Robotic management of superior mesenteric artery syndrome.

Wilkie's syndrome is an unusual cause of upper intestinal obstruction due to mechanical compression of the superior mesenteric artery (SMA) to the duodenum, with nonspecific symptoms, whose diagnosis is confirmed by angiotomography. Initially, the treatment is conservative to regain weight and restore mesenteric adipose tissue, associated with postural changes of the patient. If this fails, surgical treatment is indicated, being laparoscopic duodenojejunostomy described as the gold standard. Robotics' assistance is feasible and safe to carry out the procedure. We present the case of a 21-year-old male patient who comes with stabbing abdominal pain and persistent postprandial vomiting that has caused weight loss of 11 kilograms in the last 2 years without apparent cause, associated with gastroesophageal reflux. During the procedure, we evidenced open diaphragmatic pillars and duodenal compression due to SMA, and robotic-assisted laparoscopic hyatoplasty + Nissen fundoplication + duodenojejunostomy were performed without complications, with excellent post-surgical results.

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.

An uncommon cause of sub-acute intestinal obstruction in young adult: Wilkie's syndrome.

Abstract: Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, is a rare disease presenting as an acute abdomen. It has a clinical presentation similar to intestinal obstruction and is often missed during diagnosis. Reduced weight leading to loss of fat pad between SMA and aorta is the main pathophysiology. Diagnosis is made through barium meal and CT scan. Conservative management remains the treatment of choice; however, surgery is opted for in refractory cases. Key Clinical Message: Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, is a rare disease presenting as an acute abdomen with clinical features similar to intestinal obstruction. This is a case of SMA syndrome in an adult male with a decrease in aortomesenteric angle, with no predisposing condition.

Misdiagnosed superior mesenteric artery syndrome due to diabetic gastroparesis. Case report and literature review.

INTRODUCTION: Superior mesenteric artery syndrome (SMAS) is a rare cause of upper intestinal obstruction. This occurs due to duodenal compression between the superior mesenteric artery and the aorta. Anatomical alterations, eating disorders, after some surgical procedures, and trauma are frequent causes of this rare syndrome. Diabetes is a highly prevalent disease that can cause gastroparesis in up to 12 %. Its association with SMAS is extremely rare and challenging to identify. CASE PRESENTATION: A 32-year-old man experienced nausea and vomiting after diagnosis and treatment for type II diabetes. He was treated for diabetic gastroparesis for 2 years without improvement until he lost 40 kg of weight. After imaging studies, a distance between the superior mesenteric artery and the aorta of 5.3 mm and an angle of 17 degrees were detected, corroborating the diagnosis of SMAS syndrome. Due to medical failure, surgical treatment via duodenojejunostomy was performed. DISCUSSION: Diabetes is a very prevalent disease in the world population that can cause gastrointestinal symptoms. In our patient, diabetic gastroparesis delayed the diagnosis of SMAS until severe symptoms of upper intestinal obstruction and significant weight loss occurred. In our patient, due to medical failure, surgical treatment significantly improved his symptoms and stopped his weight loss. CONCLUSION: Superior mesenteric artery syndrome is a rare syndrome, and challenging to differentiate from diabetic gastroparesis. Delays in management may result in excessive weight loss. Surgical treatment can improve symptoms and weight loss.

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.

Duodenojejunostomy following failed gastrojejunostomy in superior mesenteric artery syndrome: A case report.

INTRODUCTION: Superior mesenteric artery (SMA) syndrome is a rare duodenal-vascular anatomic disorder leading to external compression on the duodenum. The first step of treatment usually is conservative, and in the case of failure, surgical management is the treatment choice. Treatment success with duodenojejunostomy after failure in gastrojejunostomy can show the uniqueness of this article. CASE PRESENTATION: A 14-year-old boy came to our hospital with a complaint of epigastric pain, nausea, bilious vomiting, and weight loss since 6 months ago. Conservation therapy and laparotomic Braun anastomosis and gastrojejunostomy was performed due to the SMA syndrome diagnosis 2.5 months before the admission. At our hospital, an alteration of gastrojejunostomy by duodenojejunostomy employing a diamond-shaped anastomosis between the third portion of the duodenum (D3) and a part of jejunum that was placed 15 cm away from the ligament Treitz was done. A significantly dilated stomach and the first three parts of the duodenum were observed during the procedure. After the second surgical intervention, the general condition of the patient dramatically improved. CLINICAL DISCUSSION: Conservative treatment, including nasogastric tube decompression, postural changes, and nutritional support with hyperalimentation, has a variable success rate. However, in some cases, surgery may be necessary. Surgeons prefer laparoscopic duodenojejunostomy due to its outstanding success rate, ranging from 80 % to 100 %. But, in some case reports it is suggested that gastrojejunostomy could be done in cases with severe duodenal dilation instead of duodenojejunostomy. The initial gastrojejunostomy failed because of ongoing symptoms, which was finally revised with a duodenojejunostomy. CONCLUSION: It is suggested to use duodenojejunostomy after failure of gastrojejunostomy or it can be employed as the first surgical option even in cases with severe dilation. Because it is a more efficient correction with fewer complications than gastrojejunostomy.

Gastrointestinal Complications of Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-Like Episodes (MELAS) Syndrome Managed By Parenteral Nutrition.

MELAS - an acronym for mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes - is a multiorgan disease caused by a mutation in mitochondrial DNA (mtDNA). Its clinical manifestations are highly variable; mainly stroke-like episodes, seizures, recurrent headaches, or muscle weakness. However, gastrointestinal complications such as chronic intestinal pseudo-obstruction (IPO), pancreatitis, gastroparesis and hepatopathy are also common. In this report we describe a young patient with gastrointestinal complication of MELAS which led to superior mesenteric artery syndrome (SMAS). It is rare but not surprising combination and should be considered in cases with significant weight loss and resistance to symptomatic treatment. The optimal energy support is the main pillar of the treatment. LEARNING POINTS: Gastrointestinal complications of MELAS such as chronic intestinal pseudo-obstruction, pancreatitis and gastroparesis can lead to undernutrition.Superior mesenteric artery syndrome is a rare condition but should be considered in cases with significant weight loss and resistance to symptomatic treatment.Optimal caloric intake and energy support can improve the condition of patients with MELAS.

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.

Idiopathic Megaduodenum in a Teenager: A Case Report.

Megaduodenum is a rare clinical syndrome characterized by significant duodenal dilation, elongation, and hypertrophy. Given its rarity and nonspecific clinical manifestations, megaduodenum may be misdiagnosed, leading to delays in surgical care and increased morbidity. We describe a case of idiopathic megaduodenum in a teenage Caucasian female, who presented with a five-year history of halitosis, recurrent belching, bloating, nausea and vomiting, and postprandial epigastric abdominal pain. She was diagnosed with megaduodenum by dramatic findings on contrast radiography. She developed a duodenal volvulus necessitating emergency exploratory laparotomy, during which a duodenal plication and a side-to-side duodenojejunostomy were performed. Exploratory laparotomy and histopathological analysis were unrevealing of any definitive abnormalities to explain her megaduodenum. Postoperatively, she developed two early small bowel obstructions, both from subsequent adhesions requiring repeat laparotomy with adhesiolysis. She has subsequently recovered without incident. Diagnosis and accurate classification of megaduodenum requires surgical exploration with a full-thickness biopsy and subsequent histopathologic analysis to rule out obstructive or functional disorders of the duodenum. Treatment of megaduodenum depends on the underlying cause and degree of duodenal distention. It is crucial that clinicians are knowledgeable of the various surgical options, their indications, and the potential postoperative complications that may arise.

Superior Mesenteric Artery Syndrome: A Misdiagnosed Disorder.

The superior mesenteric artery syndrome also known as Wilkie's syndrome or Benign duodenal stasis, is a condition that occurs when the third duodenum is compressed between the superior mesenteric artery anteriorly and the abdominal aorta posteriorly following the disappearance of the perivascular fatty tissue. It can emulate the symptoms of an upper abdominal obstruction. This case involves a 9-year-old female patient experiencing food vomiting that did not improve with medication. Radiological work-up, which included an abdominal X-ray and a CT angiography, led to the diagnosis of extrinsic and incomplete obstruction of the third duodenum by the aorto-mesenteric clamp. Unfortunately, this diagnosis is rarely made. The purpose of this article is to provide a review of the etiology, clinical presentation, and imaging findings of this syndrome.

Co-occurrence of ectopic pancreas and superior mesenteric artery syndrome in a child: A case report and literature review.

Ectopic pancreas refers to pancreatic tissue that lacks vascular or anatomical connection with the main body of the pancreas. Superior mesenteric artery syndrome involves the compression of the third part of the duodenum between the aorta and the superior mesenteric artery. We present a case of a 12-year-old female with a 4-year history of recurrent episodes of severe epigastric pain, improved by sitting and leaning forward, and occurred monthly for nearly 48 h. Radiological findings confirmed the diagnosis of superior mesenteric artery syndrome. However, further investigation revealed the presence of ectopic pancreas. Surgical intervention successfully treated both conditions. The patient presented with severe abdominal pain, and although there was a diagnosis of superior mesenteric artery syndrome, the pain was not entirely attributable to this condition, as there was no correlation with food intake, and the degree of obstruction was mild. Further investigations revealed the presence of ectopic pancreas, which provided an explanation for the severity of the pain. This case highlights the need for further research on the potential association between superior mesenteric artery syndrome and ectopic pancreas.

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.

Superior Mesenteric Artery Syndrome and Nutcracker Syndrome as the Presentation of Crohn's Disease in a Young Patient: A Case Report and Review of Literature.

Superior mesenteric artery syndrome and nutcracker syndrome are rare vascular complications most often seen after marked weight loss caused by compression of the duodenum and left renal vein between the superior mesenteric artery and the aorta, respectively. The coexistence of superior mesenteric artery syndrome and nutcracker syndrome has been rarely reported. Herein, we present the case of a 16-year-old male with intermittent periumbilical abdominal pain, bilious vomiting, and weight loss who was found to have both of these vascular complications of significant weight loss as the initial presentation of Crohn's disease. This report provides insight into the diagnosis and treatment of these syndromes while highlighting the importance for practitioners to keep vascular complications on their differential diagnosis of vomiting and abdominal pain in patients with Crohn's disease.

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.

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.

Síndrome de la arteria mesentérica superior o Síndrome de Wilkie. Presentación de tres casos clínicos y revisión de la literatura

Objetivo: Presentar una causa rara de obstrucción duodenal como es el sindrome de arteria mesentérica superior (SAMS). Material y Método: se presentan tres casos clínicos de sindrome de SAMS. Resultados: el tratamiento médico y nutricional fue exitoso en los primeros dos casos, mientras que en el último fue necesario realizar una derivación quirúrgica. Discusión: en este sindrome, el ángulo aortomesentérico normal se encuentra disminuído, causando la compresión de la tercera porción del duodeno entre la aorta y la arteria mesentérica superior. Esto determina una obstrucción duodenal que se ve más frecuentemente en pacientes con pérdida importante y rápida de peso. Esto determina alteraciones hidroelectrolíticas y desnutrición severas. El tratamiento médico consiste en soporte nutricional y corrección de anormalidades hidroelectrolíticas. Los procedimientos quirúrgicos se reservan frente a los casos de falla del tratamiento médico. Conclusión: el SAMS es una entidad poco frecuente y un reto diagnóstico. El cuadro clínico puede ser grave con desnutrición severa y trastornos hidroelectrolíticos que ponen en riesgo la vida del paciente. El soporte hidroelectrolítico y nutricional, junto a la duodenoyeyunostomía, son los tratamientos de elección.

Aim: To present a rare cause of duodenal obstruction known as the superior mesenteric artery syndrome (SMAS). Material and Method: we present three cases of SMAS. Results: medical and nutritional treatment were effective in the first two cases while in the last case, a derivative procedure was necessary. Discussion: in this syndrome, the normal aortomesenteric angle is narrowed, causing compression of the third portion of the duodenum between the aorta and the superior mesenteric artery. This leads to duodenal obstruction more frequently observed in patients with severe and fast weight loss. Medical treatment consists in nutritional support and correction of hydro electrolyte imbalance. Surgical procedures are reserved for cases with failure of medical treatment. Conclusion: SMAS is a rare entity and a diagnosis challenge. Clinical setting may be severe with extreme malnutrition and life-threatening dehydration and electrolyte disorder. Nutritional support, hydro electrolyte correction, combined with duodenojejunostomy, if necessary, are the treatments of choice.