A Síndrome da Artéria Mesentérica Superior (SAMS), Síndrome de Wilkie ou até mesmo Pinçamento Aorto-Mesentérico é um distúrbio gastro-vascular em que a terceira e a quarta porção do Duodeno é comprimida entre a Aorta abdominal e a Artéria mesentérica superior subjacente.
Introdução
O que você precisa saber de cara
Visão geral
A Síndrome de artéria mesentérica superior (SAMS) é uma condição digestiva que ocorre quando o duodeno (a primeira parte do intestino delgado) é comprimido entre duas artérias: a aorta e a artéria mesentérica superior. Essa compressão causa um bloqueio parcial ou completo do duodeno. A SAMS geralmente é consequência da perda do coxim adiposo mesentérico (tecido gorduroso que envolve a artéria mesentérica superior). A causa mais comum é a perda significativa de peso decorrente de distúrbios médicos, distúrbios psicológicos ou cirurgias. Em pacientes mais jovens, ocorre com mais frequência após cirurgia corretiva da coluna para escoliose.[1]
Sinais e sintomas
Os sintomas variam de acordo com a gravidade, mas podem ser severamente debilitantes. Os principais sintomas incluem dor abdominal, sensação de plenitude, náuseas, vômitos e/ou perda de peso.[1]
Causas genéticas
Diagnóstico
Tratamento e manejo
Dependendo da causa e da gravidade, as opções de tratamento podem incluir abordar a causa subjacente, mudanças na dieta (refeições pequenas ou dieta líquida) e/ou cirurgia. Os sintomas podem não desaparecer completamente após o tratamento.[1]
Prognóstico e qualidade de vida
O prognóstico varia conforme a causa e a resposta ao tratamento. Embora o manejo adequado possa aliviar os sintomas, em alguns casos os sintomas podem não se resolver completamente, impactando a qualidade de vida.[1]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
Síndrome rara de compressão da artéria mesentérica superior contra a aorta, causando obstrução do duodeno. Manifesta-se com dor abdominal pós-prandial, náuseas, vômitos e perda de peso.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Visão geral
A Síndrome de artéria mesentérica superior (SAMS) é uma condição digestiva que ocorre quando o duodeno (a primeira parte do intestino delgado) é comprimido entre duas artérias: a aorta e a artéria mesentérica superior. Essa compressão causa um bloqueio parcial ou completo do duodeno. A SAMS geralmente é consequência da perda do coxim adiposo mesentérico (tecido gorduroso que envolve a artéria mesentérica superior). A causa mais comum é a perda significativa de peso decorrente de distúrbios médicos, distúrbios psicológicos ou cirurgias. Em pacientes mais jovens, ocorre com mais frequência após cirurgia corretiva da coluna para escoliose.[1]
Sinais e sintomas
Os sintomas variam de acordo com a gravidade, mas podem ser severamente debilitantes. Os principais sintomas incluem dor abdominal, sensação de plenitude, náuseas, vômitos e/ou perda de peso.[1]
Causas genéticas
Diagnóstico
Tratamento e manejo
Dependendo da causa e da gravidade, as opções de tratamento podem incluir abordar a causa subjacente, mudanças na dieta (refeições pequenas ou dieta líquida) e/ou cirurgia. Os sintomas podem não desaparecer completamente após o tratamento.[1]
Prognóstico e qualidade de vida
O prognóstico varia conforme a causa e a resposta ao tratamento. Embora o manejo adequado possa aliviar os sintomas, em alguns casos os sintomas podem não se resolver completamente, impactando a qualidade de vida.[1]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome de artéria mesentérica superior
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5 ensaios clínicos encontrados, 2 ativos.
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Loss of Myofilaments in Gastrointestinal Smooth Muscle: A Novel Pathological Finding in MELAS-Associated Chronic Intestinal Pseudo-Obstruction.
Chronic intestinal pseudo-obstruction (CIPO) occurs in up to 40% of patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). While enteric nervous system abnormalities have been documented, 15%-38% of CIPO cases show normal histology, suggesting alternative pathogenic mechanisms. We aimed to investigate smooth muscle pathology in MELAS-associated CIPO. Comprehensive pathological examination including light and electron microscopy was performed on autopsy material from a 52-year-old male with MELAS (m.3243A > G mutation) and recurrent intestinal obstruction symptoms. Gastrointestinal tissue from the entire digestive tract was analyzed and compared with age-matched control tissue. Histological examination revealed widespread vacuolization and pallor of gastrointestinal smooth muscle throughout the digestive tract (esophagus, stomach, duodenum, and colon). Electron microscopy demonstrated abundant abnormal mitochondria in smooth muscle cells and, notably, marked loss of myofilaments in the colonic muscle. The ultrastructural preservation was limited by postmortem changes (autolysis, occurring approximately 4 h after death) and the re-embedding technique from formalin-fixed paraffin-embedded tissue; however, the striking difference was evident compared to age-matched controls. Similar abnormalities were observed in the Auerbach plexus. This represents the first report of myofilament loss in MELAS-associated CIPO, suggesting that mitochondrial dysfunction may directly impair smooth muscle contractile apparatus beyond previously described neuronal abnormalities. These findings provide novel insights into CIPO pathogenesis and may inform therapeutic strategies emphasizing early enteral nutrition interventions that bypass affected gastrointestinal segments.
Mixed Metabolic and Respiratory Alkalosis: An Uncommon Presentation of Superior Mesenteric Artery Syndrome in an Adolescent Male Patient.
Superior mesenteric artery (SMA) syndrome is an uncommon cause of proximal intestinal obstruction resulting from compression of the third portion of the duodenum between the abdominal aorta and the SMA. Patients typically present with postprandial epigastric pain, bilious vomiting, and weight loss, and most exhibit metabolic alkalosis due to persistent loss of gastric acid. We report the case of a 16-year-old male child with progressive postprandial vomiting and significant nutritional decline who was diagnosed with SMA syndrome on the basis of reduced aortomesenteric angle and marked duodenal dilatation on imaging. Uniquely, the patient demonstrated a mixed acid-base disorder, combining metabolic alkalosis from chronic vomiting with respiratory alkalosis likely secondary to hypoxia-induced hyperventilation. The patient underwent Strong's procedure with good postoperative recovery and resolution of symptoms. This case emphasizes the importance of recognizing atypical metabolic profiles in SMA syndrome, maintaining clinical suspicion in adolescents with chronic vomiting and weight loss, and intervening early to prevent complications.
Fluoroscopically guided jejunal tube placement via percutaneous gastrostomy in children: technical success, safety, and procedural parameters.
Fluoroscopically guided jejunal tube placement via percutaneous endoscopic gastrostomy (PEG-J) provides minimally invasive post-pyloric access in children. Limited data exist regarding routine application and procedural risks. To evaluate the safety and technical success of PEG-J in pediatric patients, performed without general anesthesia or sedation. All pediatric cases of fluoroscopically guided PEG-J procedures performed between 2011 and 2025 were included. Fluoroscopic images were reviewed to determine the final position of the tube tip. Technical success, complications, anatomical variants, and tube patency were assessed. Fluoroscopy time and dose area product (DAP) were documented. A total of 126 PEG-J procedures in 60 children (36 males) were analyzed. The technical success rate was 85% (107/126) with final tube tip placement in the jejunum in 88 cases (82%) and in the duodenum in 19 cases (18%). Nineteen procedures (15%) were unsuccessful, including six with documented anatomical causes (steep vertical duodenal entry, n=2; malrotation, hiatus hernia, hooked stomach in superior mesenteric artery syndrome, steep take-off of the jejunum with kinking of the tube at the ligament of Treitz, n=1 each) and 13 without documented reasons. The median fluoroscopy time was 5 min 24 s (range, 2 s-37 min), at a frame rate of 0.5 frames per second. The median DAP was 6.1 cGy·cm2 (range, 0.08-343 cGy·cm2). Fluoroscopically guided PEG-J placement is a safe and effective procedure in pediatric patients, with high technical success and low radiation exposure.
Aorto-mesenteric space reduction in women with anorexia nervosa: retrospective audit and analysis.
Superior mesenteric artery syndrome (SMAS) is a rare condition favored by weight loss, with nonspecific digestive complaints that may hinder weight recovery in anorexia nervosa. This study aimed to examine the radiological features of aorto-mesenteric (A-M) space reduction in anorexia nervosa and their association with digestive complaints. Female patients with anorexia nervosa and a history of computerized tomography angiography for digestive complaints were included. Clinical data were retrospectively collected and computerized tomography scans were reviewed by an independent, experienced radiologist to identify signs of SMAS and of Nutcracker syndrome. Adipose tissue surfaces were also calculated from the scans. Some patients were reassessed after nutritional recovery. On the 51 female patients included (mean age 27.7 ± 12.3 years) from a cohort of 202 female anorexia nervosa patients, 48 met radiological criteria for SMAS (A-M angle < 25° or distance ≤ 8 mm). A duodenal dilation was present in 35 patients (68.6%) and a left renal vein dilation in 39.2%. The type of digestive complaints did not differ significantly between patients with and without duodenal dilation, although gastroesophageal reflux approached statistical significance (p = 0.06). Body mass index and visceral adipose tissue did not correlate significantly with A-M measurements. Following nutritional recovery in ten patients, there was a significant increase in A-M measurements and a decrease in both duodenal dilation and digestive symptoms. Radiological features of A-M space reduction are common in anorexia nervosa. Left renal vein compression and its upstream dilation, as well as left dilated ovarian vein and pelvic varicose veins, are frequently associated with radiological signs of SMAS. Nutritional support alleviates digestive complaints related to SMAS. This study examined how the space between the body’s main artery (the aorta) and the artery that supplies blood to the intestines (the superior mesenteric artery) can become narrower in people with anorexia nervosa in relation with body weight decrease. This narrowing happens where the first part of the small intestine (the duodenum) passes between the two arteries. It may contribute to digestive problems that are common in anorexia nervosa. The results showed that 94.1% of patients had a significantly narrowed space between these arteries (a condition sometimes called superior mesenteric artery syndrome), and 68.6% had an enlarged duodenum. Among digestive symptoms, acid reflux (heartburn) was the most strongly linked to this enlargement. After nutritional recovery, the space between the arteries widened again, and both the duodenal enlargement and digestive symptoms improved. These findings are important because they show that this narrowing is much more common in anorexia nervosa than previously thought. It may help explain why so many patients experience digestive issues. This insight could guide clinicians in selecting the most appropriate nutritional support strategies, which are a critical component of anorexia nervosa treatment.
Superior mesenteric artery syndrome in a 26-year-old male presenting as acute pancreatitis and portal venous gas: a case report and review of the literature.
Superior mesenteric artery syndrome is a rare form of duodenal obstruction that usually requires nonoperative management. While individual complications such as acute pancreatitis, portal venous gas, and gastric emphysema have been reported, the simultaneous occurrence of this triad in a single patient with superior mesenteric artery syndrome has never been documented. This case is novel as it highlights the successful nonoperative management of superior mesenteric artery syndrome despite the presence of these severe, potentially life-threatening complications. A 26-year-old Asian male with a constitutional low body mass index of 14.53 kg/m2 presented with abrupt epigastric pain and bilious vomiting. Investigations revealed a diagnosis of superior mesenteric artery syndrome complicated by acute pancreatitis, portal venous gas, and gastric emphysema. The patient was treated nonoperatively with intensive care support, including nasogastric decompression and total parenteral nutrition. The interventions led to a significant reduction in gastric output, resolution of acute kidney injury and pain, and subsequent removal of the nasogastric tube. The patient was discharged well and showed no signs of obstruction at the 9-month follow-up, having gained weight. This case demonstrates that the presence of portal venous gas and gastric emphysema in superior mesenteric artery syndrome, which typically raises suspicion for gastrointestinal necrosis, can be successfully managed conservatively if clinical signs of perforation or sepsis are absent. Clinicians should maintain a high index of suspicion for the complex presentations of superior mesenteric artery syndrome and prioritize aggressive nonoperative nutritional support, as it can resolve obstruction and preclude the need for surgery even in the setting of severe multi-systemic complications.
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Journal of gastrointestinal and liver diseases : JGLDLaparoscopic lateral duodenojejunostomy for pediatric superior mesenteric artery compression syndrome: a cohort retrospective study.
BMC surgerySuperior Mesenteric Artery Syndrome and Nutcracker Syndrome as the Presentation of Crohn's Disease in a Young Patient: A Case Report and Review of Literature.
JPGN reportsSuperior mesenteric artery syndrome and anorexia nervosa: a case report.
Journal of medical case reportsUnderstanding the diagnosis of superior mesenteric artery syndrome: analysis of the location of duodenal impression on upper gastrointestinal studies.
Pediatric radiologyIncidence and Potential Risk Factors of Superior Mesenteric Artery Syndrome After Spinal Corrective Surgery in Patients with Adult Spinal Deformity.
World neurosurgeryAcute gastric dilatation with superior mesenteric artery syndrome after binge eating.
Pediatrics international : official journal of the Japan Pediatric SocietyGastric Pneumatosis and Its Gastrofibroscopic Findings in Life-Threatening Superior Mesenteric Artery Syndrome Complicated by Anorexia Nervosa in a Child.
Pediatric gastroenterology, hepatology & nutritionAorto-mesenteric compass syndrome (Wilkie's syndrome) in the differential diagnosis of chronic abdominal pain.
BMJ case reportsSuperior Mesenteric Artery Syndrome Masquerading As Irritable Bowel Syndrome: A Case Report.
CureusMassive Gastric Dilatation and Multi-Organ Ischemia Due to Superior Mesenteric Artery Syndrome: A Rare Case Report.
The American journal of case reportsCase report: doing the mesenteric twist.
Journal of surgical case reportsSuperior Mesenteric Artery Syndrome in Systemic Lupus Erythematosus.
CureusSuperior mesenteric artery syndrome in an 8-year-old boy: a case report.
Journal of medical case reportsSuperior mesenteric artery syndrome in a patient with fibrodysplasia ossificans progressiva.
Bone reportsA Case of Biliary Cast Syndrome with Cholangiocarcinoma-like Lesion in a Patient with No History of Liver Transplantation.
Medicina (Kaunas, Lithuania)Superior Mesenteric Artery Syndrome.
The New England journal of medicineUretero-Jejunal Fistula: A Rare Cause of Acute Pyelonephritis.
CureusUnveiling the Uncommon: Superior Mesenteric Artery Syndrome Presenting As Gastritis.
CureusRobotic modified Strong procedure for superior mesenteric artery syndrome.
Clinical case reportsSuperior mesenteric artery syndrome treatment strategies: A case report.
SAGE open medical case reportsFood Insecurity Leading to Superior Mesenteric Artery Syndrome Managed Successfully with Endoscopic Gastrojejunostomy Stent.
The American journal of case reportsCombined superior mesenteric artery syndrome and nutcraker syndrome presenting as acute pancreatitis: a case report.
Jornal vascular brasileiroSuperior Mesenteric Artery Syndrome as a Rare Cause of Postoperative Intractable Vomiting: A Case Report.
Clinical and experimental gastroenterologySuperior Mesenteric Artery (SMA) Syndrome With Enterocutaneous Fistula in a Young Woman: A Rare Association.
CureusSuperior mesenteric artery syndrome: Diagnosis and management.
World journal of clinical casesSuperior mesenteric artery syndrome with acute gastric dilatation caused by binge eating in an adolescent.
The Korean journal of internal medicine[A case of primary peritoneal cancer diagnosed with a duodenal stricture].
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterologyAn interesting presentation of a rare association of the Wilkie and Nutcracker syndromes.
Radiology case reportsAn atypical cause of vomiting: Coexisting Wilkie's syndrome and a left renal malformation mimicking a nutcracker phenomenon-A case report.
SAGE open medical case reports[A Case of Primary Jejunum Cancer Mimicking Superior Mesenteric Artery Syndrome].
Gan to kagaku ryoho. Cancer & chemotherapyA case of superior mesenteric artery syndrome characterized by deep forward bending posture in a cross-legged position on the floor.
Clinical case reportsSuperior Mesenteric Artery Syndrome in an Adolescent Female with Anorexia Nervosa.
Clinical medicine & researchSuperior mesenteric artery syndrome in a 30-year-old male patient: A case report and literature review.
International journal of surgery case reportsSuperior mesenteric artery syndrome mimicking gastric outlet obstruction: a case report and a literature review.
Annals of medicine and surgery (2012)Imaging spectrum of non-neoplastic and neoplastic conditions of the duodenum: a pictorial review.
Abdominal radiology (New York)Superior mesenteric artery syndrome following lung cancer surgery: A case report.
International journal of surgery case reportsMinimalistic approach to enhanced recovery after pediatric scoliosis surgery.
Spine deformityMega-Stomach as a Result of Superior Mesenteric Artery Syndrome.
ACG case reports journalCauses of epigastric pain and vomiting after laparoscopic-assisted radical right hemicolectomy - superior mesenteric artery syndrome.
World journal of gastrointestinal surgeryAcute superior mesenteric artery syndrome with complete foregut obstruction following Nissen fundoplication.
International journal of surgery case reportsNursing of a lactating patient with superior mesenteric artery syndrome: a case report.
The Journal of international medical researchSuperior Mesenteric Artery Syndrome: A Vicious Cycle?
ACG case reports journal[Superior mesenteric artery syndrome in gastric cancer: a case report].
The Pan African medical journalRobotic assisted kidney auto-transplantation as a safe alternative for treatment of nutcracker syndrome and loin pain haematuria syndrome: A case series report.
The international journal of medical robotics + computer assisted surgery : MRCASWell-concealed advanced duodenal carcinoma with Muir-Torre syndrome: a case report and review of literature.
Surgical case reportsSurgical Intervention Could Relieve Obstruction-Related Symptoms of Refractory Superior Mesenteric Artery Syndrome: Long-Term Follow-up Results.
Obesity surgerySuperior mesenteric artery syndrome following esophageal cancer surgery: A report of two cases and a literature review.
Medicine internationalSurgical therapy of celiac axis and superior mesenteric artery syndrome.
Langenbeck's archives of surgeryNutcracker phenomenon secondary to superior mesenteric artery syndrome.
Journal of surgical case reportsSuperior mesenteric artery syndrome: An unusual cause of abdominal compartment syndrome and bilateral lower limb ischemia.
BJR case reportsAnorexia nervosa: practical implications for the anaesthetist.
BJA educationSuperior Mesenteric Artery Syndrome in Down Syndrome: A Case Report.
CureusPaediatric Spinal Deformity Surgery: Complications and Their Management.
Healthcare (Basel, Switzerland)Total gastric necrosis following massive gastric dilatation due to superior mesenteric artery syndrome.
Asian journal of surgeryA Rare Presentation of Superior Mesenteric Artery Syndrome as Acute Abdomen.
CureusSuperior mesenteric artery syndrome identified following bubble tea ingestion and duodenal impaction: a case report.
CJEMSuperior mesenteric artery syndrome and disseminated tuberculosis: "Double troubleˮ.
The Indian journal of tuberculosisA rare cause of recurrent abdominal pain; the coexistence of Wilkie's syndrome and nutcracker syndrome.
Archivos argentinos de pediatriaGastric dilatation in patients with restrictive eating disorders.
The International journal of eating disordersDiurnal Fasting During Ramadan Leading to Superior Mesenteric Artery Syndrome.
ACG case reports journalEndoscopic Ultrasound-Guided Gastrojejunostomy for Superior Mesenteric Artery Syndrome Secondary to Rapid Weight Loss.
ACG case reports journalAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Loss of Myofilaments in Gastrointestinal Smooth Muscle: A Novel Pathological Finding in MELAS-Associated Chronic Intestinal Pseudo-Obstruction.
- Mixed Metabolic and Respiratory Alkalosis: An Uncommon Presentation of Superior Mesenteric Artery Syndrome in an Adolescent Male Patient.
- Fluoroscopically guided jejunal tube placement via percutaneous gastrostomy in children: technical success, safety, and procedural parameters.
- Aorto-mesenteric space reduction in women with anorexia nervosa: retrospective audit and analysis.
- Superior mesenteric artery syndrome in a 26-year-old male presenting as acute pancreatitis and portal venous gas: a case report and review of the literature.
- Intestinal malrotation combined with superior mesenteric artery syndrome: a case report.
- The Investigation and Management of the Abdominopelvic Vascular Compression Syndromes in Patients with Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorder.
- Identification and Management of Intraoperative Pneumothorax During Laparoscopic Surgery: A Rare Complication.
- Delayed Diagnosis of Thyroid Storm Presenting With Predominant Gastrointestinal Symptoms.
- Robotic Surgery in the Treatment of Combined Wilkie's and Dunbar's Syndromes: A Case Report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:622099(Orphanet)
- MONDO:0002687(MONDO)
- GARD:7712(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
