAS and SMAS syndrome. METHODS: A systematic literature review of medical literature databases on the surgical treatment of CAS and SMAS syndrome was performed from 2000 to 2022. Articles were included according to PROSPERO guidelines. The primary endpoint was the failure-to-treat rate, defined as persistence of symptoms at first follow-up. RESULTS: Twenty-three studies on CAS (n = 548) and 11 on SMAS (n = 168) undergoing surgery were included. Failure-to-treat rate was 28% for CAS and 21% for SMAS. Intraoperative blood loss was 95 ml (0-217) and 31 ml (21-50), respectively, and conversion rate was 4% in CAS patients and 0% for SMAS. Major postoperative morbidity was 2% for each group, and mortality was described in 0% of CAS and 0.4% of SMAS patients. Median length of stay was 3 days (1-12) for CAS and 5 days (1-10) for SMAS patients. Consequently, 47% of CAS and 5% of SMAS patients underwent subsequent interventions for persisting symptoms. CONCLUSION: Failure of surgical treatment was observed in up to every forth patient with a high rate of subsequent interventions. A thorough preoperative work-up with a careful patient selection is of paramount importance. Nevertheless, the surgical procedure was associated with a beneficial risk profile and can be performed minimally invasive.

Superior mesenteric artery syndrome after colectomy: A case report and literature review.

RATIONALE: Superior mesenteric artery (SMA) syndrome, also known as Wilkie syndrome, is a rare benign disease characterized by small bowel obstruction due to compression of the third portion of the duodenum by the SMA and the abdominal aorta. However, SMA syndrome after colectomy is extremely rare, establishing a clear diagnosis and formulating a treatment plan may be challenging for surgeons. PATIENT CONCERNS: A 64-year-old male with multiple colon polyposis and constipation underwent laparoscopic subtotal colectomy with cecal-rectal anastomosis. On the seventh postoperative day, he started vomiting and complained of abdominal bloating. DIAGNOSIS: An upper gastrointestinal series, computed tomography scan and computed tomography angiography confirmed the diagnosis of SMA syndrome. INTERVENTIONS: Gastric decompression, nasojejunal tube feeding and parenteral nutrition were performed. OUTCOMES: After 3 weeks of conservative treatment, the patient showed no clinical improvement in symptoms. Subsequently, he suffered from gastrointestinal hemorrhage, deep venous thrombosis of the lower extremity and cerebral hemorrhage successively. Unfortunately, the patient eventually died. LESSONS: Surgeons should be aware of the fact that SMA syndrome can occur after colectomy. Every attempt should be made to correct and avoid any predisposing factors perioperatively. Prompt diagnosis of SMA syndrome after colectomy and appropriate early intervention reduce mortality.

Unusual presentation of superior mesenteric artery syndrome in a child.

Superior mesenteric artery syndrome (SMAS), also known as Wilkie's syndrome, is a rare cause of upper gastrointestinal tract obstruction. We report a case of a 10-year-old girl with persistent abdominal pain for over 3 months, who on extensive investigations was diagnosed with SMAS. She underwent a surgical procedure to bypass the obstructed portion of the intestine for relief of her symptoms.

Superior Mesenteric Artery Syndrome Accompanied by Acute-onset Type 1 Diabetes Complicated with Graves' Disease.

A 35-year-old man experienced general fatigue and could not eat solid food because of nausea and vomiting. His weight abruptly decreased from 49 to 45 kg after 2 weeks. A detailed examination indicated superior mesenteric artery syndrome (SMAS) accompanied by acute-onset type 1 diabetes complicated by Graves' disease, referred to as autoimmune polyglandular syndrome type 3A (APS3A). Although SMAS has a good prognosis, some cases require emergency surgery, especially when complicated by gastric perforation. In our case, APS3A and SMAS developed rapidly and at approximately the same time, resulting in a cycle of mutual exacerbation.

[Superior mesenteric artery syndrome in gastric cancer: a case report]. / Syndrome de la pince aorto-mésentérique sur cancer gastrique: à propos d'un cas.

Superior mesenteric artery syndrome or Wilkie syndrome is due to the compression of the third duodenum between the superior mesenteric artery and the aorta. It causes acute or chronic upper bowel occlusion. Abdominal CT scan facilitates the diagnosis. Severe malnutrition is its main etiological factor. Medical treatment can be based on aspiration of gastric contents and parenteral nutrition. If this fails, surgery is necessary. We here report the case of a 46-year-old patient, with a history of smoking, presenting with profuse postprandial bile and food vomiting. He had had weight loss of 7% over a period of 6 months. Upper GI endoscopy revealed non-stenotic antro-pyloric tumour mass. Histological examination showed poorly differentiated tubular gastric adenocarcinoma. Staging was without any peculiarity and allowed for the detection of superior mesenteric artery syndrome at an angle of 8°C. The patient received parenteral nutrition for 10 days, followed by inferior pole gastrectomy and gastrojejunal anastomosis (omega loop). The postoperative course was uneventful. Adjuvant chemotherapy was indicated.

Superior mesenteric artery syndrome: A rare complication of scoliosis corrective surgery.

Superior mesenteric artery (SMA) syndrome is a rare but serious complication following scoliosis surgery. Early diagnosis and management are key factors for successful conservative treatment to avoid the need for emergency laparotomies which causes higher morbidity or even mortality. We report two adolescent idiopathic scoliosis patients with Cobb angle of 49° and 132°, respectively, and low body mass index who presented with SMA syndrome following posterior spinal fusion from T2 to L3 and were treated successfully with conservative management. Abdominal radiographs showed distended gastric shadow. Computed tomography angiography of the abdomen showed decreased aortomesenteric angle and SMA-aorta distance. Both patients were treated successfully with conservative treatment which included three principles: gastric decompression with nasogastric tube, correction of electrolytes imbalance, and nutritional support with low volume, high calorie nutritional supplement. Both patients were started with small but frequent meals. Surgeries were not required in both cases. Early diagnosis and management are the key factors to successful treatment in SMA syndrome. Patients with SMA can be treated successfully with conservative treatment comprising of nasogastric decompression, electrolyte correction, and nutritional support with small but frequent meals.

Ligament of Treitz Release With Duodenal Lowering for Pediatric Superior Mesenteric Artery Syndrome.

BACKGROUND: Operative approaches for superior mesenteric artery syndrome (SMAS) vary from Roux-en-Y duodenojejunostomy to the more conservative division of the ligament of Treitz with inferior duodenal derotation known as the Strong procedure. We sought to examine outcomes following a modified version of Strong procedure where the duodenum is lowered as opposed to derotated for the management of SMAS. MATERIALS AND METHODS: We conducted a retrospective chart review of children who underwent surgical management of SMAS between January 2008 and December 2017. An online survey regarding symptom resolution, feeding practices, and the need for additional procedures was distributed. Data are reported as medians with interquartile range (IQR) and proportions as percentages. RESULTS: Seven patients with a median age of 15 y (IQR 8, 16) and median body mass index of 16.9 (IQR, 12.6, 22.1) were included. Presenting symptoms included pain (71%), nausea (57%), and vomiting (43%). Six patients initially underwent duodenal lowering, whereas one patient underwent duodenoduodenostomy. One patient underwent adhesiolysis for bowel obstruction in the early postoperative period. All patients had symptom resolution at a postoperative follow-up of 22 d (IQR, 15, 45). Two patients had symptom recurrence, and one patient required reoperation. Six patients completed the survey at a median of 2.3 y (IQR, 1.7, 3.2) postoperatively, of which four underwent duodenal lowering. Of these, 75% (n = 3) were asymptomatic. One patient with recurrence reported occasional nausea and emesis but has not required additional surgery. CONCLUSIONS: Release of the ligament of Treitz with duodenal lowering results in resolution of symptoms in 75% of patients. This operative approach may be considered before performing more complex operations for SMAS.

Can superior mesenteric artery syndrome really be treated surgically?

AIM: Superior mesenteric artery (SMA) syndrome is a rare reason of small bowel obstruction (SBO). It is a complicated sickness. We aim to analyze the diagnosis, clinical presentation, SMAS management and postoperative outcomes after laparoscopic duodenojejunostomy. MATERIAL AND METHODS: A total of 19 patients who were diagnosed with SMAS and did not respond to the traditional treatment between January 2010 and November 2017 in Afyon Health Sciences University Hospital were included in the study. RESULTS: Their average age was 22.3 years (17-31 years). Number of males and females were 6 and 13, respectively. Clinical presentations of patients are as follow: 14 patients were referred to as postprandial distress syndrome, 3 were unexplained weight loss, and 2 were gastroesophageal reflux disease. Considering CT angiography findings, 14 patients had duodenal dilatation. The mean aortamesenteric angle was 10.6 mm. The mean of aorta-SMA distance was 5.1 mm. The mean hospital stay and follow-up times were 3.7 days and 40.2 months, respectively. No morbidity or mortality was found within patients. Preoperative, postoperative 6th month and postoperative 12th month CONUT scores were 9.1, 3.7, and 0.8, respectively. CONCLUSIONS: Laparoscopic duodenojejunostomy can be performed safely to the patients who do not benefit from conservative treatment. KEY WORDS: Aortamesenteric angle, Duodenojejunostomy, Weight loss.

[Diagnosis and treatment of superior mesenteric artery compression syndrome secondary to chronic constipation (Lee's triad syndrome)].

Objective: To summarize the experience of diagnosis and treatment of superior mesenteric artery compression syndrome (SMACS) secondary to chronic constipation according to the concept of Lee's triad syndrome. Methods: The concept of Lee's triad syndrome: (1) clinical symptoms: triad of constipation, malnutrition, upper gastrointestinal obstruction (vomiting, difficulty in eating); (2) anatomical manifestations: with triple anatomy anomaly of transverse colon sagging, elevated spleen flexure, and mesentery arterial compression; (3) treatment: with triple treatment of enteral nutrition support, chest-knee posture and fecal microbiota transplantation. A descriptive cohort study was performed. According to Lee's triad syndrome criteria, clinical data of 78 patients with superior mesenteric artery compression syndrome secondary to chronic constipation in the Tenth People's Hospital of Tongji University and General Hospital of Eastern Theater Command from June 2004 to November 2018 were prospectively collected, including basic information, symptoms and signs, imaging findings, nutritional indicators, gastrointestinal quality of life index (GIQLI) and Wexner defecation score. The above parameters based on Lee's triad syndrome criteria were followed up and recorded at 1, 3, 6, 12 months after comprehensive treatment. Results: All the patients had Lee's triple symptoms of constipation, malnutrition, upper gastrointestinal obstruction (vomiting, eating difficulties), and triple anatomy anomaly of transverse colon sagging, elevated spleen curvature, and mesentery arterial compression before treatment. After triple treatment of enteral nutrition support, chest-knee posture, and fecal microbiota transplantation, 69 (88.5%) patients had a significant improvement of symptoms, and 9 patients had no significant improvement of symptoms and then eventually received surgery. The 69 cases without operation received follow-up for 12 months. All the patients eventually returned to normal eating, and upper gastrointestinal angiography and superior mesenteric artery imaging showed duodenal compression disappeared. After 1 month, the constipation-related indexes were improved. After 12 months, the number of autonomous defecation per week increased from 1.0±0.8 to 5.0±1.6 (P<0.001). The GIQLI score increased from 52.7±8.5 to 93.2±7.5 (P<0.001), and the Wexner score decreased from 19.1±2.5 to 6.2±2.1 (P<0.001). After 1 month, nutritional indexes were improved gradually. After 12 months, the BMI increased from (17.9±1.8) kg/m(2) to (21.0±1.3) kg/m(2), total protein increased from (65.2±5.7) g/L to (68.3±4.2) g/L, albumin increased from (32.1±5.1) g/L to (40.4±3.0) g/L, prealbumin increased from (163.2±53.7) mg/L to (259.1±45.6) mg/L, fibrinogen increased from (1.9±0.5) g/L to (2.4±0.5) g/L, whose differences were statistically significant (all P<0.001). Upper gastrointestinal angiography and superior mesenteric artery imaging showed duodenal compression were relieved. The angle between superior mesenteric artery and abdominal aorta increased from (17.4±3.8)° to (37.8±5.8)° (t=-22.26, P<0.001). Conclusion: When patients with SMACS secondary to chronic constipation have Lee's triple symptoms and triple anatomy anomaly, the triple combination treatment of enteral nutrition support, chest-knee posture and fecal microbiota transplantation should be applied.

Rare bowel emphysema with superior mesenteric artery syndrome after surgery.

Superior mesenteric artery syndrome, a rare cause of duodenal obstruction, occasionally requires surgery. Bowel emphysema might also require surgery and might be an ominous sign of a serious condition. We report the case of a 69-year-old Japanese man with left pneumothorax who was also diagnosed as having bowel emphysema and superior mesenteric artery syndrome simultaneously without serious infection after surgery for the pneumothorax. Following gastric decompression via a nasogastric tube, his general condition resolved quickly with no need for surgical intervention. Prompt and precise diagnosis by computed tomography and both adequate judgment and treatment can avoid surgery in such cases.

Tratamento cirúrgico da síndrome da artéria mesentérica superior em paciente idosa / Surgical treatment of mesenteric superior artery syndrome in an older woman

A síndrome da artéria mesentérica superior (SAM), ou síndrome de Wilkie, é uma afecção rara que acomete geralmente indivíduos jovens do sexo feminino. Caracterizada pela obstrução parcial ou completa do duodeno devido à compressão pela artéria mesentérica superior anteriormente, e pela aorta, posteriormente. Apresentamos um caso não usual de SAM, em paciente idosa, diagnosticado através de história clínica e imagem tomográfica característica. Indicou-se manejo cirúrgico de derivação jejunal e anastomose com duodeno acima da obstrução, na ciência do risco do procedimento. A paciente apresentou excelente evolução e encontra-se assintomática. O diagnóstico precoce, através de uma anamnese adequada e uso dos critérios radiológicos já definidos em literatura, podem afetar positivamente o desfecho dos pacientes portadores em qualquer faixa etária. (AU)

Superior mesenteric artery syndrome (SMAS), or Wilkie syndrome, is a rare condition that usually affects young women. It is characterized by partial or complete obstruction of the duodenum due to compression by the superior mesenteric artery anteriorly and by the aorta posteriorly. We report an unusual case of SMAS in an older woman diagnosed using clinical history and tomographic imaging. Surgical management consisting of jejunal shunt and anastomosis with duodenum above the obstruction was indicated despite the risk of the procedure. The outcome was excellent, and the patient is asymptomatic. Early diagnosis using adequate anamnesis and radiological criteria defined in the literature may positively influence the outcome of patients from any age group. (AU)

The Superior Mesenteric Artery: From Syndrome in the Young to Vascular Atherosclerosis in the Old.

This review is directed at increasing awareness of two diverse rare upper gastrointestinal problems that occur at opposite ends of the age spectrum and are difficult to diagnose and treat. The Superior Mesenteric Artery Syndrome (SMAS) likely involves a young patient, especially female, and is especially associated with rapid weight loss, resulting in relative strangulation of the duodenum by a narrowing of the angle between the Superior Mesenteric Artery (SMA) and the aorta. On the other hand, atherosclerosis of the SMA is associated most likely with postprandial upper intestinal ischemia and abdominal pain occurs in the elderly at high risk for cardiovascular (CV) disease. Medical management of the SMAS in the young involves good alimentation and weight gain to overall increase the intestinal fat pad. Medical management of SMA atherosclerotic ischemia in the elderly is directed at marked lipid lowering with atherosclerotic plaque stabilization or even regression. If needed, surgery for SMAS can be attempted laparoscopically with duodenojejunoscopy which is the most popular procedure but there are also more conservative possibilities that avoid division of the duodenum. In addition, sometimes direct vision is needed to successfully operate on SMAS. If surgery is needed for SMA atherosclerotic ischemia, it is usually attempted endoscopically with angioplasty and stent placement. Most important, in the case of these two rare clinical entities, is that the clinician have a suspicion of their presence when indicated so that the young or old patient can be spared unnecessary suffering and return to good health in a timely fashion.

Superior Mesenteric Artery Syndrome Following Augmentation Cystoplasty.

Superior mesenteric artery syndrome (SMAS) is a rare condition of external duodenal compression in the angle between the superior mesenteric artery and aorta. We report a case of SMAS following augmentation cystoplasty in a young patient.