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Superior mesenteric artery syndrome (Wilkie Syndrome) with unusual clinical onset: Description of a rare case.

ABSTRACT: Wilkie's Syndrome is a very rare disease caused by reduction of aorto-mesenteric space with consequent duodenum compression. It can combine with left renal vein stenosis which, when symptomatic, is known as "Nutcracker Syndrome". We describe a clinical onset case with epigastric pain without vomiting in a normal weight patient. 28-year-old woman who came to our observation for intense epigastric pain after a weight loss of 14 kg in 4 months. Multidetector Computed Tomography and Ultrasound revealed gastric and duodenal overdistension with hydro-air levels, severe duodenum stenosis, and left renal vein compression. Wilkie's Syndrome is common in anorexic individuals suffering from recurrent postprandial vomiting, onset with severe epigastric pain, without vomiting, is quite unusual. High-calorie diet must be first therapeutic approach, in case of failure treatment of first choice should be endovascular stenting and, only in selected cases, surgical treatment should be used because it is very invasive and burdened with numerous complications. Failure to diagnose this disease can expose patients to serious health risks.

SUBMITTER: Farina R

PROVIDER: S-EPMC8358285 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Robotic modified Strong procedure for superior mesenteric artery syndrome.

Project description:Key clinical messageThe robotic modified Strong procedure is a safe and effective approach for surgical management of superior mesenteric artery syndrome in properly selected patients.AbstractSuperior mesenteric artery syndrome is a rare syndrome of small bowel obstruction resulting from vascular compression of the duodenum. Here we present our modification of a robotic Strong procedure for the surgical management of SMA syndrome. This procedure is a safe and effective approach for management in properly selected patients.

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Sanjad-sakati syndrome and its association with superior mesenteric artery syndrome.

Project description:Sanjad-Sakati syndrome (SSS) is an autosomal recessive disorder found exclusively in people of Arabian origin. It was first reported in the Kingdom of Saudi Arabia in 1988 and confirmed by a definitive report in 1991. The syndrome comprises of congenital hypoparathyroidism, seizures, severe growth and developmental retardation, low IQ, and atypical facial features. Supportive treatment in the form of vitamin D and growth hormone supplementation is often offered to patients suffering from SSS. This case study focuses on the steps taken to help a patient who was found to have very unusual symptoms and was later found to have superior mesenteric artery syndrome.

| S-EPMC4241564 | biostudies-literature

Unusual computed tomography findings of gas in the superior mesenteric artery system with no signs of porto-mesenteric venous gas in a case of acute mesenteric ischemia.

Project description:Acute Mesenteric Ischemia (AMI) is a rare life-threatening entity caused by sudden interruption of the blood supply to a segment of the bowel due to impairment of mesenteric arterial blood flow or venous drainage. Clinical presentation varies according to the time course of vascular occlusion. Contrast-enhanced Computed Tomography (CT) of the abdomen represents the main diagnostic test for AMI diagnosis, enabling fast and excellent evaluation of the intestine, mesenteric vasculature, and other ancillary characteristics of AMI. Typical CT findings of AMI include paralytic ileus, decreased or absent bowel wall contrast-enhancement, pneumatosis intestinalis, and porto-mesenteric venous gas. We hereby report a case of an 89-year-old man presenting with AMI due to Superior Mesenteric Artery (SMA) thrombotic occlusion following endovascular stenting superficial femoral arteries. Typical findings were observed on abdominal CT imaging, yet associated with the presence of gas exclusively in the SMA district, without any involvement of the porto-mesenteric venous system. Different imaging features and pitfalls can help radiologists to accurately diagnose AMI, especially when irreversible bowel damage is about to occur. Therefore, radiologists and emergency physicians should be aware of the unusual association between gas in the SMA arterial district and AMI, even in the absence of porto-mesenteric venous system involvement, in order to urge prompt surgical consultation when observed.

| S-EPMC9130093 | biostudies-literature

Comparison of Superior Mesenteric Artery Remodeling and Clinical Outcomes between Conservative or Endovascular Treatment in Spontaneous Isolated Superior Mesenteric Artery Dissection.

Project description:The aim of this study was to analyze the superior mesenteric artery (SMA) remodeling after initial conservative or endovascular treatment with a standardized definition and midterm outcomes in patients with spontaneous isolated dissection of the superior mesenteric artery (SIDSMA). This retrospective study enrolled patients with SIDSMA from January 2007 to August 2019. All patients were treated initially with conservative treatment. If they failed the medical treatment, they were converted to interventional treatment. The morphological endpoint was determined by the standardized SMA remodeling, and the clinical endpoints were determined by the in-hospital mortality, hospital stay, and the bowel-related mid-term mortality. A total of 34 consecutive patients with SIDSMA were identified. Twenty-three (67.6%) and eleven (33.4%) patients underwent conservative and interventional treatments, respectively. Clinical features and morphologic changes on CTA were available in 25 (73.5%) patients during the median follow-up of 23.3 months. Standardized SMA remodeling was significantly (p < 0.05) better in patients undergoing endovascular stenting, especially in patients with Yun's IIb classification. There was no mesenteric ischemia or SMA aneurysm during follow-up period. Patients with SIDSMA can be treated safely with initial conservative treatment. However, significant portions of patients will require endovascular intervention due to the persistent symptoms. Clinically endovascular stenting could be performed successfully, and SMA remodeling was satisfactory during the mid-term follow-up.

| S-EPMC8777763 | biostudies-literature

A rare manifestation of clinical T1 renal tumor with parasitic arterial supply from the superior mesenteric artery.

Project description:A 50-year-old man with von Hippel-Lindau syndrome who had undergone repeated tumor enucleation and transcatheter arterial embolization for multiple renal cell carcinomas (RCC) was referred to our hospital for percutaneous cryoablation (PCA) of an RCC of 42 mm in the midpole region of the right kidney. Transcatheter arterial embolization was planned prior to devascularize the RCC and selective angiography revealed parasitic arterial supply to the tumor by the ileocecal artery. Parasitic arterial supply to RCCs, particularly in patients with history of nephron-sparing treatment, can originate even from an intraperitoneal source and may lead to unexpected embolization.

| S-EPMC6083380 | biostudies-literature

Celiac artery thrombosis and superior mesenteric artery stenoses with essential thrombocythemia: a case report.

Project description:Thrombosis of the celiac artery trunk is a rare cause of acute abdominal pain. Thrombosis of the celiac artery carries a high mortality and morbidity when the diagnoses and treatment are delayed. It is frequently associated with other cardiovascular events. The most common etiology is atherosclerosis. 20-30% of cases may have symptoms of chronic mesenteric ischemia. Main goal of the treatment is to reestablish the diminished or stopped mesenteric blood flow and to avoid end-organ ischemia. Essential thrombocythemia is a chronic myeloproliferative disorder characterized by marked increase in thrombocyte number and clinical presentation may be with thrombotic episodes, hemorrhage, or both. To our knowledge this is the first report of celiac artery thrombosis and superior mesenteric artery stenoses in a patient with essential thrombocythemia. The patient was managed successfully with surgical treatment.

| S-EPMC3530234 | biostudies-literature

A man with the rare simultaneous combination of three abdominal vascular compression syndromes: median arcuate ligament syndrome, superior mesenteric artery syndrome, and nutcracker syndrome.

Project description:Median arcuate ligament syndrome and superior mesenteric artery syndrome are well-known abdominal compression syndromes, the coexistence of which is rarely described in literature. In addition, due to the common pathogenesis, anterior nutcracker syndrome may occur simultaneously to superior mesenteric artery syndrome. To our knowledge, this is the first case reporting combination of these 3 syndromes detected with ultrasound, Computed Tomography and upper gastrointestinal fluoroscopic exam. A 69-year-old man came to our attention for rapid weight loss, postprandial epigastric pain and recurrent vomiting for at least 6 months. Doppler ultrasound showed both celiac artery and left renal vein stenosis with simultaneous left varicocele. Computed tomography showed a reduction of aortomesenteric space causing both left renal vein and duodenal stenosis, this latter confirmed by upper gastrointestinal fluoroscopic exam. The diagnosis of these three vascular compression syndromes (MALS, SMAS, and anterior NCS) has been formulated, based on clinical and imaging findings. We assumed that the postprandial crises caused by median arcuate ligament syndrome may induce a reduction of meals consumption and progressive weight loss which can be a cause of anterior nutcracker syndrome and superior mesenteric artery syndrome onset. Doppler ultrasound, in expert hands, allows to accurately diagnosing these syndromes which are often underestimated. Failure to recognize it and inadequate treatment could have serious consequences for patients' health.

| S-EPMC8026914 | biostudies-literature

Retroperitoneal approach for ilio-superior mesenteric artery bypass: Technique and case series

Project description: Not available

| S-EPMC11294714 | biostudies-literature

EUS-guided gastroenterostomy for duodenal obstruction secondary to superior mesenteric artery syndrome.

Project description: Not available

| S-EPMC7806496 | biostudies-literature

Variation of the ileocolic artery and superior mesenteric artery in a patient with right-sided colon cancer with Lynch syndrome: a case report.

Project description:BackgroundComplete mesangectomy and central vascular detachment are the core elements of laparoscopic right hemicolectomy. Failure to identify vascular variations in patients undergoing laparoscopic right hemicolectomy can result in unwanted bleeding, a prolonged surgical time, transfer to open surgery, and an elevated risk of postoperative complications. In this case report, we describe a new vascular variation that has not yet been reported in the literature. Parallelly vascular variation and the management of vessels in key areas are essential for successful surgery.Case descriptionThe patient was a 32-year-old female who was referred to the department of gastrointestinal surgery of our hospital due to intermittent abdominal pain accompanied by changes in stool habits for 3 months. She had not experienced other symptoms. Physical examination revealed mild tenderness in the right lower abdomen. Subsequently, she underwent laparoscopic radical right hemicolectomy for ascending colon cancer under general anesthesia in our hospital. Preoperative abdominal contrast-enhanced computed tomography (CT) and intraoperative photos confirmed that there were two ileocolic arteries derived from the superior mesenteric artery (SMA). On the other side, the SMA and superior mesenteric vein (SMV) were found to be accompanied like "X"-shaped variant. The final surgical pathological diagnosis was pT3N1aM0 adenocarcinoma of the ascending colon. Given the patient's family history of colon and uterine cancer combined with the results of immunohistochemical staining and next-generation sequencing, we concluded that she had Lynch syndrome (LS).ConclusionsThis report describes the first case of simultaneous variation of the ileocolic artery (ICA) and SMA in a female patient with colon cancer. This type of vascular variation should be fully recognized by surgeons in order to avoid unnecessary intraoperative bleeding.

| S-EPMC9511195 | biostudies-literature

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