Pancreaticoduodenal artery aneurysm associated with coeliac trunk occlusion treated with aorto-hepatic bypass and aneurysm resection.

Pancreaticoduodenal artery aneurysms are rare visceral artery aneurysms that may be associated with stenosis of the coeliac trunk. We present a case of a 48-year-old woman with a symptomatic aneurysm of the inferior pancreaticoduodenal artery caused by occlusion of the coeliac trunk due to median arcuate ligament syndrome. The patient was treated with an aorto-hepatic bypass and excision of the aneurysm. We discuss the case and the complexities and controversies in the treatment of this disease.

Combination between Dunbar Syndrome and May-Thurner Syndrome: A Rare Case Report.

Dunbar syndrome (DS) and May-Thurner syndrome (MTS) are part of a group of rare vascular disorders known as "vascular compression syndromes." Dunbar's syndrome is caused by the median arcuate ligament of diaphragm, which, due to an abnormal course, causes celiac artery compression. MTS is caused by the left common iliac vein compression pushed against the spine by the right common iliac artery causing progressive flow congestion and leading to thrombosis. Ultrasound is the first-level examination for the diagnosis of these rare pathologies and allows to recognize vascular compressions and to obtain an estimate of stenosis degree. We describe a very rare case of DS and MTS combination in a young man with postprandial pain and left lower limb thrombosis.

The Ultrasound Findings in a Rare Case of Nutcracker Syndrome, Wilkie's Syndrome, and Dunbar Syndrome Combination.

Vascular compression syndromes represent a group of rare and poorly understood diseases. Dunbar syndrome (DS) is caused by the median arcuate ligament of diaphragm originating lower than normal and causing compression of celiac artery. The Nutcracker is caused by the superior mesenteric artery (SMA) originating from aorta at an acute angle causing a restriction of aortomesenteric space that is traversed by the left renal vein and duodenum; if the compression involves only the left renal vein and becomes symptomatic it is called Nutcracker syndrome; if the symptomatic compression involves only the duodenum it is called Wilkie's syndrome or SMA syndrome. The knowledge of these rare pathologies is essential to reduce the false negatives which still remain very high; it is, therefore, necessary to promote greater knowledge as the lack of diagnosis can be very dangerous for the patient's health. We describe a rare case of a combination of DS, Nutcracker, and SMA or Wilkie's syndrome in a young patient.

Systematic Review of the Efficacy of Treatment for Median Arcuate Ligament Syndrome.

OBJECTIVE: Since the first description of the median arcuate ligament syndrome (MALS), the existence for the syndrome and the efficacy of treatment for it have been questioned. METHODS: A systematic review conforming to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement was conducted, with a broader view on treatment for MALS including any kind of coeliac artery release, coeliac plexus resection, and coeliac plexus blockage, irrespective of age. Online databases were used to identify papers published between 1963 and July 2021. The inclusion criteria were abdominal symptoms, proof of MALS on imaging, and articles reporting at least three patients. Primary outcomes were symptom relief and quality of life (QoL). RESULTS: Thirty-eight studies describing 880 adult patients and six studies describing 195 paediatric patients were included. The majority of the adult studies reported symptom relief of more than 70% from three to 228 months after treatment. Two adult studies showed an improved QoL after treatment. Half of the paediatric studies reported symptom relief of more than 70% from six to 62 months after laparoscopic coeliac artery release, and four studies reported an improved QoL. Thirty-five (92%) adult studies and five (83%) paediatric studies scored a high or unclear risk of bias for the majority of the Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) items. The meaning of coeliac plexus resection or blockage could not be substantiated. CONCLUSION: This systematic review suggests a sustainable symptom relief of more than 70% after treatment for MALS in the majority of adult and paediatric studies; however, owing to the heterogeneity of the inclusion criteria and outcome parameters, the risk of bias was high and a formal meta-analysis could not be performed. To improve care for patients with MALS the next steps would be to deal with reporting standards, outcome definitions, and consensus descriptions of the intervention(s), after which an appropriate randomised controlled trial should be performed.

DUNBAR SYNDROME: CLINICAL MANIFESTATION IN ADULTS, DIAGNOSTIC PROBLEMS (CASE REPORT).

In this case we used CBC and biochemical tests, ECG, ultrasound of the abdominal cavity and heart, CT scan with and without IV contrast. Women with complains on recurrent severe abdominal pain in epigastric region and right part of the abdomen radiating to the back, nausea, vomiting. Making laboratory and instrumental tests for confirmation of the diagnosis. Surgical treatment of DS was performed and after one year of the follow up there were no complications. DS may mimic other medical conditions such as gallbladder diseases, gastritis/peptic ulcer, appendicitis, colorectal malignancy, hepatitis, atherosclerotic diseases etc. That is why DS is a diagnosis of exclusion. This case illustrates pathway to find correct diagnosis and improve management tactic.

Inferior pancreaticoduodenal artery aneurysms and Dunbar syndrome. Experience with the open surgery.

INTRODUCTION: Median arcuate ligament or Dunbar syndrome is a rare vascular disorder causing celiac trunk stenosis or occlusion due to the extrinsic compression by the median arcuate ligament. A visceral aneurysm may develop as a complication. PATIENTS AND METHODS: We experienced an inferior pancreaticoduodenal artery aneurysm presentation in two female patients with the median arcuate syndrome previously diagnosed. One patient presented with postprandial abdominal pain and weight loss and the other one was asymptomatic. RESULTS: We described our experience with the both patients being successfully treated surgically. CONCLUSIONS: Awareness of possible repercussions of the celiac trunk stenosis to peripancreatic arteries should be elevated. Restoration of physiological blood flow by removing pathologic anatomy should be performed. Since there is no correlation between the size of a visceral aneurysm and the risk of rupture, having a high postrupture mortality, pancreaticoduodenal artery aneurysms should be treated even if asymptomatic. Surgical treatment remains the mainstay treatment of the Dunbar syndrome complicated by visceral aneurysms.

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.

Robotic-assisted laparoscopic median arcuate ligament release: 7-year experience from a single tertiary care center.

BACKGROUND: Despite previous reports of robotic-assisted laparoscopic release for median arcuate ligament syndrome (MALS), the safety and efficacy profile of this approach has been difficult to establish due to the rarity of this diagnostic entity. We aim to present our experience from a tertiary minimally invasive surgery referral center. METHODS: A case series was performed whereby all patients who underwent robotic-assisted MAL release from July 2010 to July 2017 at our institution were included. Diagnosis of MALS was made based on consideration of symptom presentation, celiac artery duplex ultrasound, and corresponding findings on Computed Tomography (CT) or Magnetic Resonance Angiography (MRA). Outcomes up until the most recent clinic follow-up were reviewed. RESULTS: A total of 13 patients underwent robotic-assisted MAL release. Patients' age ranged from 16 to 71 years (mean 38 years) and consisted primarily of females (76.9%). Most common presenting symptoms included postprandial pain (76.9%), weight loss (76.9%), nausea and vomiting (76.9%). Mean symptom duration was 3 years (range 1-10 years). No intraoperative complications. None required conversion to open surgery. One case required a conversion back to laparoscopy due to anatomical complexity. The mean operative time for successfully completed robotic cases was 94.6 min (range 52-120 min), and for all cases including converted case was 103.5 min (52-210 min). Mean follow-up duration was 19.7 months (range 1-77 months). During subsequent follow-up, a 30-day readmission rate of 23.1% was observed. All but one of the patients experienced prompt symptom improvement. Four patients had symptom recurrence during follow-up. CONCLUSIONS: Our experience demonstrates that the robotic-assisted approach to MAL release may be safe and efficacious in selected patients. Prospective comparative studies are required to further evaluate its outcomes against conventional laparoscopic approach, the current gold standard.

Subacute cutaneous lupus erythematosus inducing unilateral sensorimotor neuropathy.

We describe the case of a 32-year-old male with previous history of subacute cutaneous lupus erythematosus (SCLE) who presented with arthritis followed by a unilateral lower-limb sensorimotor impairment, without biochemical or immunological marker abnormalities. Our patient currently satisfies only three of the systemic lupus international collaborating clinics criteria. Management of such patients is challenging due to lack of examples in the literature, with this case being the first described where a seronegative patient with SCLE demonstrated neurological involvement. A brief review of the available literature is included.

Open vascular treatment of median arcuate ligament syndrome.

BACKGROUND: Median arcuate ligament syndrome is a rare condition with abdominal symptoms. Accepted treatment options are open release of median arcuate ligament, laparoscopic release of edian arcuate ligament, robot-assisted release of median arcuate ligament and open vascular treatment. Here we aimed to evaluate the central priority of open vascular therapy in the treatment of median arcuate ligament syndrome. METHODS: We conducted a monocentric retrospective study between January 1996 and June 2016. Thirty-one patients with median arcuate ligament syndrome underwent open vascular surgery, including division of median arcuate ligament in 17 cases, and vascular reconstruction of the celiac artery in 14 cases. RESULTS: In a 20-year period, 31 patients (n = 26 women, n = 5 men) were treated with division of median arcuate ligament (n = 17) or vascular reconstruction in combination with division of median arcuate ligament (n = 14). The mean age of patients was 44.8 ± 15.13 years. The complication rate was 16.1% (n = 5). Revisions were performed in 4 cases. The 30-day mortality rate was 0%. The mean in-hospital stay was 10.7 days. Follow-up data were obtained for 30 patients. The mean follow-up period was 52.2 months (range 2-149 months). Patients were grouped into a decompression group (n = 17) and revascularisation group (n = 13). The estimated Freedom From Symptoms rates were 93.3, 77.8, and 69.1% for the decompression group and 100, 83.3, and 83.3% for the revascularisation group after 12, 24 and 60 months respectively. We found no significant difference in the Freedom From Re-Intervention CA rates of the decompression (100% at 12, 24 and 60 months post-surgery) and revascularisation (100% at 12 months, and 91.7% at 24 and 60 months post-surgery) groups during follow-up (p = 0.26). CONCLUSIONS: Open vascular treatment of median arcuate ligament syndrome is a safe, low mortality-risk procedure, with low morbidity rate. Treatment choice depends on the clinical and morphological situation of each patient.

[Laparoscopic decompression of celiac trunk in children]. / Laparoskopicheskaia dekompressiia chrevnogo stvola pri kompressionnom stenoze u detei.

MATERIAL AND METHODS: For the period 2013-2016 four patients were treated at the Filatov Children's City Clinical Hospital #13. There were 2 children aged 14 years and 2 children aged 17 years. All patients have been diagnosed via anamnesis, complaints, pulse-wave doppler sonography, contrast-enhanced MDCT and angiography. After comprehensive examination 3 patients underwent laparoscopic decompression of celiac trunk. In all cases celiac trunk compression was predominantly caused by median arcuate ligament of the diaphragm combined with neurofibrotic tissue of celiac plexus. RESULTS: All patients were discharged after laparoscopic decompression of celiac trunk. Intra- and postoperative complications, as well as cases of conversion were absent. Mean time of surgery was 65 minutes. In all cases postoperative period was smooth (4 days on the average). Two patients underwent follow-up examination in long-term postoperative period: pulse-wave doppler sonography, contrast-enhanced MDCT and angiography. In both cases reduced severity, incidence and duration of pain syndrome were observed. CONCLUSION: Clinical examples show some problems in diagnosis and treatment of compressive stenosis of celiac trunk due to rarity of pathology especially in childhood. Nevertheless, combination of abdominal ischemia and celiac trunk stenosis confirmed by instrumental diagnosis is indication for surgical treatment.

Laparoscopic treatment of celiac artery compression syndrome in children and adolescents.

BACKGROUND: The celiac artery compression syndrome (CACS) is a rarely diagnosed disorder, which is characterized by chronic abdominal pain and vegetative symptoms. The role of surgical treatment in celiac artery decompression has been discussed controversially by numerous authors. After first casuistic descriptions of a laparoscopic treatment in adults we established this novel minimally invasive procedure for treatment in children and adolescents. PATIENTS AND METHODS: Between 2005 and 2014 we operated 58 patients (47 female, 11 male) from 7 to 25 years who had been diagnosed with celiac artery compression. The patients presented with severe chronic abdominal pain, vegetative symptoms and a reduced quality of life. Doppler sonography showed an increased blood flow velocity of the celiac artery with maximum of 190 - 450 cm/s (mean 259 cm/s).MR angiography demonstrated a characteristic hook-shaped appearance of the celiac artery with severe localized compression. RESULTS: All patients underwent laparoscopic decompression of the celiac artery. We observed complications in 3 patients (5,2 %). Postoperatively all patients (100 %) were immediately free of abdominal pain. Doppler sonography showed a marked reduction in celiac blood flow velocity to 70 - 190 cm/s postoperatively (mean 178 cm/s). A return of vessel diameters to normal dimensions was documented by postoperative MR angiography. During a median follow up of 62 months we observed a recurrence of the celiac artery compression in 4 patients (6,9 %). CONCLUSIONS: Laparoscopic treatment of celiac artery compression syndrome offers a novel, safe, reliable and, compared to open surgery, less invasive approach. The surgical treatment is indicated in patients with characteristic symptoms and typical findings at Doppler sonography and MRA after exclusion of other abdominal pathologies. The work-up of chronic abdominal pain in children and adolescents should include a color Doppler sonography to look for celiac artery compression.

Practice-Based Management Data of Consecutive Subjects Assessed for the Median Arcuate Ligament Syndrome at a Single Tertiary Institution.

BACKGROUND: The pathophysiology of median arcuate ligament syndrome (MALS) is poorly understood. The diagnostic process remains inadequately standardized, with an absence of precise criteria to guide therapeutic management. METHODS: We studied consecutive subjects referred to the Department of Angiology at the University Hospital of Zurich over the past 17 years due to suspected MALS. We focused on (1) the imaging criteria that led to diagnosis, notably the results of color duplex ultrasound and the consistency with different imaging tests; (2) the clinical consequences focusing on symptom resolution. RESULTS: We included 33 subjects; in 8 subjects (24.2%), the diagnosis of MALS was retained. The median expiration peak systolic velocity (PSV) on ultrasound was 3.05 (Q1; 2.1-Q3; 3.3). To confirm the sonographic results, either a CT or MRI was performed on all patients, with consistent findings confirming a significant stenosis. Seven patients underwent surgery, all involving arcuate ligament release. Four procedures were laparoscopic, one was via laparotomy, and two were robot-assisted. Additionally, two patients required angioplasty with stenting as a secondary intervention. Only two (28.6%) of the seven operated patients experienced a relief of symptoms. None experienced a relief of symptoms following secondary angioplasty, despite stent patency. The prevalence of psychiatric disorders was comparable between patients with retained and rejected diagnoses, 38% and 36%, respectively. CONCLUSIONS: Our study confirmed sonography and CT/MRI consistency. However, most patients with MALS did not benefit from invasive treatment. The majority (83%) of patients without MALS were diagnosed with alternative conditions, mainly functional disorders.

A Novel Minimally Invasive Robotic-Assisted Surgery Technique for Treatment of Median Arcuate Ligament Syndrome: A Case Report.

Median arcuate ligament syndrome (MALS), also known as Dunbar syndrome, is a rare but significant cause of chronic abdominal pain resulting from the extrinsic compression of the celiac trunk. This condition typically manifests with symptoms such as postprandial pain, nausea, vomiting, and weight loss, often leading to a diagnostic challenge due to its mimicry of other abdominal disorders. Diagnosis is based on exclusion and requires a high index of suspicion combined with precise imaging findings. This case report presents a 44-year-old female patient presenting with chronic abdominal pain, diaphoresis, and nausea, underscoring the complexity and diagnostic challenge of MALS, highlighting the significance of early intervention to mitigate morbidity and novelty treatment utilizing robotic-assisted surgical techniques. This report aims to contribute to the understanding of clinical presentations, diagnostic challenges, and treatment modalities of Dunbar syndrome, especially the option of minimally invasive robotic-assisted surgery for the treatment of this condition.

Recurrent aneurysmatic bleeding of pancreaticoduodenal aneurysm due to median arcuate ligament syndrome: a case report.

Median arcuate ligament syndrome (MALS) involves coeliac artery compression, causing a range of symptoms from chronic pain to life-threatening complications. This case features a 52-year-old patient with recurrent retroperitoneal bleeding from MALS-related inferior pancreaticoduodenal artery aneurysms (PDAAs). Emergency interventions, including surgical bleeding control, angioplasty, percutaneous drainage, and median arcuate ligament release, were conducted. The case highlights challenges in diagnosing and managing MALS-related PDAA, emphasizing the importance of early identification and tailored interventions based on clinical symptoms and imaging. Surgical intervention to release the ligament is the primary treatment, with considerations for prophylactic intervention in PDAA cases. Lack of established PDAA management protocols underscores the need for prompt intervention to prevent complications. In conclusion, this report stresses the association between MALS and PDAA, advocating for early identification and tailored management to mitigate complications.

Median arcuate ligament syndrome: A rare case report from Nepal.

INTRODUCTION: Median arcuate ligament syndrome (MALS) is a rare condition caused by the compression of the celiac trunk by the median arcuate ligament, leading to a typical symptom triad: postprandial abdominal pain, weight loss, nausea, and vomiting. CASE PRESENTATION: A 41-year-old female patient presented to our center with mild postprandial abdominal pain over the epigastric region, and bloating sensation. Ultrasonography of the abdomen showed multiple stones in the gall bladder lumen, and the computed tomography scan showed median arcuate ligament impingement along the proximal aspect of the celiac trunk causing moderate narrowing with post-stenotic dilation. Laparoscopic release of the median arcuate ligament with laparoscopic cholecystectomy was performed. DISCUSSION: The diagnosis of Median Arcuate Ligament Syndrome is based on the classical post-prandial symptoms and abdominal imaging technologies like Doppler ultrasonography, computed tomography angiography, or magnetic resonance angiography. Exclusion of other intestinal disorders should be considered before making the diagnosis. Celiac artery decompression through different means is the principle of treatment of this condition. CONCLUSION: The diagnosis of median arcuate ligament syndrome should be considered in patients with postprandial abdominal pain that does not have an established etiology. Celiac artery decompression by releasing the median arcuate ligament is the treatment.

Laparoscopic Treatment of a Symptomatic Young Woman With Median Arcuate Ligament Syndrome.

Median arcuate ligament syndrome (MALS), also known as Dunbar syndrome, celiac axis syndrome, or celiac artery compression syndrome, is caused by a band of tissue called the median arcuate ligament that compresses the celiac artery and sometimes the celiac plexus too. MALS does not always cause symptoms, but when symptoms occur, surgery is the treatment of choice. This case report focuses on the case of a 27-year-old woman presenting with postprandial episodes of abdominal pain and vomiting accompanied by loss of weight, which was found to be MALS.

Celiac Artery Compression Syndrome in an Adolescent Male.

Celiac artery compression syndrome is not frequent in the pediatric population. The syndrome may entail long-standing abdominal pain, recurrent vomiting, bloating, weight loss, and an abdominal bruit, which in the case of our patient, was an incidental finding. Notably, patients may be asymptomatic.  Our patient is a 16-year-old male who presented with concerns about multiple, non-tender chest lymph nodes lasting for two weeks. He had also lost 80 lbs. over one year. On examination, however, an abdominal bruit was discovered, and a diagnostic workup was significant for celiac artery compression following a magnetic resonance angiography of the abdomen. Due to his significant weight loss and mediastinal lymphadenopathy, a chest computed tomography (CT) scan was done to rule out malignancy. The chest CT scan was reported as normal. Additionally, a renal duplex ultrasound was done to rule out renal artery stenosis, considering he had presented with elevated blood pressure; this was also unremarkable. Although this patient had a history of marijuana use, his assessment did not show marked dependence. Substance abuse and atherosclerotic vascular disease can be predisposing factors for celiac artery compression syndrome in older individuals. However, compression of the celiac trunk by the median arcuate ligament is a congenital anomaly more appreciated in younger age groups. The patient was referred to vascular surgery for possible median arcuate ligament release.

Abdominal Pain Associated With Celiac Artery Compression and Superior Mesenteric Artery (SMA) Syndrome: A Case Report.

Celiac artery compression syndrome (CACS) is an elusive cause of postprandial abdominal pain, commonly mistaken for conditions such as gall bladder disease. A 36-year-old male presented with postprandial abdominal pain. Imaging studies revealed compression of the celiac artery by the median arcuate ligament and an incidentally detected narrow aortomesenteric angle, indicating superior mesenteric artery (SMA) syndrome. The patient underwent laparoscopic release of the median arcuate ligament followed by celiac artery stenting to alleviate symptoms associated with CACS. This case highlights the coexistence of CACS and SMA syndrome in a single patient. In this case, the treatment was focused on addressing the symptoms of CACS, as the SMA syndrome did not significantly impact the patient's well-being. Thus, attention should be given to vascular phenomena when evaluating abdominal pain.

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.