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Journal of the American College of... Dec 1988Congenital arteriovenous fistulas presenting in the newborn period pose difficult diagnostic problems and simulate structural heart disease. Angiocardiography, when...
Congenital arteriovenous fistulas presenting in the newborn period pose difficult diagnostic problems and simulate structural heart disease. Angiocardiography, when performed, demonstrates enlarged brachiocephalic vessels and rapid cerebral venous return. The value of echocardiographic imaging and measurement of the aortic arch and brachiocephalic vessels, and evaluation of the Doppler flow profile in these vessels as a means of making a rapid diagnosis of cerebral or thoracic arteriovenous fistula, was therefore assessed in 10 infants with these diagnoses seen over a 4 year period (1983 to 1987). Twenty-nine infants (median age 6 weeks) undergoing two-dimensional echocardiography but with no significant lesions were prospectively selected as controls. Nine of the 10 patients had congestive heart failure at presentation (mean age 2 days). A cranial bruit was heard in three and arteriovenous fistula was suspected in five patients. Aortic arch segments and brachiocephalic vessel dimensions expressed as ratios of the abdominal aorta showed significantly larger values in patients for the ascending aorta (p = 0.01), innominate artery (p less than 0.001), right and left subclavian arteries (p less than 0.001) and left common carotid artery (p less than 0.05). The thoracic descending aorta was, however, significantly smaller in patients (p less than 0.002). Retrograde diastolic Doppler flow in the descending aorta proximal to the ductus arteriosus and anterograde diastolic flow with a mean spectral flow-time integral 27% of systolic were present in patients only, whereas Doppler diastolic flow in brachiocephalic vessels, present in 5 of 29 control infants, was less than 15% of systolic flow and not accompanied by dilation of these vessels.(ABSTRACT TRUNCATED AT 250 WORDS)
Topics: Aorta, Thoracic; Arteriovenous Malformations; Brachiocephalic Trunk; Cerebral Angiography; Diastole; Echocardiography; Humans; Infant; Infant, Newborn; Intracranial Arteriovenous Malformations; Ultrasonography
PubMed: 3057034
DOI: 10.1016/s0735-1097(88)80021-4 -
Archives of Rheumatology Jun 2020Takayasu arteritis (TA) is classified as a large vessel vasculitis of predominantly aorta and its main branches, resulting in fibrosis and stenosis. Only a minority of...
Pre-Pulseless Takayasu Arteritis in a Child Represented With Prolonged Fever of Unknown Origin and Successful Management With Concomitant Mycophenolate Mofetil and Infliximab.
Takayasu arteritis (TA) is classified as a large vessel vasculitis of predominantly aorta and its main branches, resulting in fibrosis and stenosis. Only a minority of TA patients are diagnosed in pre-stenosis phase when constitutional symptoms including fever, arthralgia, weight loss, headache, abdominal pain, and elevated acute phase reactants are dominant insidious characteristics. In this article, we present a 12-year-old female patient, who was referred to our department with a one-year history of low-grade fever, fatigue, and myalgia. Physical examination did not reveal pulse and blood pressure discrepancies between any extremities. Acute phase reactants were markedly elevated, and autoantibodies were negative. Magnetic resonance angiography (MRA) findings have confirmed TA diagnosis with prominent vessel wall thickening in the ascendant and abdominal aorta, focal ectasias and a thoracoabdominal fusiform aneurysm. As methotrexate and methylprednisolone treatment during three months was unsuccessful, infliximab was induced. During the next 12 months, patient had clinical improvement, but worsening of MRA findings and new onset of carotidynia forced us to switch methotrexate to mycophenolate mofetil. Six months later, laboratory and radiological remission were achieved. In conclusion, we report a challenge to diagnose pre-pulseless childhood-TA (c-TA) in the state of prolonged fever with no signs of vascular stenosis, systemic hypertension, pulses and blood pressure discrepancies, bruits and claudication. Therefore, we wish to discourse the importance of early diagnosis of TA since, to our knowledge, there are no studies investigating treatment success only in the early phases of c-TA.
PubMed: 32851379
DOI: 10.46497/ArchRheumatol.2020.7599 -
Canadian Medical Association Journal May 1982
Topics: Adult; Contraceptives, Oral; Female; Hemangioma, Cavernous; Humans; Hyperplasia; Liver; Liver Neoplasms
PubMed: 7074431
DOI: No ID Found -
Cureus Jun 2019Median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome (CACS), is a rare clinical entity that is characterized by extrinsic...
Median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome (CACS), is a rare clinical entity that is characterized by extrinsic compression of the celiac artery by the median arcuate ligament of the diaphragm. It mostly presents as a triad of post-prandial abdominal pain, weight loss, and bruit in the epigastrium. Given its variable and atypical presentation, it is a diagnostic challenge for physicians. MALS is supposed to be a diagnosis of exclusion and, thus, many investigations need to be done before coining it as a definitive diagnosis. Herein, we present a case of a 17-year-old female patient, a known case of common variable immunodeficiency (CVID) who presented to our setup with progressive, excruciating epigastric pain and bilious vomiting after undergoing excision of CVID-associated gastric adenocarcinoma (CAGA). Multiple sets of tests were conducted to rule out possible cardiopulmonary, musculoskeletal, and abdominal etiology. The diagnosis was made on the basis of findings depicted on three-dimensional computed tomographic angiography (3D-CTA) and ultrasound Doppler studies of the celiac artery. She underwent laparoscopic dissection of the median arcuate ligament with a celiac plexus block, which yielded immediate relief in symptomatology and was later followed up with duplex scans and showed complete remission of symptoms.
PubMed: 31417810
DOI: 10.7759/cureus.4865 -
Cureus Nov 2016Takayasu arteritis (TA) is an idiopathic chronic inflammatory vasculitis of the aorta and its main branches, which if not treated can lead to severe vascular damage and...
Takayasu arteritis (TA) is an idiopathic chronic inflammatory vasculitis of the aorta and its main branches, which if not treated can lead to severe vascular damage and fatal vascular events. Glucocorticoids (GCs) are the mainstay of the therapy of TA but a significant proportion of patients tend to experience flare-ups when their GCs are tapered. We report a case of a 42-year-old female with TA, diagnosed according to the 1990 American College of Rheumatology Criteria for TA. Cardiovascular assessment showed normal carotid upstrokes with bilateral carotid bruits and soft right and left subclavian bruits with weak peripheral pulses. A computed tomography (CT) aortogram of the chest showed severe stenosis of bilateral subclavian arteries and mild stenosis of right and left common carotid arteries at the origin. A CT aortogram of the abdomen showed an occluded left renal artery, a very small left kidney, and mild narrowing of the abdominal aorta below the level of renal arteries. She was initially managed with GCs along with immunosuppressive therapy including methotrexate, azathioprine, and cyclophosphamide, but her disease remained active. She was then sequentially treated with inhibitor etanercept (ETN), inhibitor tocilizumab (TCZ) and monoclonal anti-CD20 antibody rituximab (RTX), and in spite of aggressive biologic therapy she continued to have active disease. To the best of our knowledge, this is the first case of refractory TA treated sequentially with three different biologic drugs.
PubMed: 27994990
DOI: 10.7759/cureus.872 -
BMJ Case Reports Jan 2016A 33-year-old Caucasian man was admitted to the hospital with chest pain and hypertensive urgency. Physical examination revealed widespread arterial bruits and marked...
A 33-year-old Caucasian man was admitted to the hospital with chest pain and hypertensive urgency. Physical examination revealed widespread arterial bruits and marked difference in blood pressure between the upper limbs. Vascular imaging showed widespread narrowing in multiple vascular territories. He met the established American College of Rheumatology criteria for Takayasu's arteritis. His resistant hypertension was considered to be a consequence of bilateral renal artery stenosis and he subsequently underwent sequential stenting of his renal arteries leading to improvement in blood pressure and reduction in the number of antihypertensive medications. Subsequent imaging revealed progression of aortic thrombus in the setting of an elevated erythrocyte sedimentation rate, and persistently elevated antiphospholipid antibodies fulfilling diagnostic criteria for primary antiphospholipid syndrome, requiring initiation of immunosuppression and anticoagulation.
Topics: Adult; Antiphospholipid Syndrome; Aorta, Abdominal; Humans; Hypertension; Immunosuppressive Agents; Male; Renal Artery Obstruction; Stents; Takayasu Arteritis; Tomography, X-Ray Computed
PubMed: 26783006
DOI: 10.1136/bcr-2015-211752 -
Cureus Jun 2020Median arcuate ligament (MAL) syndrome (MALS) is a rare clinical entity characterized by chronic abdominal pain resulting from compression of the celiac artery by the...
Median arcuate ligament (MAL) syndrome (MALS) is a rare clinical entity characterized by chronic abdominal pain resulting from compression of the celiac artery by the MAL. We present a case of MALS with imaging evidence of anterior compression of the celiac artery on expiration, which was relieved on inspiration. A 33-year-old woman presented with intermittent upper abdominal pain since three months. The pain was associated with nausea, abdominal bloating and diarrhea. Physical examination revealed a palpable abdominal aorta with no bruit. Abdominal sonogram, upper and lower gastrointestinal endoscopies, celiac disease screening, clostridium difficile toxin assays and hepatobiliary iminodiacetic acid scan were all normal. Computerized tomography angiogram (CTA) revealed subtle narrowing at the origin of the celiac artery without any atherosclerosis or calcification. Lateral aortic angiography showed anterior impression on the celiac artery at its origin on expiration. The constriction was relieved on inspiratory film - findings most consistent with MALS. MALS is rare, typically presenting with non-specific symptoms including nausea, vomiting, chronic post-prandial abdominal pain or unintentional weight loss. Angiography with respiratory maneuvers remains the diagnostic standard. However, non-invasive vascular imaging during both phases of respiration can be considered as an initial diagnostic test. The primary goal of therapy is celiac artery decompression with the additional objective of neuronolysis of the celiac ganglion. Our case highlights that MALS should be considered as a differential diagnosis in chronic, recurrent abdominal pain, particularly with an unrevealing initial evaluation. Evidence of celiac artery compression on vascular imaging, with characteristic respiratory variation, is highly suggestive of MALS.
PubMed: 32617247
DOI: 10.7759/cureus.8865 -
BMJ Case Reports Apr 2017A 73-year-old woman presented with a 6-hour history of sudden onset lower abdominal pain. Her comorbidities included chronic obstructive pulmonary disease and...
A 73-year-old woman presented with a 6-hour history of sudden onset lower abdominal pain. Her comorbidities included chronic obstructive pulmonary disease and hypertension. She was under surveillance for a known thoracoabdominal aneurysm. On presentation, she was hypotensive with a systolic blood pressure of 50 mm Hg and a pulse of 60 beats per minute. On examination, she had a pulsatile mass with bruit in her right lower abdomen. Pedal pulses were palpable in both feet. Blood gas analysis revealed a metabolic acidosis with a pH of 7.21 and a lactate of 7.1. Haemoglobin remained stable between 90-100 g/dL. Her other routine blood tests were unremarkable, and blood cultures were negative. Imaging showed a ruptured right common iliac artery aneurysm into the right common iliac vein with secondary arteriovenous fistula communication. Surgical intervention was discussed with the patient but due to her frailty, it was deemed not in the patient's best interests.
Topics: Aged; Aneurysm, Ruptured; Arteriovenous Fistula; Disease Management; Female; Humans; Iliac Aneurysm; Iliac Artery; Rupture, Spontaneous; Tomography, X-Ray Computed
PubMed: 28446483
DOI: 10.1136/bcr-2016-218863 -
North American Journal of Medical... Jul 2011Ganglioneuromas are benign tumors of the sympathetic nervous system that rarely arise in the adrenal gland. Majority of cases are detected incidentally since they are...
CONTEXT
Ganglioneuromas are benign tumors of the sympathetic nervous system that rarely arise in the adrenal gland. Majority of cases are detected incidentally since they are usually asymptomatic. Up to the current era of laparoscopic adrenal mass excision, this unusual entity has not been adequately reported in the surgical literature.
CASE REPORT
A 51 year old male with history of hypertension was found to have abdominal bruit during a regular physical examination. A 4 cm right adrenal mass with upper pole calcification and a 6 cm retro-pancreatic mass were subsequently found on a computed tomography scan. Endoscopic ultrasound-guided needle biopsy was indeterminate. Preoperative endocrine evaluation showed mildly elevated vanillyl mandelic acid with normal 24-hour cathecolamine, metanephrine and cortisol levels. Histopathologic examination after an uneventful laparoscopic excision was consistent with ganglioneuroma.
CONCLUSIONS
Ganglioneuroma occurs rarely in adrenal gland and preoperative diagnosis is difficult since symptoms are usually nonspecific. Due to widespread utilization of abdominal imaging, however, it should be included in differential diagnosis of adrenal or retroperitoneal mass. Histopathologic examination is currently the mainstay of diagnosis.
PubMed: 22540109
DOI: 10.4297/najms.2011.3336 -
Cureus Jul 2017A spontaneous aortocaval fistula is a rare complication of abdominal aortic aneurysms. In 50 percent of the patients, it presents with the classic signs of a pulsatile...
A spontaneous aortocaval fistula is a rare complication of abdominal aortic aneurysms. In 50 percent of the patients, it presents with the classic signs of a pulsatile abdominal mass, continuous bruit, and low back pain. A high degree of clinical suspicion and a well-performed physical examination are important for its timely diagnosis.
PubMed: 28929042
DOI: 10.7759/cureus.1459