-
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 -
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 -
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 -
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 -
Journal of Medical Case Reports Oct 2021Thyroid storm is an endocrine emergency and life-threatening condition discovered in 1926. There is no specific laboratory parameter that can differentiate or...
BACKGROUND
Thyroid storm is an endocrine emergency and life-threatening condition discovered in 1926. There is no specific laboratory parameter that can differentiate or distinguish between thyroid storm and primary hyperthyroidism. Diagnosis is made on a clinical scoring system, including the Burch-Wartofsky point scale and Japanese Thyroid Association scoring system. The management is early diagnosis, immediate initiation of anti-thyroid medications, intensive care monitoring, and prevention of multiorgan failure.
CASE PRESENTATION
A 30-year-old Pakistani female presented with complaint of headache, vomiting, and generalized weakness for 3 weeks. She had an episode of seizure-like activity at home, and so was rushed to the emergency department. A detailed thyroid examination revealed a soft, nontender gland with no enlargement or bruit and no exophthalmos. Her thyroid-stimulating hormone was extremely low, with high free triiodothyronine and thyroxine. Thyroglobulin was 425 ng/ml (normal reference range ≤ 55 ng/ml), and thyroid-stimulating hormone receptor antibody was 0.87 IU/L (normal reference range 0-1.75 IU/L). She had high levels of beta-human chorionic gonadotropin hormone on initial presentation. Transvaginal ultrasound showed no intrauterine pregnancy, but an echogenic focus was found adherent to the right ovary with no vascularity. With the chief complaint of headache, she underwent magnetic resonance imaging of the brain that showed multiple scattered hemorrhagic lesions in supratentorial and infratentorial brain parenchyma that were highly suspicious for metastases. Computed tomography scan of the chest, abdomen, and pelvis revealed multiple neoplastic lesions in the lung, liver, spleen, and kidneys. A Tru-Cut liver biopsy showed linear cores of liver tissue with metastatic carcinoma with morphological features consistent with choriocarcinoma. Our patient scored 65 on the Burch-Wartofsky point scale. As per the Japanese Thyroid Association scoring system, our patient met the criteria for a "definite thyroid storm." She had initiated propranolol to achieve adequate control of her heart rate and dexamethasone. Carbimazole was started to control her thyroid function. Her thyroid function after 2 weeks of treatment showed significant improvement. Methotrexate and etoposide were given for choriocarcinoma. She made a good recovery and was discharged home. She will undergo rehabilitation along with ongoing chemotherapy (methotrexate and etoposide weekly till beta-human chorionic gonadotropin levels normalize). Unless her source of beta-human chorionic gonadotropin is carefully under control, she will continue to take anti-thyroid medications.
CONCLUSION
Choriocarcinoma is not only associated with hyperthyroidism but can induce thyroid storm. Beta-human chorionic gonadotropin is directly associated with promoting thyroid dysfunction. Patients with gestational trophoblastic disease should be under close surveillance to prevent thyroid storms.
Topics: Adult; Choriocarcinoma; Chorionic Gonadotropin, beta Subunit, Human; Female; Humans; Pregnancy; Thyroid Crisis; Thyroid Function Tests
PubMed: 34686220
DOI: 10.1186/s13256-021-03123-7 -
European Journal of Case Reports in... 2023Unstable angina, characteristic of coronary artery disease, is caused by in-situ clot formation complicating ruptured atheromatous plaque. Abdominal angina, however,...
UNLABELLED
Unstable angina, characteristic of coronary artery disease, is caused by in-situ clot formation complicating ruptured atheromatous plaque. Abdominal angina, however, usually reflects chronic mesenteric ischaemia, caused by multi-vessel stable plaques involving mesenteric arteries. Herein, we describe a patient with new-onset abdominal pain caused by a ruptured atheromatous plaque at the superior mesenteric root. The diagnosis was based on an evident reversible epigastric bruit and high-degree eccentric stenosis caused by a non-calcified atheroma. Symptoms and bruit resolved within 3 weeks on aspirin and statins with regression of the stenotic lesion. Although the condition is likely common, this is the first clear-cut report compatible with 'unstable' abdominal angina, resolved by conservative treatment.
LEARNING POINTS
Resembling unstable angina pectoris, ruptured atheromatous plaque in mesenteric vessels can develop, clinically manifested by new-onset abdominal angina.This condition may be reversible under treatment with antiplatelet medications and statins.Searching for abdominal bruit is invaluable in the assessment of unexplained abdominal pain.
PubMed: 37051483
DOI: 10.12890/2023_003766 -
Jornal Vascular Brasileiro 2023Ischemic gastritis is a rare illness caused by localized or systemic vascular insufficiency. This condition is rarely seen in medical practice due to the vast arterial...
Ischemic gastritis is a rare illness caused by localized or systemic vascular insufficiency. This condition is rarely seen in medical practice due to the vast arterial collateral blood supply to the stomach through the celiac trunk and superior mesenteric artery and also because other etiologies are much more frequent. The classic presentation of chronic ischemia is comprises the triad of postprandial pain, weight loss, and abdominal bruit. Intervention is indicated in symptomatic patients and endovascular treatment is an alternative to surgery in patients with high comorbidity that offers good results. We report a case of a 71-year-old female patient with severe ischemic gastritis with ulcers and bleeding caused by chronic mesenteric ischemia with occlusion of the celiac trunk and inferior mesenteric artery and critical stenosis of the superior mesenteric artery. The diagnosis was confirmed by imaging, and the patient underwent endovascular treatment. This is a rare condition that is difficult to diagnose and treat and a multidisciplinary team is needed for proper management.
PubMed: 37790892
DOI: 10.1590/1677-5449.202300022 -
Annals of Medicine and Surgery (2012) Oct 2020Dunbar syndrome or median arcuate ligament syndrome is a rare disorder. In this disorder, a malposition of the arcuate ligament compresses the celiac trunk and causes...
BACKGROUND
Dunbar syndrome or median arcuate ligament syndrome is a rare disorder. In this disorder, a malposition of the arcuate ligament compresses the celiac trunk and causes nonspecific symptoms including postprandial pain, abdominal bruit and weight loss. Surgical management is the primary treatment.Pancreatic neuroendocrine tumors (PNETS) are also rare. It comprises about 1-3% of pancreatic neoplasm. The patient could be symptomatic or asymptomatic depends on the tumor being functional or nonfunctional. In addition, surgical therapy is the choice.
CASE PRESENTATION
In this paper, we report a case of 28 old female patient complaining from a long term of recurrent abdominal pain that doesn't releive on any kind of treatment, the multislices computerised tomography scan showed compress of the median arcuate ligament with an incidental mass in the tail of pancreas turned out to be a pancreatic neuroendocrine tumor.
CONCLUSIONS
The Dunbar syndrome and the Pancreatic neuroendocrine tumors must be kept in mind of phyciciens while the differential diagnosis of any recurrent abdominal pain.
PubMed: 32953106
DOI: 10.1016/j.amsu.2020.08.038 -
Cureus Sep 2021Celiac artery compression syndrome (CACS), also known as median arcuate ligament syndrome, can sometimes represent a diagnostic challenge. Here, we present the case of a...
Celiac artery compression syndrome (CACS), also known as median arcuate ligament syndrome, can sometimes represent a diagnostic challenge. Here, we present the case of a 29-year-old man who presented with recurrent diabetic ketoacidosis (DKA), abdominal pain, and vomiting thought to be due to cyclical vomiting syndrome. However, the lack of a clear precipitant for DKA, the presence of chronic gastrointestinal symptoms, and a revealing physical examination of abdominal bruit led to clinical suspicion of CACS and its diagnosis after appropriate investigations. While angiography has traditionally been considered the gold standard diagnostic test, hemodynamic and geometric ultrasound criteria can, however, be diagnostic. The patient was managed by releasing the celiac artery through robotic surgery and serial monitoring as an outpatient revealed resolution of his symptoms and no further readmissions for DKA. This case highlights how a presumptive and erroneous diagnosis (cyclical vomiting syndrome) can misguide clinicians, especially when dealing with a rare diagnosis of exclusion.
PubMed: 34660028
DOI: 10.7759/cureus.17818