Supradiaphragmatic origin of the celiac trunk leading to median arcuate ligament syndrome with superior mesenteric artery involvement.

Median arcuate ligament (MAL) syndrome (MALS) is a rare condition caused by compression of the celiac artery by the MAL. Symptoms include abdominal pain, nausea, and weight loss. Rarely, the MAL can compress both the celiac artery and the superior mesenteric artery (SMA). We describe the case of a young man with MALS involving the celiac artery and SMA. Laparoscopic release of the MAL was performed, and the patient had resolution of his symptoms at 6 months of follow-up. A review of the literature identified only six cases of MALS involving the SMA and celiac artery, making this a rare occurrence.

Median Arcuate Ligament Syndrome with Orthostatic Intolerance: Intermediate-Term Outcomes following Surgical Intervention.

OBJECTIVES: To report the intermediate-term outcome following surgical intervention for median arcuate ligament syndrome (MALS) in adolescents and young adults with orthostatic intolerance (OI) to assess clinical improvement in the gastrointestinal and 5 other functional domains and if relief of arterial obstruction is associated with resolution of clinical symptoms. STUDY DESIGN: Thirty-one patients were given 2 dysautonomia-designed questionnaires to assess changes in symptoms following operative intervention in 6 functional domains and underwent postoperative repeat abdominal ultrasound examinations. RESULTS: Average follow-up after surgery was 22.4 ± 14.8 months. Self-assessed quality of health on a Likert scale (1-10 with 10 being normal) improved from 4.5 ± 2.1 preoperatively to 5.3 ± 2.4 postoperatively (P = not significant). Gastrointestinal symptoms of abdominal pain, nausea, and vomiting improved in 63% (P = .007), 53% (P = .040), and 62% (P = .014) of patients, respectively. Cardiovascular symptoms of dizziness, syncope, chest pain, and palpitations improved in 45% (P = not significant), 50% (P = not significant), 54% (P = .043), and 54% (P = .037) of patients, respectively. Transabdominal ultrasound peak supine expiratory velocity decreased from 348 ± 105 cm/s preoperatively to 251 ± 109 cm/s at 6 months or more after a ligament release procedure. Decrease of the postoperative celiac artery Doppler velocity was not associated with an improvement in gastrointestinal symptoms (P = .075). CONCLUSIONS: Adolescent and young adult patients with median arcuate ligament syndrome and OI have a good response to surgical intervention. About two-thirds of patients report significant improvement in symptoms of abdominal pain, nausea, and vomiting. Despite these encouraging data, many patients with MALS and OI continue to have an impaired quality of health.

Treatment of median arcuate ligament syndrome: outcome of laparoscopic approach / Tratamento da síndrome do ligamento arqueado mediano: resultado da abordagem laparoscópica

ABSTRACT Background: Median arcuate ligament syndrome(MALS) is a rare condition thatmay cause significant clinical manifestations, including abdominal pain and weight loss. Its diagnosis may be difficult and very often delayed. The laparoscopic approach became the standard treatment of MALS. Aim: To assess the outcome of laparoscopic treatment in patients with MALS. Method: The data of sixpatients with MALS who were subjected to laparoscopic sectioning of the median arcuate ligament were retrospectively reviewed.The following data were evaluated: age, gender, clinical and diagnostic tests findings, ASA score, operative findings and complications, postoperative complications and mortality, hospital stay duration, and hospital readmission.The diagnosis of MALS was established by CT angiography and/or MR angiography. Results: There were four (66.7%) women and two (33.3%) men aged from 32 to 60 years. The main symptoms were epigastric pain (100%) and weight loss (66.7%). The findings of high-grade stenosis of the proximal celiac axis and poststenotic dilation confirmed on angiography confirmed the diagnosis in all patients. Surgical procedure was uneventful in all patients. The only postoperative complication was urinary retention that occurred in a male. At three-month follow-up, all patients were asymptomatic. Conclusion: Laparoscopic treatment of MALS is safe and effective in relieving the clinical manifestations of patients.

RESUMO Racional: A síndrome do ligamento arqueado mediano (SLAM) é condição rara que pode causar manifestações clínicas significativas, incluindo dor abdominal e perda de peso. Seu diagnóstico pode ser difícil e muitas vezes estabelecido tardiamente. A abordagem laparoscópica tornou-se o tratamento padrão para ela. Objetivo: Avaliar o resultado do tratamento laparoscópico em pacientes com SLAM. Método: Os dados de seis pacientes com SLAM submetidos a ressecção laparoscópica do ligamento arqueado mediano foram revisados ​​retrospectivamente. Os seguintes dados avaliados foram: idade, gênero, resultados dosexames clínicos e complementares, escore ASA, achados e complicações operatórias, complicações e mortalidade pós-operatórias, tempo de internação e readmissão hospitalar. O diagnóstico de SLAM foi estabelecido por angiotomografia e/ou angiorressonância. Resultados: Havia quatro (66,7%) mulheres e dois (33,3%) homens com idades entre 32 e 60 anos. Os principais sintomas foram dor epigástrica (100%) e perda de peso (66,7%). Os achados de estenose de alto grau do tronco celíaco proximal e dilatação pós-estenótica observados na angiografia confirmaram o diagnóstico em todos os pacientes. O procedimento cirúrgico transcorreu sem intercorrências em todos os pacientes. A única complicação pós-operatória foi retenção urinária, que ocorreu em um homem. No seguimento de três meses, todos os pacientes estavam assintomáticos. Conclusão: O tratamento laparoscópico da SLAM é seguro e eficaz no alívio das manifestações clínicas dos pacientes.

Celiac artery compression: Dunbar syndrome.

Celiac artery compression syndrome, also referred to as median arcuate ligament syndrome, celiac axis syndrome or Dunbar syndrome is a rare disorder consequent to extrinsic compression of the celiac trunk by the median arcuate ligament. Doppler ultrasound, multi-slice computed tomography angiography, magnetic resonance angiography, or invasive selective angiography can identify stenosis of the initial segment of the celiac artery and confirm diagnosis. Treatment options include open surgical or videolaparoscopic section of the median arcuate ligament and the fibers of the celiac plexus, or percutaneous transluminal angioplasty via an endovascular approach. We report herein an interesting case of a 38-year-old woman diagnosed with this rare condition and successfully treated with the surgical strategy.

A síndrome da compressão da artéria celíaca, também denominada síndrome do ligamento arqueado mediano, síndrome do eixo celíaco ou síndrome de Dunbar, é uma doença rara causada pela compressão extrínseca do tronco celíaco pelo ligamento arqueado mediano. Ultrassonografia Doppler, angiotomografia computadorizada, angiorressonância magnética ou angiografia seletiva invasiva conseguem identificar a estenose do segmento inicial da artéria celíaca e confirmar o diagnóstico. As opções de tratamento incluem secção videolaparoscópica ou laparotômica (a céu aberto) do ligamento arqueado mediano e das fibras do plexo celíaco, assim como angioplastia transluminal percutânea. Relatamos o interessante caso de uma mulher de 38 anos de idade diagnosticada com essa rara condição e adequadamente tratada pela estratégia cirúrgica.

[Síndrome de compresión del ligamento arcuato medio en el adulto: a propósito de dos casos]. / Median arcuate ligament syndrome in adults with laparoscopic treatment: report of two cases.

The medium arcuate ligament syndrome is a rare disorder that results from luminal narrowing of the celiac artery by the insertion of diaphragmatic muscle or the celiac nerve plexus. The syndrome is characterized by weight loss, postprandial abdominal pain, nausea, vomiting and an epigastric murmur. Surgical management involves the complete division of the medial arcuate ligament. We present two cases satisfactorily treated by laparoscopic surgery. The role of minimally invasive surgery in the treatment of this syndrome is a safe alternative approach to open surgery.

El síndrome de ligamento arcuato medio es un trastorno poco frecuente que resulta del estrechamiento luminal de la arteria celíaca por la inserción de fibras musculares diafragmáticas o bandas fibrosas del plexo nervioso celíaco. El síndrome se caracteriza por pérdida de peso, dolor abdominal posprandial, náuseas, vómitos y un soplo epigástrico. El manejo quirúrgico implica la división completa del ligamento arqueado mediano. Presentamos dos casos tratados satisfactoriamente mediante cirugía laparoscópica. El papel de la cirugía mínimamente invasiva en el tratamiento de este síndrome es una vía de abordaje segura alternativa a la cirugía abierta.

Celiac artery compression: Dunbar syndrome / Síndrome da compressão da artéria celíaca (síndrome de Dunbar)

Celiac artery compression syndrome, also referred to as median arcuate ligament syndrome, celiac axis syndrome or Dunbar syndrome is a rare disorder consequent to extrinsic compression of the celiac trunk by the median arcuate ligament. Doppler ultrasound, multi-slice computed tomography angiography, magnetic resonance angiography, or invasive selective angiography can identify stenosis of the initial segment of the celiac artery and confirm diagnosis. Treatment options include open surgical or videolaparoscopic section of the median arcuate ligament and the fibers of the celiac plexus, or percutaneous transluminal angioplasty via an endovascular approach. We report herein an interesting case of a 38-year-old woman diagnosed with this rare condition and successfully treated with the surgical strategy

A síndrome da compressão da artéria celíaca, também denominada síndrome do ligamento arqueado mediano, síndrome do eixo celíaco ou síndrome de Dunbar, é uma doença rara causada pela compressão extrínseca do tronco celíaco pelo ligamento arqueado mediano. Ultrassonografia Doppler, angiotomografia computadorizada, angiorressonância magnética ou angiografia seletiva invasiva conseguem identificar a estenose do segmento inicial da artéria celíaca e confirmar o diagnóstico. As opções de tratamento incluem secção videolaparoscópica ou laparotômica (a céu aberto) do ligamento arqueado mediano e das fibras do plexo celíaco, assim como angioplastia transluminal percutânea. Relatamos o interessante caso de uma mulher de 38 anos de idade diagnosticada com essa rara condição e adequadamente tratada pela estratégia cirúrgica

Prevalence of signs of celiac axis compression by the median arcuate ligament on computed tomography angiography in asymptomatic patients.

OBJECTIVE: The increasing use of computed tomography (CT) angiography has led to more frequent diagnoses of celiac artery compression (CAC) by the median arcuate ligament (MAL). The signs of CAC by the MAL have been described as stenosis and a hook or J appearance on sagittal views. The importance of the "hook signal," however, has not been documented by studies of the normal anatomy of the celiac axis. METHODS: CT angiography images of 344 completely asymptomatic, live kidney donors (without history of chronic abdominal pain or weight loss) were reviewed. The angle of emergence (AE) of the celiac axis from the aorta and the angle of upward or downward shifting of the celiac axis before its first branch (fold angle [FA]) were measured. Weight, height, and body mass index were obtained from our electronic database, and correlations with the angles measured were tested. The occurrence of stenosis >50% at the origins of the celiac axis was also determined in the sample. RESULTS: Measurements were possible in 321 cases. The celiac axis was found to leave the aorta at an angle of <90 degrees in all patients (AE range, 7-83 degrees) and <45 degrees in 292 (90%) patients. The FA ranged from 66 to 208 degrees. Before the first branch, the celiac trunk shifted upward in 306 (95%) patients, remained straight in just one of them, and shifted downward in 14 (4%). The AE was positively correlated with weight in women. The FA was negatively correlated with weight in men and women. Body mass index was positively correlated with AE and negatively correlated with FA in both men and women. In 11 cases (3.4%), stenosis >50% was found at the origin of the celiac axis. In only two patients, the celiac axis had an upward slope after the stenosis, which could be interpreted as a hook shape. CONCLUSIONS: The normal anatomy of the celiac axis, when seen on CT angiography images, demonstrates that it exits the aorta downward and then shifts upward. This hook or J shape should not be interpreted as resulting from external compression. CAC by the MAL occurs in 3.42% of the normal asymptomatic population; a hook or J shape is not visible in most cases in that subgroup.

Laparoscopic treatment of celiac axis compression by the median arcuate ligament and endovascular repair of a pancreaticoduodenal artery aneurysm: case report.

Compression of the celiac axis by the median arcuate ligament of the diaphragm can cause nonspecific symptoms such as abdominal pain, vomiting, and weight loss. There is a known association between stenosis or occlusion of the celiac trunk and aneurysms of the pancreaticoduodenal artery. Treatment strategies for patients who have this association should be selected on a case-by-case basis. We describe the case of a patient with pancreaticoduodenal artery aneurysm associated with compression of the celiac trunk by the arcuate ligament, which were managed with endovascular and laparoscopic techniques, respectively.