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.

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.

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.

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.

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

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

Superior Mesenteric Artery Syndrome Following Scoliosis Surgery: A Systematic Review of Case Reports.

BACKGROUND: Superior mesenteric artery (SMA) syndrome is a well-recognized, rare complication of undergoing surgical correction of a spinal deformity. The objective of this study was to summarize the best available evidence on SMA syndrome treatment after scoliosis surgery. To identify differences, special attention was paid to scoliosis pathology, intervention and SMA syndrome presentation. METHODS: A systematic review of the literature was performed on SMA syndrome following scoliosis surgery. The Web of Science, PubMed, Wanfang (Chinese) and EMBASE databases were systematically searched for articles from January 1971 to October 2020. The main subjects discussed are scoliosis pathology, intervention and SMA syndrome presentation. RESULTS: A total of 32 articles with 52 cases were included in our report. The mean age of patients with SMA syndrome following scoliosis surgery was 14.7 ± 2.9 years and the majority (n = 42, 80.8%) were girls. The most frequently reported scoliosis type was adolescent idiopathic scoliosis (n = 34, 65.4%). The mean postoperative days (POD) (interval between the surgical treatment and the onset of symptoms) was 9.6 ± 9.5 days. Different treatment methods were analyzed in 46 patients. We noted a trend toward a higher percentage of patients with POD < 48 hr in the group with surgical treatment than in the conservative treatment group (P = 0.002). CONCLUSION: In summary, in this systematic review, for the first time, we found that the time of onset of postoperative symptoms can be used as an important reference index for surgical intervention. We also believe that recommendations about the treatment of SMA syndrome following scoliosis surgery should include this finding. Given the lack of robust clinical evidence, these findings warrant verification in a prospective multicenter screening trial.

Unusual presentation of superior mesenteric artery syndrome after trauma.

Superior mesenteric artery (SMA) syndrome is a rare but severe condition. SMA syndrome's association with trauma has been reported to present weeks to months after significant weight loss due to head or spinal cord injury. We present an unusual case of SMA syndrome presenting with obstructive symptoms, which developed immediately after clavicle fracture and was not associated with weight loss. CT of the abdomen showed small bowel obstruction in the third part of the duodenum. CT angiogram of the abdomen confirmed SMA syndrome. The patient was managed conservatively with enteral nutrition via jejunostomy tube. He was discharged after symptoms resolved, and repeat imaging revealed resolution of obstruction. This case emphasises the importance of having SMA syndrome as one of the differential diagnoses for patients presenting with obstructive symptoms after trauma because early diagnosis can be managed with conservative treatment.

[A Case of Superior Mesenteric Artery Syndrome Following Transverse Colon Cancer Surgery].

We reported a case of superior mesenteric artery(SMA)syndrome after decreased body weight and unstable oral intakes due to anastomotic leakage of postoperative transverse colon cancer. A 63-year-old man underwent laparoscopic left hemicolectomy and D3 lymph node dissection with a diagnosis of transverse colon cancer. He had postoperative anastomotic leakage and was discharged after conservative treatment on postoperative day 35. However, he had visited our hospital by the symptom of bowel obstruction caused by anastomotic stenosis and had been admitted twice. After second colonoscopic balloon dilation on postoperative day 129, he was diagnosed as anastomotic perforation and emergency reoperation was performed. In the postoperative course, he repeated vomiting and his body weight decreased and superior mesenteric artery syndrome was diagnosed. He improved after conservative treatment by fasting, a nasogastric intubation and total parenteral nutrition and was discharged 22 days after the diagnosis.

SMA syndrome: management perspective with laparoscopic duodenojejunostomy and long-term results.

BACKGROUND: Superior mesentery artery syndrome (SMAS) is a rare vasculo-anatomic occlusive pathologic entity for which a period of conservative medical management is advocated with surgery reserved for nonresponsive cases. We present our management plan that entails a single admission approach and complete rendering of medical and surgical treatment to the patient on a background of the socioeconomic and cultural trends prevalent in this geographic region. METHODS: A retrospective analysis of 22 cases of SMAS admitted in our health care system who underwent a period of preoperative conditioning followed by laparoscopic duodenojejunostomy from September 2009 to June 2019 was performed. Patients were followed up at regular intervals. RESULTS: The mean follow-up of the cohort was 41.2 months (2-108 months). The median length of stay was 6 days. The mean postoperative stay was 4.13 days. A subgroup of six patients who had severe physiological depletion required a period of preoperative optimisation. Five of the 22 (22.7%) patients suffered from postoperative complications in the form of delayed return of bowel functions. None of the patients had complications more than Clavien-Dindo grade 2 with no mortality. Long-term data are available for 19 patients (86.3%) which showed no symptom recurrence. CONCLUSION: Management of SMAS that entails an antecedent medical therapy followed by surgery can be accomplished in a single admission with good to excellent results in the intermediate and long-term follow-up. Physiologically depleted patients do require a period of intensive preconditioning but on long-term follow-up, they have excellent results.

An extensive posterior circulation infarction secondary to primary hyperthyroidism accompanied with superior mesenteric artery syndrome: A case report and description of patho-physiological association.

INTRODUCTION: Hyperthyroidism-related anterior circulation ischemic events have been well documented; however, posterior circulation infarction is rarely reported, not to mention with superior mesenteric artery syndrome (SMAS), which has never been reported concurrently. We describe, to the best of our knowledge, the first case of hyperthyroidism-related cerebellar infarction accompanied with SMAS. PATIENT CONCERNS: A 22-year-old women presented with palpitation, postprandial vomiting, and acute body weight loss. Enlarged thyroid gland was discovered in physical examination and Graves disease was diagnosed by blood test; therefore, Propylthiouracil and ß-blocker were prescribed. Sudden onset conscious disturbance accompanied with apnea was noted during hospitalization. DIAGNOSIS: Computed tomography (CT) revealed cerebellar infarction with severe cerebellar swelling and tonsil herniation; hence, emergent suboccipital craniotomy and bilateral tonsillectomy were performed. INTERVENTIONS: Nevertheless, persisted poor passage of liquid diet during nasogastric tube feeding was noted after operation. CT of abdomen showed a sharp aorta-SMA angle (15°) and a short distance between aorta and SMA (6 mm) indicating a diagnosis of SMAS. OUTCOMES: After parental nutrition supplement and progressive rehabilitation program, she recovered to a modified Rankin Scale of 3. CONCLUSION: Although rarely reported, hyperthyroidism-related sympathetic hyperstimulation, vasculopathy could result in potentially deadly posterior circulation infarction. Furthermore, SMAS should be considered in the cases of hyperthyroidism with prolonged gastrointestinal symptoms even after treatment and should be treated simultaneously, since SMAS exacerbates depletion of intravascular volume. Further study to clarify the relation between hyperthyroidism and posterior circulation hemodynamic status is suggested.

[Wilkie's syndrome caused by exogenous hyperthyroidism in a patient with primary autoimmune hypothyroidism: a case report and literature review]. / Síndrome de Wilkie secundario a hipertiroidismo exógeno en una paciente con hipotiroidismo primario autoinmune: reporte de un caso y revisión de la literatura.

Wilkie's syndrome or superior mesenteric artery syndrome is an unusual cause of proximal intestinal obstruction, primarily attributed to recent weight loss. We report the case of a 19-year-old woman comes to our clinic and reports weight loss, abdominal pain, nausea, and vomiting. Laboratory tests revealed anemia, hypoalbuminemia, hypomagnesemia, and a suppressed thyroid stimulating hormone secondary to levothyroxine. A barium swallow test showed gastric dilatation, delayed gastric emptying and an axial computed tomography revealed an aortomesenteric angle of 11.7°. Conservative management with total parenteral and enteral nutrition was initiated, being the first-line treatment. In refractory cases surgery is a safe and effective option.

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

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

Minimal weight loss related to a short fasting period causes superior mesenteric artery syndrome in a patient with amyotrophic lateral sclerosis: A case report.

INTRODUCTION: Superior mesenteric artery syndrome (SMAS) is rare cause of small bowel obstruction and is characterized by an extrinsic vascular compression of the duodenum. The most common cause of SMAS is known as rapid and significant weight loss. PATIENT CONCERNS: A 61-year-old man who was diagnosed with amyotrophic lateral sclerosis and maintained a stable diet before admission. When the patient re-started feeding by gastrostomy tube after 5 days of therapeutic fasting due to gastric ulcer caused by gastrostomy tube irritation, he presented postprandial vomiting, abdominal distention, and tachycardia. Since fasting, his weight has been reduced by about 3 kg. DIAGNOSIS: Based on clinical symptoms and radiological findings, diagnose of SMAS was finally made. Abdomen computed tomography confirmed decreased aortomesenteric distance and tubography confirmed gastric and proximal duodenum distension above the compressed part. INTERVENTIONS: We performed jejunal tube insertion and the amount of feeding through the jejunal tube was gradually increased while maintaining parenteral nutrition. OUTCOMES: The presenting symptoms of the patient gradually improved. Follow-up abdomen computed tomography and tubography showed improvement in duodenal narrowing and stomach distension. CONCLUSION: SMAS should be considered when there is an abrupt observation of symptom of gastrointestinal obstruction in patients with predisposing condition such as a low body weight, even if the weight loss is relatively small.

Síndrome de Wilkie secundario a hipertiroidismo exógeno en una paciente con hipotiroidismo primario autoinmune: reporte de un caso y revisión de la literatura / Wilkie's syndrome caused by exogenous hyperthyroidism in a patient with primary autoimmune hypothyroidism: a case report and literature review

RESUMEN El síndrome de Wilkie o de arteria mesentérica superior es una causa poco común de obstrucción intestinal proximal, relacionada a pérdida de peso reciente. Reportamos el caso de una mujer de 19 años que se presenta a la clínica con pérdida de peso, dolor abdominal, nausea y vomito. Los exámenes de laboratorio reportaron anemia, hipoalbuminemia, hipomagnesemia y una hormona estimulante de la tiroides suprimida secundario al uso con levotiroxina. Se realizó una serie esofagogastroduodenal con datos compatibles con dilatación gástrica severa, gastroparesia y una tomografía axial computada reveló un ángulo aortomesentérico de 11,7°. Se inicio manejo conservador a base de nutrición enteral y parenteral total, siendo este el tratamiento de elección. En casos refractarios, la cirugía es una opción segura y efectiva.

ABSTRACT Wilkie's syndrome or superior mesenteric artery syndrome is an unusual cause of proximal intestinal obstruction, primarily attributed to recent weight loss. We report the case of a 19-year-old woman comes to our clinic and reports weight loss, abdominal pain, nausea, and vomiting. Laboratory tests revealed anemia, hypoalbuminemia, hypomagnesemia, and a suppressed thyroid stimulating hormone secondary to levothyroxine. A barium swallow test showed gastric dilatation, delayed gastric emptying and an axial computed tomography revealed an aortomesenteric angle of 11.7°. Conservative management with total parenteral and enteral nutrition was initiated, being the first-line treatment. In refractory cases surgery is a safe and effective option.