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Cureus Jul 2021Celiac artery compression syndrome (CACS), also known as median arcuate ligament syndrome, celiac axis syndrome, and Dunbar Syndrome, is a rare disorder that results...
Celiac artery compression syndrome (CACS), also known as median arcuate ligament syndrome, celiac axis syndrome, and Dunbar Syndrome, is a rare disorder that results from compression of the celiac artery by the median arcuate ligament. The following is a case that depicts an interesting presentation of a patient diagnosed with this rare condition. A 44-year-old male with a history of mutism was brought in by his family for weight loss of 100 lbs with intermittent abdominal pain, weakness and lethargy over a period of five years. His family reported that he had poor nutritional intake, and could only eat a small amount before he seemed to be in pain, and eventually refused to eat. He had no other prior medical history except for mutism, no family history of malignancy, no history of trauma, surgeries, smoking or substance use, and did not take any medications. Physical exam was largely unremarkable. Mesenteric vascular duplex demonstrated severe grade stenosis of the celiac trunk with post-stenotic velocity of 520 cm/sec. Contrast enhanced computed tomography angiography revealed acute angle J-configuration of the takeoff of the celiac axis, with stenosis at its origin and focal post-stenotic dilatation, confirming the diagnosis of CACS. CACS is an elusive diagnosis that should be considered in patients where other causes of abdominal pain and weight loss have been ruled out. The disease can present with the classic triad of post-prandial abdominal pain, weight loss, and an abdominal bruit. Imaging modalities including mesenteric vascular duplex, computed tomography abdominal angiography, magnetic resonance angiography and celiac artery angiography can help make the diagnosis. Treatment involves surgical decompression via division of the median arcuate ligament, with most patients experiencing significant and long-lasting relief from their symptoms.
PubMed: 34262830
DOI: 10.7759/cureus.16175 -
Revista Espanola de Enfermedades... Nov 2022A 74-year-old patient presented to our emergency department with melena for 24 hours, associated with postprandial abdominal pain, predominantly in the epigastrium, for...
A 74-year-old patient presented to our emergency department with melena for 24 hours, associated with postprandial abdominal pain, predominantly in the epigastrium, for one month. Urgent gastroscopy showed a pale mucosa with loss of vascular pattern in the gastric antrum, as well as several superficial ulcers, Forrest III, at that level and in the duodenal bulb highly suggestive of ischaemia. An abdominal computed tomography angiography (CTA) revealed a filiform celiac trunk, with calcified atherosclerotic plaques in the ostium, superior mesenteric artery and both renal arteries, with absence of enhancement in a large part of the intestinal wall, suggestive of ischaemia. An exploratory laparoscopy confirmed patchy ischaemia of the small intestine, as well as of the cecum, ascending colon, and portion of the transversus. Atherosclerosis is the most common cause of occlusive chronic mesenteric ischaemia. The 'classic triad' consisting of postprandial pain, weight loss and abdominal bruit, is found in only a minority of patients, with a significant percentage of paucisymptomatic patients due to abundant colateral circulation, making diagnosis difficult. The endoscopic finding of edema, erythema or signs of mucosal atrophy, as well as gastric or duodenal ulcers, not justified by other causes can guide us in the diagnosis. However, more distal sections of the digestive tract which are not accessible with the conventional endoscope can often be affected, and the absence of these findings, does not exclude the diagnosis. Abdominal CTA is the gold-standard imaging test. Early diagnosis of mesenteric ischaemia is a challenge in clinical practice. Recognizing its endoscopic signs can facilitate its early diagnosis and treatment.
Topics: Humans; Aged; Mesenteric Ischemia; Celiac Artery; Mesenteric Artery, Superior; Ischemia; Angiography; Acute Disease; Abdominal Pain
PubMed: 35545898
DOI: 10.17235/reed.2022.8857/2022 -
Journal of Investigative Medicine High... 2017Median arcuate ligament syndrome is a rare disorder that is clinically characterized by the triad of postprandial abdominal pain, weight loss, and often an abdominal...
Median arcuate ligament syndrome is a rare disorder that is clinically characterized by the triad of postprandial abdominal pain, weight loss, and often an abdominal bruit due to compression of the celiac artery by the median arcuate ligament. Given the nonspecific symptoms, this is a rare and difficult diagnosis to obtain. We present a patient with nonspecific abdominal pain in whom etiology was ultimately determined to be median arcuate ligament syndrome.
PubMed: 28904981
DOI: 10.1177/2324709617728750 -
Clinical and Experimental Rheumatology 2020Aneurysm formation can cause life-threatening complications in Takayasu's arteritis (TAK). The objective of this study was to evaluate the demographic, clinical and...
OBJECTIVES
Aneurysm formation can cause life-threatening complications in Takayasu's arteritis (TAK). The objective of this study was to evaluate the demographic, clinical and angiographic features, and outcomes of aneurysm secondary to TAK in Chinese patients.
METHODS
The medical charts of patients diagnosed with TAK in Changhai Hospital between 2001 and 2017 were retrospectively reviewed.
RESULTS
Aneurysms were identified in 66 (16.6%) of 397 patients with TAK. The mean age at onset was 30.4±11.5 years, with a male:female ratio of 1:2.7. Patients with aneurysm had a higher proportion of male (p<0.01), higher incidences of bruit, chest tightness and aortic regurgitation (all p<0.001), and a lower incidence of visual disturbances (p<0.01) as compared with patients without aneurysm. The prevalence of elevated ESR and CRP and ITAS2010 score were higher in patients with than without aneurysm (all p<0.01). Angiographic classification showed that type V (30.3%) was the most frequent pattern in patients with aneurysm though Type I was dominant in patients without aneurysm. Multiple aneurysms were found in 30.3% of patients and the most common site of aneurysms was abdominal aorta (22.1%). Glucocorticoids were prescribed in 86.4% of patients with aneurysm, and surgical procedures were performed in 80.3%. Five of 52 patients died during the median 3-year follow-up period.
CONCLUSIONS
These findings could provide useful information on the demographical, clinical and angiographic features of TAK patients with aneurysm. Aneurysm formation in TAK may be associated with male gender and active vascular inflammation.
Topics: Adult; Aneurysm; Angiography; Aorta, Abdominal; Asian People; China; Female; Humans; Male; Retrospective Studies; Takayasu Arteritis; Young Adult
PubMed: 31820727
DOI: No ID Found -
Medicine Mar 2017A novel case is reported of upper gastrointestinal (UGI) bleeding from sinistral portal hypertension, caused by a left gastric artery (LGA) pseudoaneurysm (PA)... (Review)
Review
Case report and systematic literature review of a novel etiology of sinistral portal hypertension presenting with UGI bleeding: Left gastric artery pseudoaneurysm compressing the splenic vein treated by embolization of the pseudoaneurysm.
INTRODUCTION
A novel case is reported of upper gastrointestinal (UGI) bleeding from sinistral portal hypertension, caused by a left gastric artery (LGA) pseudoaneurysm (PA) compressing the splenic vein (SV) that was successfully treated with PA embolization.
CASE REPORT
A 41-year-old man with previous medical history of recurrent, alcoholic pancreatitis presented with several episodes of hematemesis and abdominal pain for 48 hours. Physical examination revealed a soft abdomen, with no abdominal bruit, no pulsatile abdominal mass, and no stigmata of chronic liver disease. The hemoglobin declined acutely from 12.3 to 9.3 g/dL. Biochemical parameters of liver function and routine coagulation profile were entirely within normal limits. Abdominal CT revealed a 5-cm-wide peripancreatic mass compressing the stomach and constricting the SV. Esophagogastroduodenoscopy showed blood oozing from portal hypertensive gastropathy, small nonbleeding gastric cardial and fundal varices, gastric compression from the extrinsic mass, and no esophageal varices. MRCP and angiography showed that the mass was vascular, arose from the LGA, compressed the mid SV without SV thrombosis, and caused sinistral portal hypertension. At angiography, the PA was angioembolized and occluded. The patient has been asymptomatic with no further bleeding and a stable hemoglobin level during 8 weeks of follow-up.
DISCUSSION
Literature review of the 14 reported cases of LGA PA revealed that this report of acute UGI bleeding from sinistral portal hypertension from a LGA PA constricting the SV is novel; one previously reported patient had severe anemia without acute UGI bleeding associated with sinistral portal hypertension from a LGA PA.
CONCLUSION
A patient presented with UGI bleeding from sinistral portal hypertension from a LGA PA compressing the SV that was treated by angiographic obliteration of the PA which relieved the SV compression and arrested the UGI bleeding. Primary therapy for this syndrome should be addressed to obliterate the PA and not the secondarily constricted SV.
Topics: Adult; Aneurysm, False; Embolization, Therapeutic; Hematemesis; Humans; Hypertension, Portal; Male; Splenic Vein; Tomography, X-Ray Computed
PubMed: 28353569
DOI: 10.1097/MD.0000000000006413 -
Canadian Journal of Kidney Health and... 2022Transplant renal artery stenosis (TRAS) is a well-recognized and potentially reversible cause of resistant hypertension post transplantation and can affect 1% to 23% of...
RATIONALE
Transplant renal artery stenosis (TRAS) is a well-recognized and potentially reversible cause of resistant hypertension post transplantation and can affect 1% to 23% of recipients. Stenosis of the iliac segment proximal to the transplant renal artery (proximal TRAS) causing dysfunction of the transplanted kidney is less common with reported incidence of 2% to 3%. Presentation typically occurs between 3 months and 2 years post transplant but may happen at any time. Noninvasive investigations such as Doppler ultrasound, computed tomography (CT) angiogram, and magnetic resonance angiogram are useful in initial evaluation, but definitive diagnosis of hemodynamically significant stenosis often requires formal angiogram. Transplant renal artery stenosis should be suspected in any kidney transplant recipient with worsening hypertension and/or deterioration in kidney function which is otherwise unexplained. We present the case of a kidney transplant recipient with resistant hypertension and impaired graft function, secondary to severe impairment of graft blood flow from proximal iliac system occlusion.
PRESENTING CONCERNS OF THE PATIENT
A 74-year-old female 15 years post live donor kidney transplant presented with graft dysfunction (serum Cr 229 μmol/L) and resistant hypertension, requiring use of 8 antihypertensive medications. On physical examination, blood pressure was 160/92 mm Hg with no tenderness over the renal graft in the right lower abdominal quadrant and no audible bruit in kidney allograft area.
DIAGNOSIS
Transplant Doppler ultrasound showed reversal of flow in the right external iliac artery suggestive of ipsilateral proximal iliac occlusion. Pre-procedure CT demonstrated severe atherosclerotic burden within the aorta and bilateral iliac systems. The anastomosed right renal artery appeared patent.
INTERVENTIONS
Conventional angiogram showed occlusion of the right common and proximal external iliac arteries with retrograde perfusion of the transplant kidney via the contralateral left iliac system and aorta. Subintimal recanalization of the right iliac system was performed with angioplasty and kissing stent placement at the aortic bifurcation with stents extending into the proximal right external iliac artery. Post deployment angiogram demonstrated renewed patency of the right iliac system, with restoration of antegrade perfusion to the transplant kidney.
OUTCOMES
The patient's blood pressure decreased significantly after the procedure, with improvement in graft function. After 6 months, the patient continued to have optimally controlled blood pressure (on 3 medications) and stable graft function (serum Cr 74 μmol/L).
TEACHING POINTS
Our case describes proximal TRAS and the contribution of renal hypoperfusion to hypertension and impaired graft function, with the potential for reversibility.
PubMed: 36160314
DOI: 10.1177/20543581221119896 -
Arthritis Research & Therapy Jan 2019Childhood Takayasu's arteritis (c-TA) is scarcely reported but is characterized by devastating morbidity and mortality. This study aims to investigate the clinical... (Clinical Trial)
Clinical Trial Observational Study
BACKGROUND
Childhood Takayasu's arteritis (c-TA) is scarcely reported but is characterized by devastating morbidity and mortality. This study aims to investigate the clinical course of c-TA and prognostic factors associated with rehospitalization and events including vascular complications, flares, and death.
METHODS
An ambispective study of 101 c-TA patients satisfying the American College of Rheumatology (ACR) criteria and/or the European League against Rheumatism (EULAR)/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS) criteria was conducted from January 2002 to December 2017. Data on demographic, clinical, laboratory, imaging, and therapeutic features were collected. Event-free survival, complication-free survival, flare-free survival, rehospitalization-free survival, and associated prognostic factors were assessed by Kaplan-Meier survival curve and propensity score analysis.
RESULTS
The median age at c-TA onset was 14 (interquartile range (IQR) 12-16) years and 76.2% were female. Hypertension (70.3%), blood pressure discrepancy (55.4%), bruits (51.5%), and pulse deficits (37.6%) were core presentations. Major vascular involvement included the renal artery (62.4%), abdominal aorta (42.6%), subclavian artery (43.6%), and carotid artery (42.6%). Glucocorticoids (78.2%), antihypertensive drugs (72.3%), antiplatelet agents (72.3%), and revascularization (57.4%) were made up the majority administered. At a median 2.4 (IQR 0.7-6.1) years of follow-up, events, rehospitalization, vascular complications, flares and death were observed in 44.6%, 37.6%, 44.6%, 26.7%, and 3%, respectively. The 5-year event-free survival, rehospitalization-free survival, vascular complication-free survival, and flare-free survival were 42.8%, 55.8%, 45.9%, and 62.3%, respectively. Body mass index (BMI) (hazard ratio (HR) = 0.49, 95% confidence interval (CI) 0.30-0.81, p = 0.005), stroke (HR = 7.37, 95% CI 2.35-23.1, p = 0.001), and revascularization (HR = 0.51, 95% CI 0.27-0.94, p = 0.032) were independent prognostic predictors of events. Predictors for rehospitalization include age at admission (HR = 0.81, 95% CI 0.69-0.94, p = 0.006), renal artery involvement (HR = 0.49, 95% CI 0.25-0.96, p = 0.037), and elevated C-reactive protein (CRP; HR = 2.50, 95% CI 1.24-5.00, p = 0.01). BMI level (p = 0.024) and renal artery involvement (p = 0.015) were also associated with vascular complications, while revascularization (p = 0.002) independently correlated with re-flares.
CONCLUSIONS
This large ambispective study of c-TA revealed an early 3% mortality at the first year and around 50% morbidity within 5 years after diagnosis. Hypertension, renal artery involvement, and revascularization based on anti-inflammation, antihypertension, and antiplatelet medications dominated c-TA with indications for optimistic prognosis. Patients with initial lower BMI level, a younger age at admission, stroke, and elevated CRP have a high risk of poor outcomes, requiring close c-TA monitoring and more aggressive management.
TRIAL REGISTRATION
NCT03199183 , unique protocol ID: 2016-ZX43. June 26, 2017.
Topics: Adolescent; Adult; Antihypertensive Agents; Child; Disease Progression; Female; Glucocorticoids; Humans; Hypertension; Kaplan-Meier Estimate; Male; Patient Readmission; Platelet Aggregation Inhibitors; Prognosis; Retrospective Studies; Takayasu Arteritis
PubMed: 30670069
DOI: 10.1186/s13075-018-1790-x -
Korean Journal of Pediatrics Nov 2016Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the...
Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.
PubMed: 28018454
DOI: 10.3345/kjp.2016.59.11.S84 -
Annals of the Royal College of Surgeons... Sep 2015Median arcuate ligament syndrome (MALS) is a rare disorder due to coeliac trunk compression by the median arcuate ligament, resulting in coeliac artery stenosis... (Review)
Review
Median arcuate ligament syndrome (MALS) is a rare disorder due to coeliac trunk compression by the median arcuate ligament, resulting in coeliac artery stenosis characterised by chronic, recurrent abdominal pain. Patients with MALS are often middle-aged females presenting with a triad of postprandial epigastric pain, weight loss and abdominal bruit. It is a diagnosis of exclusion and confirmed by computed tomography or magnetic resonance imaging. Laparoscopic or open surgical decompression are the only treatment options in MALS. We present two cases of MALS treated by laparoscopic decompression as well as a literature review on this treatment.
Topics: Adult; Celiac Artery; Constriction, Pathologic; Decompression, Surgical; Female; Humans; Laparoscopy; Median Arcuate Ligament Syndrome; Tomography, X-Ray Computed
PubMed: 26320770
DOI: 10.1308/rcsann.2015.0025 -
International Journal of Applied &... 2021Dandy-Walker Syndrome (D-WS) is a rare disorder with an incidence of 1%-2% of all central nervous system anomalies. The diagnosis can be challenging, especially in the...
Dandy-Walker Syndrome (D-WS) is a rare disorder with an incidence of 1%-2% of all central nervous system anomalies. The diagnosis can be challenging, especially in the prenatal period. Here, we present an extremely rare case of D-WS associated with bilateral congenital cataracts. A 36 weeks and 6 days old male baby presented with a Dandy-Walker variant associated with bilateral congenital cataract. Ophthalmological examination revealed microphthalmos and congenital cataracts present in both eyes with sclerocornea, iris coloboma, and zone 3 retinopathy of prematurity involving only the right eye. However, the right eye was salvageable. Skull transillumination was negative with no cranial bruit. He was admitted to the neonatal intensive care unit with breathing difficulties, maintained SpO with oxygen through prongs, and noninvasive continuous positive airway pressure for 7 days. He had two episodes of hypoglycemia with hypothermia. There was no significant finding in sepsis evaluation. The abdominal ultrasonography was normal. Echocardiogram was suggestive of patent foramen ovale. Mother's torch panel tested positive for cytomegalovirus immunoglobulin G antibodies. Magnetic resonance imaging brain suggested variant D-WS with dilation of cerebellar fossa and occipital lateral ventricle horn and lack of usual corpus callus structure. Intravenous antibiotics cefotaxime and amikacin were administered along with fluid supplementation. He was shifted to mother feed. The neonate was referred to the pediatric surgery department for further management.
PubMed: 34912695
DOI: 10.4103/ijabmr.ijabmr_343_21