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.

Vascular compression of Duodenum: A case report of superior mesenteric artery syndrome.

Superior mesenteric artery (SMA) passes over the third part of duodenum and can cause its obstruction when the aorto-mesenteric angle is hyper acute. Although the condition is rare, the diagnosis can be made using radiography and the treatment consists of division of ligament of Treitz and duodenojejunostomy. We present a case of SMA syndrome in an elderly lady, who was managed successfully with surgery.

Surgical treatment of abdominal compression syndromes: The significance of hypermobility-related disorders.

Case reports and systematic studies of the most common hypermobility-related disorders, hypermobile Ehlers-Danlos syndrome (hEDS), and hypermobility spectrum disorder (HSD) typically describe gastroenterological symptoms and complaints attributed to structural malfunction, autonomic dysfunction, or inflammation of the gastrointestinal tract. However, abdominal compression syndromes (CS) may also contribute to pain and dysfunction in these individuals and be the leading pathology given symptoms significantly reduce or cease after decompressive surgery. Arising not only in the abdomen and causing pain (median arcuate ligament syndrome [MALS] and superior mesenteric artery syndrome [SMAS]), CS also occur in the retroperitoneum and the pelvis (nutcracker syndrome and May-Thurner syndrome), these latter conditions causing chronic pelvic congestion syndrome (PCS). Here, we report primarily on our experience of the assessment and management of MALS and SMAS in a cohort of cases with a surprising prevalence of HSD and hEDS. To our knowledge, this is the first cohort report of its kind in hEDS, HSD, and CS. We recommend that CS are considered in hEDS and HSD individuals with gastrointestinal and other painful complaints within the "belt" area. These CS can be identified using functional ultrasound duplex examination in experienced hands, and in appropriate cases stabilizing surgery can substantially improve quality of life.

Superior Mesenteric Artery Syndrome in association with Abdominal Tuberculosis: An Eye Opener.

Superior Mesenteric Artery Syndrome (SMAS) is a rare clinical entity presenting as acute or chronic upper gastrointestinal obstruction. It occurs due to compression of third part of duodenum between abdominal aorta and overlying superior mesenteric artery caused by a decrease in angle between the two vessels. Rapid loss of retroperitoneal fat, in conditions leading to severe weight loss is the main factor responsible for this disorder. Superior mesenteric artery syndrome in association with abdominal tuberculosis has not been reported earlier to the best of our knowledge. Therefore, an unknown cause (SMAS) of upper gastrointestinal obstruction in a patient of abdominal tuberculosis is being presented for the first time through this case report. An imaging diagnosis of SMAS was made on contrast enhanced CT abdomen which also confirmed the clinical suspicion of abdominal tuberculosis in the patient. The patient was managed conservatively and recovered without requiring any surgical intervention for the obstructive symptoms.

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.

A rare cause of upper gastrointestinal system obstruction: Superior mesenteric artery syndrome (Wilkie's syndrome); two different case reports.

Superior mesenteric artery syndrome is defined as a collection of clinical symptoms and findings that result from compression of the third part of the duodenum between the aorta and the superior mesenteric artery. Here, we describe two patients who were diagnosed with superior mesenteric artery syndrome. Two patients, 18 and 38 years old, respectively, presented to our clinic with complaints of nausea, vomiting, and weight loss. Computed tomography scans of both patients supported diagnoses of superior mesenteric artery syndrome. The 18-year-old patient recovered with conservative treatment. However, our 38-year-old patient did not recover with conservative treatment and required two surgeries. In the first operation, duodenal release with Treitz's ligament dissection and pyloroplasty were performed because of concomitant hypertrophic pyloric stenosis. Because the patient exhibited gastroparesis and gastric ptosis after the first operation, subtotal gastrectomy and Roux-n-Y gastrojejunostomy were performed in the second operation. No complications were observed during follow-up after the second operation. Superior mesenteric artery syndrome should be considered in the differential diagnosis of patients with nausea, vomiting, and weight loss of unknown cause. During treatment, weight-gaining conservative approaches should be attempted initially, but surgical treatment should not be excessively delayed in patients who do not respond to medical treatment.

Superior mesenteric artery syndrome following spine surgery in idiopathic adolescent scoliosis: a systematic review.

Superior mesenteric artery syndrome (SMAS) is a rare and unpredictable complication after correction spine surgery for adolescent idiopathic scoliosis (AIS). The management of this condition is poorly investigated, with controversial outcomes. This investigation systematically reviewed current evidence on pathogenesis, risk factors, management, and outcomes of SMAS following correction spine surgery for AIS. The present systematic review was conducted according to the 2020 PRISMA statement. All the included investigations reported SMAS presentation following scoliosis correction surgery in AIS. 29 articles with 61 eligible patients were included in this review. The mean age of the patients was 15.8 ± 7.2 years. The mean weight was 45.3 ± 8.0 kg, the mean height 159.6 ± 13.6 cm, and the mean BMI 16.5 ± 2.9 kg/m2. The mean duration of the treatment for SMAS was 21.6 ± 10.3 days. The mean interval between spine surgery and symptoms of SAMS was 69 days, with high between-studies variability (3 days to 4 years). Prompt identification of risk factors and an early diagnosis are necessary to manage SMAS and reduce the risk of complications. Additional investigations are required to establish risk factors and diagnostic criteria.Level of evidence Level IV, systematic review.

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.

Single Anastomosis Sleeve Jejunal Bypass in Acute Mesenteric Artery Syndrome: A Case Report.

Superior mesenteric artery syndrome is a rare vascular compression syndrome in which the duodenum is compressed between the aorta and the overlying superior mesenteric artery. This condition is often chronic and secondary to cachexia. It can trigger further weight loss due to the subsequent proximal intestinal obstruction, causing a positive feedback loop. We report a case of acute presentation of superior mesenteric artery syndrome, complicated by gastric necrosis and treated surgically using the principles of a novel bariatric procedure.

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.

Association between underweight status or low body mass index and the risk of developing superior mesenteric artery syndrome following scoliosis corrective surgery in pediatric patients: a review of the literature.

Superior mesenteric artery (SMA) syndrome is the compression of the third portion of the duodenum between the abdominal aorta and the superior mesenteric artery. Although multifactorial, the most frequent cause of SMA syndrome is significant weight loss and cachexia often induced by catabolic stress. SMA syndrome resulting from scoliosis surgery is caused by a reduction of the aortomesenteric angle and distance. Risk factors include rapid weight loss, malnutrition, and a rapid reduction in the mesenteric fat pad and are the most common causes of a decrease in the aortomesenteric angle and distance. Surgically lengthening the vertebral column can also lead to a reduction of the aortomesenteric distance, therefore, has been identified as a risk factor unique to spinal surgery. Despite a reported decline in SMA syndrome cases due to improved surgical techniques, duodenal compression is still a risk and remains a life-threatening complication of scoliosis surgery. This article is a cumulative review of the evidence of being underweight or having a low body mass index as risk factors for developing SMA syndrome following surgical scoliosis instrumentation and correction.

Understanding Superior Mesenteric Artery Syndrome: Etiology, Symptoms, Diagnosis, and Management.

Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, poses a diagnostic challenge due to its rarity and varied clinical manifestations. This review explores the syndrome's etiology, symptoms, diagnostic challenges, and management strategies. Symptoms range from early satiety to severe abdominal pain, often leading to malnutrition. Diagnosis involves a thorough gastrointestinal evaluation and various imaging modalities. Management includes medical interventions like nasogastric decompression and nutritional support, along with surgical interventions such as duodenojejunostomy. A thorough understanding of SMA syndrome's complexities is crucial for its timely diagnosis and effective management, especially considering its potential overlap with other gastrointestinal disorders or eating disorders. Further research is needed to enhance understanding and improve patient outcomes.

Superior Mesenteric Artery Syndrome: A Report of a Rare Case.

Superior mesenteric artery (SMA) syndrome is a rare disease in which the third part of the duodenum between the SMA and the abdominal aorta is compressed, leading to small bowel obstruction. Treatment is usually conservative, such as parenteral and nasojejunal nutrition. The pathophysiology includes loss of the retroperitoneal fat layer and subsequent duodenal compression. We present a 53-year-old malnourished female patient who came with complaints of vomiting, constipation, abdominal pain, and distension for four days. This article highlights the diagnostic challenges associated with SMA syndrome and emphasizes the importance of early diagnosis and intervention.

Superior mesenteric artery syndrome: Diagnosis and management.

Superior mesenteric artery (SMA) syndrome (also known as Wilkie's syndrome, cast syndrome, or aorto-mesenteric compass syndrome) is an obstruction of the duodenum caused by extrinsic compression between the SMA and the aorta. The median age of patients is 23 years old (range 0-91 years old) and predominant in females over males with a ratio of 3:2. The symptoms are variable, consisting of postprandial abdominal pain, nausea and vomiting, early satiety, anorexia, and weight loss and can mimic anorexia nervosa or functional dyspepsia. Because recurrent vomiting leads to aspiration pneumonia or respiratory depression via metabolic alkalosis, early diagnosis is required. The useful diagnostic modalities are computed tomography as a standard tool and ultrasonography, which has advantages in safety and capability of real-time assessments of SMA mobility and duodenum passage. The initial treatment is usually conservative, including postural change, gastroduodenal decompression, and nutrient management (success rates: 70%-80%). If conservative therapy fails, surgical treatment (i.e., laparoscopic duodenojejunostomy) is recommended (success rates: 80%-100%).

Superior mesenteric artery syndrome in an 8-year-old boy: a case report.

BACKGROUND: Superior mesenteric artery syndrome (SMAS), also known as Wilkie's syndrome, is a rare disease that mainly presents as intestinal obstruction with a variable incidence ranging from 0.013 to 0.3% (Salehzadeh et al. in Case Rep Gastrointest Med, 2019, 10.1155/2019/3458601). In this syndrome, the third part of the duodenum, located between the abdominal aorta and the superior mesenteric artery, is wholly or partially obstructed (Mosalli et al. in J Pediatr Surg 46:e29-31, 2011). Case presentation An 8-years-old Arabian male patient was admitted to the gastrointestinal department at the pediatric hospital in Damascus, Syria, with complaints of chronic abdominal pain and periodic vomiting since he was two years old. At the age of Seven, he was diagnosed with appendicitis, and after two months of persistent symptoms, he was diagnosed with an umbilical hernia. Finally, after a long time of suffering, he was diagnosed with SMAS and underwent a successful surgical operation. After 3 months of follow-up, he was in good health with no symptoms. CONCLUSION: Whenever a patient complains of vomiting and chronic abdominal pain, intestinal obstruction is suspected, Common differential diagnoses were excluded and the cause is anonymous, we should consider superior mesenteric artery syndrome.

An interesting presentation of a rare association of the Wilkie and Nutcracker syndromes.

Superior mesenteric artery syndrome also known as Wilkie's syndrome (WS) and Nutcracker syndrome (NCS) are 2 rare vascular syndromes characterized by the reduction of the aortomesenteric space. In the WS the reduction of the aortomesenteric angle leads to compression of the third portion of the duodenum. In the NCS the reduced aortomesenteric space usually causes a left renal vein (LVR) entrapment and the clinical presentation is a left flank pain, micro/macrohematuria and proteinuria. Arterial hypertension can be an unusual manifestation of the NCS. Herein, we describe the case of a 37-year-old woman with a history of breast cancer and abdominal subocclusion, with a recent onset of arterial hypertension whose enhanced computed tomography (CT) showed a reduced angle between the abdominal aorta and superior mesenteric artery with the CT findings of both the WS and NCS.

Superior Mesenteric Artery (SMA) Syndrome With Enterocutaneous Fistula in a Young Woman: A Rare Association.

Superior mesenteric artery (SMA) syndrome is also known as Wilkie's syndrome. Sometimes it is the cause of obstruction in the duodenum. In SMA syndrome, the acute angulation of the SMA against the abdominal aorta can prevent duodenal contents from draining into the jejunum (upper small intestine); hence inadequate intake of nutrition leads to weight loss and malnutrition. This is primarily attributed to the loss of intervening pad of mesenteric fat tissue due to various debilitating conditions. Abnormal connections between the intra-abdominal gastrointestinal tracts and skin over the abdomen are known as enterocutaneous fistulas (ECF).  In this case report, a 37-year-old woman with a history of chronic dull pain in the upper abdominal region over the last seven months, who also complained of bloating, infrequent vomiting, nausea, and upper abdominal fullness for the same amount of time, was seen in the emergency room. Her symptoms had deteriorated by the time she approached the hospital. Additionally, she reports having had a foul-smelling, purulent discharge for the past five years right below the umbilicus. Upon close investigation, it was determined to be feces, and it was later discovered to be a low-output enterocutaneous fistula. She describes having an exploratory laparotomy and adhesiolysis for an intra-abdominal abscess and an acute intestinal obstruction caused by adhesions. This case emphasizes the provocation given a diagnosis of SMA syndrome with enterocutaneous fistula and demands increased awareness of this entity. This will ameliorate early identification to reduce immaterial tests and irrelevant treatments.

An atypical cause of vomiting: Coexisting Wilkie's syndrome and a left renal malformation mimicking a nutcracker phenomenon-A case report.

The superior mesenteric artery syndrome and nutcracker phenomenon are rare vascular disorders due to the abnormal development of the superior mesenteric artery stemming from the abdominal aorta with reduced angle (<22°) and resultant compression of the left renal vein and duodenum. It is an underreported entity due to the absence of specific pathognomonic signs. We report the case of a 59-year-old man, admitted for acute bilious vomiting, who underwent a gastroscopy and a computed tomography scan revealing a Wilkie's syndrome associated with a dilated posterior left renal vein communicating with the left ascending lumbar vein without connection with the inferior vena cava mimicking a nutcracker phenomenon.

Superior mesenteric artery syndrome in a 30-year-old male patient: A case report and literature review.

INTRODUCTION AND IMPORTANCE: Due to a decrease in the aortomesenteric angle, the third section of the duodenum can become acutely or chronically compressed in the superior mesenteric artery syndrome (SMAS). CASE PRESENTATION: A 31-year-old male patient complained of one-year-long recurrent postprandial abdominal pain, periumbilical, intermittent, and colicky. The pain increased in severity in the last 4 months and was relieved only with self-induced vomiting and partially with the knee-to-chest position. A CT scan was done and is most consistent with superior mesenteric artery syndrome. The patient was admitted to the operating room and underwent a successful laparoscopic duodenectomy of the third part of duodenum followed by duodenojejunostomy. CLINICAL DISCUSSION: When conservative therapy fails, an open duodenojejunostomy is traditionally advised. A less invasive option that has been documented in up to 10 cases is laparoscopic duodenojejunostomy. We discuss the research on this issue and demonstrate our surgical method on one patient. CONCLUSION: Even if there has been just a modest amount of weight loss, SMAS should be taken into account whenever a sudden observation of gastrointestinal obstruction symptoms is noted in patients with susceptible conditions such as low body weight.

Superior Mesenteric Artery Syndrome as a Rare Cause of Postoperative Intractable Vomiting: A Case Report.

Background: Superior mesenteric artery syndrome is a very rare cause of proximal intestinal obstruction. The objective of this clinical case report is to highlight that this unusual condition can occur in the early postoperative period and medical management may completely cure the condition. Clinical Case: A middle-aged female who was being treated for pulmonary tuberculosis underwent exploratory laparotomy with limited ileal resection and a loop ileostomy for multiple ileal perforations. Postoperatively, she was restarted on anti-tubercular drugs (ATD) but developed a drug reaction, recurrent bilious vomiting, and jaundice and ATD had to be stopped. But her vomiting did not abate and she progressively developed septicemia. An abdominal CT scan diagnosed Wilkie's syndrome, and she was managed non-operatively by decubitus, parenteral nutrition, and nasojejunal tube feeding supplemented with prokinetics and antibiotics. But her sepsis did not resolve. Intraoperative histopathology suggested Candida infection, and she recovered only after systemic antifungal therapy. Discussion: Debilitation conditions like tuberculosis cause weight loss and loss of intra-abdominal fat pad, which is known to precipitate SMA syndrome. However, its presentation in the early post-operative period is rare. Symptoms may vary from non-specific abdominal fullness and weight loss to features of acute bowel obstruction. CECT of whole abdomen can help in confirming the diagnosis. SMA syndrome is often not considered in differential diagnosis and can delay treatment. Medical management is the mainstay treatment option, although surgery is reserved for cases, which fail medical treatment. Conclusion: High suspicion is needed to diagnose SMA syndrome in the postoperative period, which precipitates with intractable bilious vomiting. Medical management may be curative. The precipitating factor for SMA syndrome should also be addressed to improve the overall patient outcome.