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.

[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.

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 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.

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.

[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.

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.

A Traumatic Quinceañera: Acute Superior Mesenteric Artery Syndrome in an Adolescent Girl.

ABSTRACT: A 15-year-old girl presented with 3 days of progressive abdominal distention, pain, and bilious hematemesis. Her symptoms began after her quinceañera, during which she wore a tight corset. On examination, she was thin and had significant abdominal distention and pain. A computed tomography revealed a massively dilated stomach and proximal duodenum to the region of the superior mesenteric artery (SMA) with distal decompression. An upper gastrointestinal fluoroscopy demonstrated marked dilation of the stomach through the mid third portion of the duodenum with distal decompression and an associated linear compression on her duodenal wall. We believe that she developed acute SMA syndrome. Superior mesenteric artery syndrome is a partial bowel obstruction caused when the third portion of the duodenum is compressed as it passes between the SMA and the aorta. Although the SMA syndrome is most commonly described as a condition associated with chronic, severe weight loss resulting in a narrowing of the SMA to aorta angle and subsequent duodenal compression, it can present acutely from causes such as a postoperative complication, blunt trauma, or external compression. Previously described acute SMA syndrome from external compression has been the result of medically necessary causes, such as body casting. In this case, the tight gown was likely the inciting factor for her development of SMA syndrome; however, she was placed at high risk for the condition by being underweight at baseline and experiencing food restriction for several days preceding her quinceañera. She was treated conservatively with nasogastric decompression and parenteral nutrition, and has since completely recovered.

Small Bowel Obstruction in an Adolescent Boy With No Apparent Risk Factors: A Rare Case of Superior Mesenteric Artery Syndrome.

ABSTRACT: Superior mesenteric artery syndrome (SMAS) is an uncommon condition that can lead to upper gastrointestinal obstruction. Although SMAS is a rare diagnosis, our patient, a 13-year-old adolescent boy with no relevant medical or surgical history, had a classical presentation of the disorder. In this article, we discuss the risk factors, common presentation, and treatment options of SMAS, and why it is important to consider in the differential diagnosis of a pediatric patient presenting with bilious emesis and no other risk factors for intestinal obstruction.

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.

[Autistic female with anxiety disorder and superior mesenteric artery syndrome]. / Autisme bij een jonge vrouw met een angststoornis en het A.-mesenterica-superiorsyndroom.

An anxiety disorder can manifest in multiple ways. We saw a 21-year-old woman with an anxiety disorder in combination with superior mesenteric artery syndrome. For we know, this is the first description of this combination. There was also hypersensitivity to certain foods and rigid thinking patterns, and an autism spectrum disorder was discovered later. The recognition of the influence of autism on comorbid disorders is important in order to create an individual adapted treatment protocol.

Superior mesenteric artery syndrome coexists with Nutcracker syndrome in a female: a case report.

BACKGROUND: Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome or Benign duodenal stasis, is a rare benign disease. It could threaten the life if the manifestation is severe and the treatment is inappropriate. In the patients with SMA syndrome, the third portion (transverse part) of the duodenum is compressed externally between the SMA and abdominal aorta (AA) leading to duodenal stasis and gastrointestinal obstruction. SMA syndrome may rarely combine with Nutcracker syndrome when left renal vein (LRV) was compressed between SMA and AA. CASE PRESENTATION: A 32-year-old female patient presented with complaints of gradually severe bloating, epigastric pain, left flank ache, nausea and occasional vomiting of 1 month's duration. The epigastric and left flank ache was aggravated when the patient was supine and relieved in a prone or left lateral decubitus. The abdominal bloating was associated with early satiety. The vomiting always started 40 min after meal. The patient gave a history of urine stone with drotaverine hydrochloride tablets treatment for two weeks before the gastrointestinal symptoms arising. The patient had no significant surgical history, but had a rapid weight loss of approximately 10 kg with a body mass index (BMI) from 21 kg/m2 to less than 18 kg/m2 over the last two months. An abdominal examination revealed upper abdominal tenderness and distention. The urine routine examination showed no significant abnormality. The findings of initial blood tests and other laboratory investigations were unremarkable. CONCLUSIONS: This case reports a female patient with SMA syndrome with Nutcracker syndrome predisposed by Antispasmodics. We highlight the importance of the combination therapy of long-term nutritional supporting and prokinetic agents. Rehabilitating practice after discharge is beneficial to reduce recurrence.

Iatrogenic superior mesenteric artery syndrome.

We have carefully read the article "Superior mesenteric artery syndrome: an uncommon cause of intestinal obstruction" by José Barquín-Yagüez et al. and we would like to report one case with the same diagnosis but with another etiology.

Superior mesenteric artery syndrome after lung transplantation: a rare early gastrointestinal complication of lung transplantation.

Gastrointestinal complications sometimes occur after lung transplantation and remain a cause of postoperative morbidity. Superior mesenteric artery syndrome is caused by the compression of the duodenum by the superior mesenteric artery, but few reports have described superior mesenteric artery syndrome after lung transplantation. We herein report two cases of superior mesenteric artery syndrome as an early complication after lung transplantation. Both patients were emaciated and had lost weight before transplantation. They also lost an additional 2-4 kg early after transplantation. They were medically treated with enteral nutrition and recovered without recurrence of the syndrome. Since critically ill patients with pulmonary disease usually lose weight both before and early after lung transplantation, superior mesenteric artery syndrome is important to consider in the differential diagnosis of gastrointestinal complications after transplantation.

Sudden Death Due to Undiagnosed Wilkie Syndrome.

A 56-year-old transgender woman with mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes syndrome and diabetes presented to hospital with headaches and experiencing with malnutrition. She was agitated and refused medical and physical assistance. Soon after admission, she started to vomit and developed abdominal pain, becoming rapidly unresponsive on the ward after attending the radiology department, and was pronounced deceased. Autopsy revealed a cachectic transgender woman with a grossly distended stomach and proximal duodenum containing 2 L of liquid. The postmortem computed tomography scan showed compression of the duodenum by the superior mesenteric artery, diagnostic of Wilkie syndrome. Superior mesenteric artery syndrome, or Wilkie syndrome, was first described in 1861 by Von Rokitansky. It is an uncommon but well-recognized clinical entity characterized by compression of the third, or transverse, portion of the duodenum between the aorta and the superior mesenteric artery. This results in chronic, intermittent, or acute complete or partial duodenal obstruction. It is a well-recognized complication of anorexia.

Superior mesenteric artery (Wilkie's) syndrome: a rare cause of upper gastrointestinal system obstruction.

Background Superior mesenteric artery syndrome (SMAS) results from the compression of the third part of the duodenum between the aorta and the proximal part of the superior mesenteric artery (SMA). Clinical presentation of SMAS is characterized by the dilatation of the proximal part of the third part of the duodenum. SMAS is a rare cause of the upper gastrointestinal system (UGS) obstruction. In this study, we aimed to present our clinical experience in the treatment of five patients with SMAS, which is a rare clinical condition requiring surgery. Patients and methods The retrospective study included five patients who were treated due to SMAS at our clinic between January 2010 and January 2014. Results All the patients were underweight, with a mean BMI of 15.73 (14-16). The clinical symptoms included epigastric pain after food intake, large volume bilious emesis, early satiety, failure to gain weight, indigestion, esophageal reflux, sense of fullness, and persistent weight loss. SMAS was diagnosed using barium meal studies, upper gastrointestinal endoscopy, abdominal ultrasonography, and CT angiography. Four patients underwent duodenojejunostomy and one patient was managed with gastrojejunostomy. No complication was observed during the postoperative period, and all the patients achieved significant improvement in symptoms. Conclusion SMAS is a rare cause of UGS obstruction, and the diagnosis of SMAS is often delayed. SMAS should be suspected in the differential diagnosis of the patients with unsubstantiated symptoms of persistent nausea, emesis, and significant weight loss.

Minimally invasive surgery for superior mesenteric artery syndrome: a case report and literature review.

INTRODUCTION: Superior mesenteric artery syndrome is a rare condition characterised by nausea, vomiting, postprandial pain, anorexia and early satiety. Conservative management is tried initially, but if this fails, surgery is indicated. There are few reports in the literature concerning superior mesenteric artery syndrome in children, and fewer still managed surgically by minimally invasive means. CASE PRESENTATION: A 12-year-old girl presented with weight loss, early satiety and vomiting after corrective scoliosis surgery. After upper gastrointestinal endoscopy, contrast study and computed tomography imaging, a diagnosis of superior mesenteric artery syndrome was made. Conservative management by nasojejunal feeding failed; therefore, a laparoscopic duodeno-jejunostomy was undertaken. At follow-up, her symptoms had improved. CONCLUSION: This report describes the youngest child to undergo laparoscopic duodeno-jejunostomy for superior mesenteric artery syndrome. Laparoscopic duodeno-jejunostomy appears to be the most widely employed and reliable minimally invasive approach to superior mesenteric artery syndrome with a high success rate and acceptably low complication rate.