Delayed presentation of Wilkie's syndrome after scoliotic curve correction surgery: a case report.

BACKGROUND: Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, is a rare but serious complication following scoliosis correction surgery. It occurs as a result of mechanical compression of third part of duodenum between the SMA and aorta. This condition occurs most commonly in significantly underweight patients with deformities, and usually during the first week following spinal deformity corrective surgeries. The angle between the abdominal aorta and the SMA gets reduced following spinal lengthening during deformity correction surgery causing compression of third part of duodenum resulting in development of SMA syndrome. CASE PRESENTATION: We present a case of 17-year-old male with congenital scoliosis with a 70-degree scoliotic curve who underwent spinal deformity correction surgery with posterior instrumented fusion. Post-operative course was uneventful and the patient was discharged after suture removal on post-operative day 15. The patient presented after 21-days of symptom onset on post-operative-day 51, with a 3 week history of post-prandial vomiting, abdominal pain and distension which resulted in rapid weight loss of 11 kg. A CT-angiogram showed obstruction at third part of duodenum. After reviewing clinical and radiological profile of the patient, a diagnosis of SMA syndrome was made. Conservative management was tried, but due to rapid deterioration of patient condition and symptoms of complete intestinal obstruction, the patient was treated surgically by gastro-jejunostomy and side-to-side jejuno-jejunostomy, which improved his condition. CONCLUSION: SMA syndrome can occur much later than previously reported cases and with potentially life-threatening symptoms following scoliosis correction. Having a high index of suspicion, early recognition of condition and institution of appropriate treatment are essential to prevent occurrence of severe complications including risk of intestinal perforation and mortality. This case highlights management of delayed onset of SMA syndrome, with presentation further delayed after symptom onset, as is common in developing parts of the world, due to limited availability and accessibility of resources, and low socio-economic status of large segments of the population.

Acute superior mesenteric artery syndrome complicated by severe gastric, pancreatic and renal ischaemia.

Superior mesenteric artery syndrome (SMAS) is a rare and potentially life-threatening cause of small bowel obstruction in which the superior mesenteric artery impinges on the third portion of the duodenum. SMAS is typically encountered in patients with low body fat and a history of rapid weight loss and is often diagnosed as a chronic or subacute condition. Here, we describe a case of a healthy adolescent boy without typical SMAS prodromal symptoms presenting with a severe, hyperacute proximal small bowel obstruction due to SMAS. Complications arising from massive gastric and duodenal distension, including gastric, pancreatic and renal ischaemia, necessitated emergent surgical intervention consisting of the duodenojejunostomy bypass with partial gastric resection. The patient recovered without significant lasting consequences.

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.

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.

Massive Gastric Dilatation and Multi-Organ Ischemia Due to Superior Mesenteric Artery Syndrome: A Rare Case Report.

BACKGROUND Superior mesenteric artery (SMA) syndrome, a rare condition in which the SMA and aorta occlude the third duodenal portion, can cause serious complications. We present the case of an 83-year-old Japanese man who presented with shock because of massive gastric dilatation due to SMA syndrome and developed multi-organ ischemia. CASE REPORT The day before admission, the patient had visited the emergency department with abdominal pain and was sent home following spontaneous symptom resolution, but experienced abdominal pain flare-up. His history included diabetes mellitus, hypertension, gastric ulcer, prostatic hypertrophy, esophageal hiatal hernia, and esophageal cancer. Plain computed tomography showed gastric dilatation and obstruction of the duodenal third portion by the SMA, leading to SMA syndrome diagnosis. Since a nasogastric tube could not be manually inserted into the stomach and the gastric dilatation could not be decompressed, the tube was inserted endoscopically. Endoscopy revealed mechanical obstruction of the gastric cardia and gastric mucosal ischemia. He was admitted to intensive care, and blood pressure was maintained with vasopressors and blood transfusion. The next day, contrast-enhanced computed tomography performed for persistently elevated lactate levels revealed extensive ischemia affecting multiple gastrointestinal organs. Surgery and other treatments were considered too risky due to the patient's advanced age and condition. Best supportive care was administered after discussion with the family, and he died on the second day of hospitalization. CONCLUSIONS SMA syndrome with extensive ischemia and infarction is rare. Given this, clinicians should remain vigilant for its potential complications.

A Case of Biliary Cast Syndrome with Cholangiocarcinoma-like Lesion in a Patient with No History of Liver Transplantation.

Background and Objectives: Biliary cast syndrome, which was first reported in 1975, is a rare disease that occurs after liver transplantation. The incidence is even lower in patients who have not undergone liver transplantation. This study reports a rare case of biliary cast syndrome with cholangiocarcinoma-like lesions in a patient who did not undergo liver transplantation. Case Report: Herein, we report a case of a 69-year-old man with right upper quadrant pain and elevated levels of alkaline phosphatase and gamma-glutamyl transferase, who had a history of total gastrectomy for gastric cancer and laparoscopic cholecystectomy for acute cholecystitis. Computed tomography (CT) revealed longitudinal bile stones in the extrahepatic and intrahepatic bile ducts and abrupt narrowing of the left main bile duct accompanied by a narrowing of the upstream bile duct in the left lobe of the liver. Based on the CT findings, the removal of the bile stones in the bile duct and additional examinations of the suspected cholangiocarcinoma were performed. The patient's symptoms improved, and examinations for suspected cholangiocarcinoma showed no abnormal findings, and he was discharged one month later. Conclusions: The purpose of this case report is to share a rare case of Biliary Cast Syndrome (BCS) occurring without liver transplantation. Additionally, the report aims to share image findings that mimic cancer in BCS, with the goal of reducing unnecessary repetitive biopsies, minimizing patient discomfort, and decreasing unnecessary costs by aiding in the diagnosis of BCS.

[A Case of Primary Jejunum Cancer Mimicking Superior Mesenteric Artery Syndrome].

In this report, we described a case of jejunum cancer that presented with abdominal pain and vomiting as chief complaints, which mimicked superior mesenteric artery syndrome. An elderly woman in her 70s was referred to our department for prolonged abdominal discomfort. Findings from CT and abdominal echo indicated that superior mesenteric artery syndrome may be responsible for jejunum cancer. Upper gastrointestinal endoscopy revealed a peripheral type 2 lesion in the upper jejunum. Upon biopsy, the patient was diagnosed with adenocarcinoma(papillary type). Surgical resection of the small intestine was performed. Although small intestinal cancer is a fairly rare disease, it should be considered a differential diagnosis. Comprehensive evaluations including medical history and imaging should be considered.

Surgical Intervention Could Relieve Obstruction-Related Symptoms of Refractory Superior Mesenteric Artery Syndrome: Long-Term Follow-up Results.

PURPOSE: Superior mesenteric artery syndrome (SMAS) is one of the rare causes of chronic duodenal obstruction. The aim of our study was to evaluate the outcomes of surgical management of refractory SMAS (Re-SMAS) in our institution during the last decade. MATERIALS AND METHODS: Consecutive patients diagnosed as Re-SMAS and underwent surgical intervention were retrospectively enrolled. A modified Likert-scale-based questionnaire was used to quantify the symptoms of SMAS. The primary outcome was the increase of BMI and symptomatic relief after surgery. RESULTS: From January 2010 to January 2020, 22 patients diagnosed with Re-SMAS and underwent surgery were included. Age distribution of included patients were < 19 (10/22, 45%), 19-45 (11/22, 50%), and > 45 (1/22, 5%). A significant BMI increase was recorded, [16.1 (14.6-23.7) kg/m2 vs 21.9 (15.6-29.5) kg/m2 before and after surgery, respectively, p < 0.001]. Up to 20 patients (20/22, 91%) reported symptomatic relief. The obstruction-related symptom score decreasing significantly (p < 0.001), with reported nausea, vomiting, and regurgitation incidences dropping from 77 to 41% (p = 0.031), 68 to 23% (p = 0.006), and 32 to 5% (p = 0.046), respectively. CONCLUSION: Surgical interventions could relieve obstruction-related symptoms of Re-SMAS and promote weight gain. For patients who suffered from Re-SMAS and referred to surgeons mainly for obstruction-related symptoms, surgical intervention is recommended.

Causa rara de dolor abdominal recurrente; coexistencia del síndrome de Wilkie y el síndrome del cascanueces / A rare cause of recurrent abdominal pain; the coexistence of Wilkie's syndrome and nutcracker syndrome

El síndrome del cascanueces es un síndrome que presenta síntomas clínicos como hematuria, proteinuria ortostática, congestión pélvica, varicocele del lado izquierdo, hipertensión y dolor en fosa renal. Estos síntomas se producen por la compresión de la vena renal izquierda entre la aorta y la arteria mesentérica superior. En el síndrome de Wilkie, la tercera porción del duodeno está comprimida entre la arteria mesentérica superior y la aorta abdominal, lo que provoca diversos síntomas gastrointestinales. La coexistencia de estos dos síndromes constituye una afección rara y se incluye como casos clínicos en la bibliografía. En este artículo, se presentan los resultados clínicos y radiológicos de un paciente de 17 años que presentaba dolor abdominal recurrente debido al síndrome de Wilkie, acompañado del síndrome del cascanueces que le provocaba proteinuria, por lo que el paciente fue derivado a los consultorios externos de reumatología pediátrica con un diagnóstico preliminar de fiebre mediterránea familiar.

Nutcracker syndrome is a syndrome that has clinical symptoms such as hematuria, orthostatic proteinuria, pelvic congestion, left-sided varicocele, hypertension, and flank pain. These symptoms occur because of the compression of the left renal vein between the aorta and the superior mesenteric artery. In Wilkie's syndrome, the third part of the duodenum is compressed between the superior mesenteric artery and the abdominal aorta, causing various gastrointestinal symptoms. The coexistence of these two syndromes is a rare condition and is included as case reports in the literature. This article presents the clinical and radiological results of a 17-year-old male patient who had recurrent abdominal pain due to Wilkie's syndrome, which was accompanied by nutcracker syndrome that caused proteinuria, and for this reason, the patient was referred to the Pediatric Rheumatology outpatient clinic with a preliminary diagnosis of familial Mediterranean fever.

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.

Síndrome de Wilkie: relato de caso / Wilkie's syndrome: case report

Objetivo: Apresentar um raro caso de Síndrome de Wilkie, devido a sua dificuldade diagnostica. Relato do caso: Paciente masculino, 18 anos, em tratamento no INCA para Osteossarcoma de fêmur esquerdo, retorna ao hospital no pós-operatório, com queixa de dores epigástricas de forte intensidade e vômitos incoercíveis. Tomografia Computadorizada (TC) de abdome total evidenciou distensão gástrica até a terceira porção do duodeno, onde há redução abrupta de calibre por compressão da artéria mesentérica superior. Diagnóstico estabelecido, através dos exames de imagem, como Síndrome da Artéria Mesentérica Superior (Síndrome de Wilkie), por complicação pós-operatória da doença de base. O tratamento consistiu em uma anastomose duodenojejunal, sem intercorrências. Discussão: uma das teorias mais aceitas como fator desencadeante desta síndrome é a diminuição ou perda de gordura mesentérica, que serve como conexão entre a aorta e a artéria mesentérica superior. As causas congênitas incluem um ligamento de Treitz curto. A TC deve seguir alguns critérios para o diagnóstico, como: obstrução abrupta da terceira porção do duodeno, ângulo aorto-mesentérico e distância da aorta para artéria mesentérica reduzidos, fixação alta do duodeno pelo ligamento de Treitz (causa congênita). Conclusão: Apesar da grande quantidade de relatos de caso publicados, a Síndrome de Wilkie continua sendo de difícil diagnóstico. Ela deve ser considerada um diagnóstico diferencial em pacientes com sintomas de trato gastrointestinal alto. Os estudos de imagem constituem uma ferramenta fundamental para identificar precocemente os casos

Objective: Report a rare case of Wilkie's Syndrome, due to its diagnostic difficulty. Case Report: Male patient, 18 years old, under treatment at INCA for Osteosarcoma of the left femur, returns to the hospital postoperatively, complaining of severe epigastric pain and incoercible vomiting. Computed tomography (CT) of the abdomen revealed gastric distension up to the third portion of the duodenum, where there was an abrupt reduction in caliber due to compression of the superior mesenteric artery. Diagnosis was established, through imaging exams, as Superior Mesenteric Artery Syndrome (Wilkie's Syndrome), by postoperative complication of the underlying disease. Treatment consisted of an uneventful duodenojejunal anastomosis. Discussion: One of the most accepted theories as a triggering factor of this syndrome is the decrease or loss of mesenteric fat, which serves as a connection between the aorta and the superior mesenteric artery. Congenital causes include a short Treitz ligament. CT should follow some criteria for diagnosis, such as: abrupt obstruction of the third portion of the duodenum, reduced aorto-mesenteric angle and distance from aorta to mesenteric artery, high fixation of the duodenum by the ligament of Treitz (congenital cause). Conclusion: Despite the large number of published case reports, Wilkie's Syndrome remains difficult to diagnose. It should be considered a differential diagnosis in patients with upper GI tract symptoms. Imaging studies are a fundamental tool to identify cases early

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.

Unusual presentation of superior mesenteric artery syndrome in a child.

Superior mesenteric artery syndrome (SMAS), also known as Wilkie's syndrome, is a rare cause of upper gastrointestinal tract obstruction. We report a case of a 10-year-old girl with persistent abdominal pain for over 3 months, who on extensive investigations was diagnosed with SMAS. She underwent a surgical procedure to bypass the obstructed portion of the intestine for relief of her symptoms.

[Superior mesenteric artery syndrome 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.