[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.

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.

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.

Superior Mesenteric Artery Syndrome in an Adolescent Female with Anorexia Nervosa.

Superior mesenteric artery syndrome (SMAS) is a rare condition that develops from compression of the duodenum between the superior mesenteric artery (SMA) and abdominal aorta. SMAS is an atypical complication of restrictive eating disorders. The SMA is supported by adipose tissue to create an aortomesenteric angle that varies from 25-60 degrees. A reduction in adipose tissue causes narrowing of this angle, and SMAS develops when the aortomesenteric angle is narrow enough that it compresses the distal duodenum passing through. Patients present with small bowel obstructive symptoms. We report a severe case of SMAS in an adolescent female with anorexia nervosa who presented with acute and chronic symptoms of bowel obstruction. Awareness of the association between SMAS and restrictive eating disorders can help guide clinical decision-making and prevent delay of diagnosis and serious 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 and disseminated tuberculosis: "Double troubleË®.

Superior Mesenteric Artery (SMA) Syndrome is a rare condition characterized by compression of the third part of the duodenum between the aorta and superior mesenteric artery due to decreased angle between these two vessels due to loss of intervening pad of fat. Tuberculosis is one of the causes, and its association with it is rare. However, SMA syndrome with significant gastrointestinal symptoms in TB poses a greater challenge in management, particularly in dissemination. Strong clinical suspicion, timely diagnosis and appropriate antituberculosis therapy are the keys to successful management.

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.

Avoidant restrictive food intake disorder emerging during COVID-19 pandemic resulting in superior mesenteric artery syndrome.

The COVID-19 pandemic has significantly increased the prevalence of psychiatric disorders within pediatric populations. However, only a limited number of studies have sought to understand the correlation between the pandemic and increased incidence of eating disorders. This case study highlights the hospital course of an 18-year-old female who presented with restrictive eating patterns and intensive exercise regimen, self-attributed to the COVID-19 pandemic, leading to superior mesenteric artery syndrome. In understanding the patient's avoidant restrictive food intake disorder (ARFID), this case study seeks to inform readers of this newer DSM-V diagnosis with the intent of educating pediatric providers of the severity and long-term impact of this disease. Moreover, the case study highlights the importance of gaining a more holistic view of psychiatric disorders emerging as a result of the COVID-19 pandemic.

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.