The laparoscopic approach in the median arcuate ligament syndrome: report of a case

A malposition of the median arcuate ligament (MAL) is a rare entity causing the celiac axis compression syndrome (CACS), first described by Harjola in 1963. The presence of anomalous fibrous diaphragmatic bands of the diaphragm compresses the celiac axis, especially during the expiration. In this report we present the fourth case in literature that was ever successfully treated by laparoscopy. A 38-year-old male presented with a history of intermittent epigastric pain, 15 kg weight loss caused by inappetence and frequent diarrhoea, over a 5-year period. The clinical examination revealed only a loud systolic bruit in the epigastrium, with loss of intensity during deep inspiration. Suspecting CACS, a spiral CT angiography was requested. The CT demonstrated the MAL crossing anteriorly to the celiac artery (CA) and the sagittal and the tridimensional reconstructions demonstrated the CA narrowing due to compression, while the superior mesenteric artery (SMA) was normal. The MAL was laparoscopically divided, releasing the celiac axis. The surgical time was 130 minutes, without significant blood losses. At 3-months follow-up, the CT-scan demonstrated no evidence of CACS with complete recovery.     

Laparoscopic approach for the multiple abdominal vascular entrapment syndrome

We report an unusual case of a 25-year-old man affected by several vascular entrapment abdominal processes: celiac axis compression or Dunbar syndrome, superior mesenteric artery syndrome or Wilkie's disease and Nutcracker syndrome implying compression of the left renal vein. We discuss the diagnosis that was established by contrast-enhanced spiral computed tomography scan and magnetic resonance angiography. Once diagnosed the patient was treated conservatively by enteral high caloric diet in order to obtain a weight gain, however as it did not relieve the symptomatology of pain and vomit we opted for a surgical procedure. The therapeutic options were reviewed, highlighting the laparoscopic approach, which was the one we chose since it provides a less invasive procedure than open surgery but is an equally effective method of treatment.     

Chronic abdominal pain associated with intermittent compression of the celiac artery

Recurrent abdominal pain (RAP), surely one of the most frequent causes of medical intervention, is frequently present in many gastrointestinal disease. Usually no structural and/or biochemical alterations can be demonstrated. This condition is, therefore, considered to be due to functional disorders such as irritable bowel syndrome (IBS) or functional dyspepsia. Previous observations suggest the presence of a rare alteration of celiac vessels among the possible causes of RAP. This pathological condition was known as Dunbar syndrome. We report 2 cases of chronic abdominal pain. The former reported weight loss and the latter anemia with iron deficiency. It is remarkable that patients with initial diagnosis of IBS can be affected by celiac disease (CD), which is the cause of their abdominal pain. Our patients were tested for CD; the former was negative and IBS was diagnosed, the latter was positive and a gluten free diet was prescribed. The presence of an epigastric bruit, accentuated during expiration, suggested a possible vascular alteration known as tripod celiac artery compression syndrome. Duplex Doppler sonography suggests the diagnosis of celiac arterial constriction due the diaphragmatic ligament. These cases show that tripod celiac artery compression syndrome might be a cause of RAP and that it may be evaluated and investigated when the clinical examination discloses an abdominal systolic bruit.     

Sobreposición entre los trastornos funcionales de dolor abdominal y enfermedades orgánicas en niños

Functional abdominal pain disorders are highly prevalent in children. These disorders can be present in isolation or combined with organic diseases, such as celiac disease and inflammatory bowel diseases. Intestinal inflammation (infectious and non-infectious) predisposes children to the development of visceral hypersensitivity that can manifest as functional abdominal pain disorders, including irritable bowel syndrome. The new onset of irritable bowel syndrome symptoms in a patient with an underlying organic disease, such as inflammatory bowel disease, is clinically challenging, given that the same symptomatology may represent a flare-up of the inflammatory bowel disease or an overlapping functional abdominal pain disorder. Similarly, irritable bowel syndrome symptoms in a child previously diagnosed with celiac disease may occur due to poorly controlled celiac disease or the overlap with a functional abdominal pain disorder. There is little research on the overlap of functional abdominal disorders with organic diseases in children. Studies suggest that the overlap between functional abdominal pain disorders and inflammatory bowel disease is more common in adults than in children. The causes for these differences in prevalence are unknown. Only a handful of studies have been published on the overlap between celiac disease and functional abdominal pain disorders in children. The present article provides a review of the literature on the overlap between celiac disease, inflammatory bowel disease, and functional abdominal pain disorders in children and establish comparisons with studies conducted on adults.     

Diagnosis and management of splanchnic ischemia

Splanchnic or gastrointestinal ischemia is rare and randomized studies are absent. This review focuses on new developments in clinical presentation, diagnostic approaches, and treatments. Splanchnic ischemia can be caused by occlusions of arteries or veins and by physiological vasoconstriction during low-flow states. The prevalence of significant splanchnic arterial stenoses is high, but it remains mostly asymptomatic due to abundant collateral circulation. This is known as chronic splanchnic disease （CSD）. Chronic splanchnic syndrome （CSS） occurs when ischemic symptoms develop. Ischemic symptoms are characterized by postprandial pain, fear of eating and weight loss. CSS is diagnosed by a test for actual ischemia. Recently, gastro-intestinal tonometry has been validated as a diagnostic test to detect splanchnic ischemia and to guide treatment. In single- vessel CSD, the complication rate is very low, but some patients have ischemic complaints, and can be treated successfully. In multi-vessel stenoses, the complication rate is considerable, while most have CSS and treatment should be strongly considered. CT and MR-based angiographic reconstruction techniques have emerged as alternatives for digital subtraction angiography for imaging of splanchnic vessels. Duplex ultrasound is still the first choice for screening purposes. The strengths and weaknesses of each modality will be discussed. CSS may be treated by minimally invasive endoscopic treatment of the celiac axis compression syndrome, endovascular antegrade stenting, or laparotomy-assisted retrograde endovascular recanalization and stenting.The treatment plan is highly individualized and is mainly based on precise vessel anatomy, body weight, co- morbidity and severity of ischemia.     

Recurrent celiac artery compression syndrome

The celiac artery compression syndrome (CACS) is an infrequently described clinical condition with poorly defined diagnostic criteria and an obscure pathophysiology. It is usually associated with an extrinsic compression upon the celiac axis near its takeoff from the aorta by fibrous diaphragmatic bands or sympathetic neural fibers. We present a patient with CACS who suffered a recurrence of her original abdominal complaints.     

Superior mesenteric artery syndrome caused by celiac axis compression syndrome: a case report and review of the literature

Celiac axis compression syndrome (CACS) is a rare entity of mesenteric ischemia, secondary to inadequate blood supply to the intestine, resulting in weight loss because of postprandial abdominal pain. Superior mesenteric artery (SMA) syndrome is an uncommon cause of intestinal obstruction manifesting with epigastric pain, bilious vomiting, and postprandial discomfort. Although the coexistence of both syndromes is very rare and has been reported only in eight patients in the literature, the CACS as a rare etiology of SMA syndrome has not yet been reported. Herein, we describe an uncommon case of SMA syndrome secondary to the CACS. The 27-year-old woman presented with epigastric pain, postprandial vomiting, and rapid body weight loss. The diagnosis of SMA syndrome was made by hypotonic duodenography and multidetector computer tomographic angiography. The CACS was also suspected by multidetector computer tomographic angiography. Surgical intervention was performed and the presence of CACS was confirmed. Her symptoms subsided shortly after operation and she was in good health at 1-year follow-up.     

Median arcuate ligament syndrome

Opinion statement Median arcuate ligament syndrome (MALS) can cause a range of symptoms, including abdominal pain, nausea, vomiting, and weight loss. Because all patients have some degree of celiac artery compression by the median arcuate ligament (MAL), it may be difficult to discern which patients have a pathologic compression. Based on the multiple theories of MALS etiology, it is unlikely that we know the true cause of this syndrome. In fact, there are many physicians who question the validity of the diagnosis of MALS. Before offering intervention for MALS, a thorough gastrointestinal evaluation should be performed, including consideration of diagnostic temporary percutaneous celiac ganglion block. Patients who are on chronic narcotics preoperatively have a lower likelihood of postoperative symptom relief and therefore should be evaluated by a pain specialist preoperatively. The most reliable treatment comprises open surgical treatment with division of the MAL, removal of surrounding celiac ganglion, evaluation of the celiac artery with pressure measurements or ultrasound, and celiac artery reconstruction if indicated. Laparoscopic and endovascular interventions are novel treatments and may be considered in select patients who cannot undergo an open surgical procedure.     

Celiac artery compression syndrome

Celiac artery compression syndrome has been referred to in the literature as an entity in limbo, yet there have been many cases that have been well documented. This report describes two cases of abdominal pain with epigastric bruits in which the diagnosis of celiac artery stenosis was made. The abdominal bruit with its diastolic component had been the “tip-off” in both of these patients. Both patients did poorly with conservative treatment and eventually required surgical decompression.     

Lethal nature of ischemic gastropathy: a case report of celiomesenteric vascular insufficiency

Celiomesenteric ischemia has an insidious onset and the non-specific symptoms are often misdiagnosed as cholecystitis or peptic ulcer disease with a consequential delay between the onset of symptoms and radiological evidence of vascular occlusive disease. A elderly man was hospitalized after a 2–3 week history of acute abdominal pain, frequent vomiting, and chronic diarrhea associated with bloody stools. Upper gastrointestinal endoscopy showed a cobblestone gastric pattern with multiple ulcerated areas and the specimens indicated focal full thickness coagulative necrosis of the mucosa. A magnetic resonance angiography (MRA) revealed a widespread and severe atheromatous disease characterized by a hemodynamically significant stenosis of the celiac tripod, pre-occlusive stenosis of the superior mesenteric artery and complete occlusion of the inferior mesenteric artery. MRA is now the best and most accessible noninvasive examination to help establish a diagnosis, providing high-quality three-dimensional images of the celiac axis and mesenteric arteries. However, the various features observed in this case such as the clinical history, the presence of a cobblestone pattern with multiple ulcerated areas in the stomach, the coagulative-type necrosis and ischemic atrophy of the adjacent mucosa were considered diagnostic of gastric ischemia.     

Isolated Spontaneous Celiac Artery Dissection in a 47-Year-Old Man with von Willebrand Disease

Isolated spontaneous dissection of the celiac artery is rare, and its occurrence without aortic dissection is even rarer. The typical symptom of this dissection is acute-onset abdominal pain. Complications of the condition include aneurysm formation, rupture, and abdominal-organ ischemia or infarction, especially in the liver or spleen. We report the case of a 47-year-old man with von Willebrand disease who had an isolated spontaneous dissection of the celiac artery. We used computed tomography and computed tomographic angiography in the diagnosis and characterization of the dissection. To our knowledge, this is the first report of celiac artery dissection in a patient with von Willebrand disease.     

Association syndrome de Budd-Chiari et maladie c?liaque : une nouvelle observation

The association between Budd-Chiari syndrome and celiac disease is rare and has been reported in only 15 cases. We report 21-year-old women who developed a celiac disease and Budd-Chiari syndrome. The etiology for the Budd-Chiari syndrome was the antiphospholipid antibody syndrome and a deficiency of protein C. A gluten-free diet and an anticoagulant therapy led to a favorable outcome. Through this observation and a review of literature, we study the pathogenic link between these two diseases.     

Techniques and results of neurolysis for chronic pancreatitis and pancreatic cancer pain

Chronic abdominal pain can be associated with benign and malignant disease. Pain associated with pancreatic cancer and chronic pancreatitis can be severely debilitating, with significant impairment in quality of life. Frequently, chronic abdominal pain is not adequately responsive to conventional medical therapies, including nonsteroidal anti-inflammatory drugs and opioids. For this reason, alternative methods to alleviate pain have been developed. Celiac plexus neurolysis and celiac block involve injecting an agent at the celiac axis, with the goal of either selectively destroying the celiac plexus or temporarily blocking visceral afferent nociceptors to alleviate chronic abdominal pain. Agents most commonly used for this purpose include alcohol or phenol for neurolysis and bupivacaine and triamcinolone for temporary block. Methods to administer such agents to the celiac ganglion include CT imaging, percutaneous ultrasound, fluoroscopy, endoscopic ultrasound, or surgery (ganglionectomy). Response rates and complications vary depending on technique but are relatively low. This review highlights the techniques of celiac plexus neurolysis and celiac block and their status in the treatment of chronic pancreatitis and pancreatic cancer pain.     

Exercise-related abdominal pain as a manifestation of the median arcuate ligament syndrome

Abdominal pain related to exercise, often loosely referred to as 'stitch', is not uncommon, particularly among participants in sports that involve running. The cause of this typically transient pain is poorly understood with several aetiologies proposed including diaphragmatic ischaemia (1, 2). Other gastrointestinal symptoms that are common during prolonged or high-intensity exercise include nausea, diarrhoea and gastrointestinal bleeding (3, 4). These symptoms are also usually transient and are thought to protect against critical organ damage by promoting cessation of exercise. Decreased gastrointestinal blood flow, increased motility and altered neuroendocrine modulation are postulated disease mechanisms (3). We report here a case of an elite runner with exercise-related severe abdominal pain and diarrhoea related to compression of the coeliac axis by the median arcuate ligament. Complete symptom relief was achieved with surgical division of the constricting ligament. The clinical characteristics and pathogenesis of coeliac axis compression syndrome are discussed.     

Syndrome de Budd-Chiari associé à une maladie cœliaque

The association of Budd-Chiari syndrome and celiac disease is rare and has been reported in only 13 cases. We report a 23-year-old man with celiac disease, treated with gluten-free diet since the age of 16 years. He presented with epigastric pain that was secondary to a Budd-Chiari syndrome. No other cause than celiac disease could be identified.     

Pancreaticoduodenal artery aneurysms associated with celiac axis stenosis due to compression by median arcuate ligament and celiac plexus

Celiac axis stenosis is frequently associated with pancreaticoduodenal artery aneurysms. Although the cause of stenosis was not clear in most of the reported cases, compression of the median arcuate ligament of the diaphragm was found to be responsible for the stenosis in 7 of 42 reported cases of this type of aneurysm. We report a case of aneurysm caused by compression of the median arcuate ligament of the diaphragm and celiac plexus. An asymptomatic 43-year-old Japanese man was admitted with a low echoic lesion in the uncus of pancreas. Computed tomographic scan and angiogram revealed stenosis of the celiac axis and two aneurysms in the inferior posterior pancreaticoduodenal artery. The celiac plexus and median arcuate ligament were divided surgically and normal flow was reestablished in the celiac axis. One of the aneurysms was resected and the afferent artery of the other aneurysm was ligated. In the setting of pancreaticoduodenal artery aneurysm associated with celiac axis stenosis, management of stenosis should be considered in addition to local treatment of the aneurysm. In this context, division of median arcuate ligament and celiac plexus or aorto-celiac bypass may normalize the flows in the pancreaticoduodenal arcade and could be effective in preventing aneurysm reformation. (Received May 12, 1997; accepted Sept. 26, 1997)     

Arcuate Ligament Syndrome Mimicking Celiac Occlusion

In a patient with weight loss and a systolic epigastric murmur, aortography demonstrated positional celiac axis occlusion. Color Doppler imaging of the celiac axis showed occlusion in the supine position, but patency was documented during deep inspiration and with the patient in the upright position. This report documents a rare example of extreme celiac axis compression with position-dependent celiac patency.     

An uncommon association of abdominal vascular compression syndromes: Dumbar and Nutcracker

Abdominal pain associated with nausea and vomiting in a young patient led to a diagnosis of median arcuate ligament syndrome. The presence of mild haematuria was associated with a concomitant Nutcracker syndrome. Diagnosis was achieved by a computed tomography scan, which showed compression of the vessels of the coeliac axis and left renal vein. These syndromes are very rare, and their association in the same patient has not been described before. There is no relationship in the aetiology of these entities. In this report we discuss the diagnosis and therapeutic options, and review the literature.     

33-jährige Patientin mit thorako-abdominellen Schmerzen und Blutdruckdifferenz

We report on a 33-year-old women with ulcerative colitis, who presented with inspiratory thoracic pain due to perimyocarditis. Furthermore, postprandial abdominal pain associated with diarrhea and different blood pressure values on both arms were recorded. Computed tomography revealed embolism of the pulmonal arteries and angiography demonstrated stenoses of the subclavian and renal arteries and the celiac trunc suggesting the diagnosis of Takayasu's arteritis. A coronary or infectious etiology could be excluded. Since a coincidence of Takayasu's arteritis and ulcerative colitis has been reported in the literature, abdominal pain and diarrhea can either be explained by the inflammatory bowel disease or by chronic ischemia due to intestinal vasculitis. Differential diagnosis will be facilitated by the future course of the disease.     

Laparoscopic management of median arcuate ligament syndrome

Median arcuate ligament syndrome is a rare disorder resulting from luminal narrowing of the celiac trunk. The classic management of median arcuate ligament syndrome involves the surgical division of the median arcuate ligament fibers in order to decompress the celiac trunk. This has traditionally required an upper midline incision. A few authors have described a successful laparoscopic release of celiac artery compression syndrome. Laparoscopy provides a less invasive, but equally effective method for decompressing the celiac trunk.