Our case is a 72-year-old female with Fibromuscular Dysplasia (FMD) presented to the Emergency Department (ED) with acute coronary syndrome (ACS), more specifically NSTEMI. In ED, troponin levels were elevated and non...Our case is a 72-year-old female with Fibromuscular Dysplasia (FMD) presented to the Emergency Department (ED) with acute coronary syndrome (ACS), more specifically NSTEMI. In ED, troponin levels were elevated and non-ST segment elevation myocardial infarction (NSTEMI) was detected on electrocardiogram (ECG). Computed tomography (CT) scan showed Internal Carotid artery (ICA) pseudoaneurysm. Cardiac catheterization (CATH) was performed and revealed occlusion of the Left anterior descending (LAD) artery. Spontaneous coronary artery dissection (SCAD) was suspected due to the history of FMD without a history of hypertension, smoking or substance use.展开更多
Extrarenal fibromuscular dysplasia causing gastro- intestinal bleeding without other manifestations and especially sparing renal vasculature is uncommon. The diagnosis of this entity is usually made by radiographic ap...Extrarenal fibromuscular dysplasia causing gastro- intestinal bleeding without other manifestations and especially sparing renal vasculature is uncommon. The diagnosis of this entity is usually made by radiographic appearance and the treatment is controversial. To our knowledge only seven cases of visceral fibromuscular dysplasia as a primary manifestation of the disease have been described, symptoms range from abdominal pain to gangrene. This is the first case of visceral fibromuscular dysplasia presenting with otherwise asymptomatic gastrointestinal bleeding, without bowel necrosis or ischemic changes. We provide a review of the literature.展开更多
Renal artery stenosis is a common cause of secondary hypertension and chronic kidney disease. We present here a case of fibromuscular dysplasia that was treated with surgical revascularization, resulting in recovery o...Renal artery stenosis is a common cause of secondary hypertension and chronic kidney disease. We present here a case of fibromuscular dysplasia that was treated with surgical revascularization, resulting in recovery of kidney function with eventual cessation of chronic dialysis. The case involves a 25-year-old female with coincidentally discovered hypertension, who underwent further investigations revealing a diagnosis of renal artery stenosis due to fibromuscular dysplasia. She subsequently developed two episodes of malignant hypertension, with flash pulmonary oedema and worsening renal failure that resulted in dialysis dependence. After evidence was obtained that the right kidney was still viable, a revascularization procedure was performed, improving blood pressure control and restoring kidney function, thereby allowing dialysis to be stopped. This case highlights the importance of evaluating patients with renal artery stenosis for revascularization before committing them to a life of chronic dialysis.展开更多
Fibromuscular dysplasia(FMD)is a rare disease with a typical clinical manifestations.This article will describe the clinical features of a FMD case with severe headache misdiagnosed as cerebral artery dissection(CAD)....Fibromuscular dysplasia(FMD)is a rare disease with a typical clinical manifestations.This article will describe the clinical features of a FMD case with severe headache misdiagnosed as cerebral artery dissection(CAD).Treatment remedies:we collected the clinical data of a patient with severe headache and performed brain MRI(magnetic resonance imaging)and cerebral arteriovenous MRA(magnetic resonance angiography).The preliminary diagnosis was considered as right vertebral artery dissection,and then cerebral DSA(digital subtraction angiography)was performed for further diagnosis.Post treatment evaluating:cerebral angiography showed that the vessel wall was stiff,with multiple(insect phagocytic)-like changes,and severe stenosis of local long segments.Some vessels showed“double lumen sign”,which was consistent with the performance of FMD.After anticoagulant treatment,the patient’s condition was improved.Conclusions:for severe headache,in addition to common diseases,the possibility of FMD should also be paid great attention.Early cerebrovascular screening is conducive to early diagnosis and timely treatment.展开更多
The vascular diseases including aneurysm, occlusion, and thromboses in the mesenteric lesions could cause severe symptoms and appropriate diagnosis and treatment are essential for managing patients. With the developme...The vascular diseases including aneurysm, occlusion, and thromboses in the mesenteric lesions could cause severe symptoms and appropriate diagnosis and treatment are essential for managing patients. With the development and improvement of imaging modalities, diagnostic frequency of these vascular diseases in abdominal lesions is increasing even with the small changes in the vasculatures. Among various vascular diseases, fibromuscular dysplasia(FMD) and segmental arterial mediolysis(SAM) are noninflammatory, nonatherosclerotic arterial diseases which need to be diagnosed urgently because these diseases could affect various organs and be lethal if the appropriate management is not provided. However, because FMD and SAM are rare, the cause, prevalence, clinical characteristics including the symptoms, findings in the imaging studies, pathological findings, management, and prognoses have not been systematically summarized. Therefore, there have been neither standard diagnostic criteria nor therapeutic methodologies established, to date. To systematically summarize the information and to compare these disease entities, we have summarized the characteristics of FMD and SAM in the gastroenterological regions by reviewing the cases reported thus far. The information summarized will be helpful for physicians treating these patients in an emergency care unit and for the differential diagnosis of other diseases showing severe abdominal pain.展开更多
Segmental arterial mediolysis (SAM) is an uncommon non-inflammatory iatrogenic arteriopathy caused by alpha-1 adrenergic agonists or Beta-2 agonists able to release norepinephrine from the peripheral nervous system. C...Segmental arterial mediolysis (SAM) is an uncommon non-inflammatory iatrogenic arteriopathy caused by alpha-1 adrenergic agonists or Beta-2 agonists able to release norepinephrine from the peripheral nervous system. Causative agents include adrenergic agonists used to control blood pressure, B-2 tocolytic agonists, and ractopamine used as a repartitioning agent in animal husbandry. The liberated norepinephrine both injures and stimulates a robust reparative response in the muscular arteries in the abdomen, brain base, and coronary arteries. This response may be augmented by endothelin-1 formed in the arterial adventitia. Three types of arterial lesions develop in the injurious stage: 1) apoptotic induced mediolysis, 2) separation of the outer media from the adventitia and 3) the formation of arterial gaps. The latter enlarge, particularly in elderly patients, to form gap-aneurysms complicated by dissections and dissecting an- eurysms that when ruptured cause the calamitous hemorrhages that clinically announce SAM. The other types of injury remain clinically silent but with repair develop sequelae and can metamorphose into fibromuscular dysplasia. The sequelae are mainly asymptomatic but may cause arterial stenosis and ischemic lesions. The definitive diagnosis of SAM re- quires histological conformation but misinterpreta- tion of smooth muscle vacuolar change has caused di- agnostic errors. Muscular artery cystic necrosis a newly named non-inflammatory muscular artery ar- teriopathy may be confused with SAM both clinically and pathologically. This arteriopathy represents the muscular artery equivalent of cystic media necrosis of the elastic arteries since it exhibits similar morphol- ogic features and can occur concomitantly with this entity. Adrenergic agents to counter hemorrhagic shock in SAM are contraindicated since they may intensify injury and create new lesions. The use of norepinehrine antagonists introduces a new, but as yet untested, treatment option for SAM.展开更多
Background: Fibromuscular dysplasia (FMD) is a rare idiopathic segmental non-arteriosclerotic and non-inflammatory arterial disease of small to medium-sized vessels. Although it is considered a benign entity, FMD can ...Background: Fibromuscular dysplasia (FMD) is a rare idiopathic segmental non-arteriosclerotic and non-inflammatory arterial disease of small to medium-sized vessels. Although it is considered a benign entity, FMD can lead to potentially severe cerebrovascular complications. We present an atypical clinical case of ischemic stroke (IS) caused by bilateral carotid dissection revealing multifocal FMD. Case Report: A 43-year-old right-handed female with no medical history suddenly developed somnolence with left-sided weakness. Clinical examination revealed left hemiplegia and left central facial paralysis with an NIHSS score: 22. Cerebral tomography (CT) with arterial angiogram revealed abilateralis of the middle cerebral arteries (MCA), a double dissection of the internal carotids (IC), aneurysmal ectasia of the left IC, and aneurysm of the M2 segment of the MCA. Brain magnetic resonance imaging (MRI) with arterial angiography confirmed the bilateral dissection. A cerebral angiography (CA) revealed a radiological pattern of multifocal FMD. The angiography of the aorta and its branches showed adiffuse dysplasia of the renal arteries. The patient was put under antiaggregants. The modified Rankin score (mRS) after three months was three. Conclusion: Cerebrovascular FMD has typical angiographic features, the “string of beads” appearance being the most common. Although the risk of a stroke in patients with cervical FMD is low, it can lead to a severe stroke and the long-term risks are unknown. Patient registries should be encouraged to better understand the mechanisms and evolutionary profile of FMD and alert clinicians to this potentially disabling condition.展开更多
Segmental arterial mediolysis (SAM), an uncommon arteriopathy putatively caused by norepinephrine released by alpha-1 adrenergic agonists or some Beta-2 agonists capable of releasing norepinephrine from the peripheral...Segmental arterial mediolysis (SAM), an uncommon arteriopathy putatively caused by norepinephrine released by alpha-1 adrenergic agonists or some Beta-2 agonists capable of releasing norepinephrine from the peripheral sympathetic nervous system potentially can present ischemic and organ injury symptoms caused by sequelae created in its reparative phase in lieu of catastrophic hemorrhages announced in its injurious phase. The case documents this presentation—the patient presenting renal infarcts and ischemic lesions causing abdominal angina, hypertension and a nephrectomy event developing 10 years after prolonged ritodrine treatment for premature labor. This agent may have directly caused SAM or sensitized the patient to agonists causing SAM encountered at a latter date. A variety of lesions derived from injurious phase arterial injuries characterize reparative phase SAM. All were encountered in a hilar branch of the resected renal artery. These included side-by-side sequela aneurysms grossly forming a large fusiform aneurysm, granulation tissue filling adventitial medial tear spaces in which a dissecting hematomas developed, medial muscle loss centered to the outer media repaired with fibrous tissue, arterial stenosis created by reparative intimal plaques, and arterial thrombo-embolism. These lesions were mirrored in accompanying radiologic studies. The accompanying renal vein exhibited changes consistent with repair of the spastic venous angiopathy that often accompanies abdominal SAM. This angiopathy, putatively induced by Endothelin-1, suggested that this agent played a role in the genesis of the arterial lesions. Angiographic resolution of non-treated sequelae occurred in 5 months either spontaneously or due to treatment with bosentem. Conclusions: The histologic and angiographic changes demonstrate that the clinical onset of reparative SAM may be significantly delayed to produce ischemic lesions, renal infarction and in this case report, medial fibromuscular dysplasia in the hilar branch of the renal artery.展开更多
A previously healthy 25-year-old man with no known risk factors was presented at the emergency room with a 3 h history of abdominal and loin pain.Physical examination and lab data showed no specific findings except te...A previously healthy 25-year-old man with no known risk factors was presented at the emergency room with a 3 h history of abdominal and loin pain.Physical examination and lab data showed no specific findings except tenderness,slight white cell count elevation and decreased haemoglobin level.The patient’s condition deteriorated over the following hours and he died despite resuscitation attempts.Autopsy revealed a 2.5-cm longitudinal tear in the intima of the right common iliac artery,which formed a thrombosed false lumen extending to the abdominal aorta proximally and to the left common iliac artery.Histopathologic examination revealed the characteristic changes of fibromuscular dysplasia(FMD).FMD involving the common iliac arteries is extremely rare;only six cases have been reported previously,and only two of those included forensic findings.The presented case is the first case of FMD with intimal tearing in the right common iliac artery,with propagation to the left common iliac artery and abdominal aorta.When a previously healthy young adult without hypertension or other risk factors presents with acute abdominal and loin pain,systemic vascular disease should be on the list of differential diagnoses.Careful and complete evaluation of multiple arteries can be critical.展开更多
Fibromuscular dysplasia(FMD)is a noninflammatory,nonatherosclerotic,and multifocal vascular disease,commonly involving the cerebral and renal arteries.Cerebrovascular stenosis and spontaneous dissection resulting from...Fibromuscular dysplasia(FMD)is a noninflammatory,nonatherosclerotic,and multifocal vascular disease,commonly involving the cerebral and renal arteries.Cerebrovascular stenosis and spontaneous dissection resulting from cerebrovascular FMD(cFMD)is one of the important causes of young stroke.Here,we reported the case of cFMD in a 28-year-old male patient with stroke.Digital subtraction angiogram demonstrated a dissecting aneurysm in the carotid artery and multiple stenoses in both vertebral arteries.Endovascular angioplasty with balloon predilation and stenting was successfully performed for the patient when the medical treatment was not adequate.The follow-up showed a remarkable improvement and no recurrence of stroke.展开更多
文摘Our case is a 72-year-old female with Fibromuscular Dysplasia (FMD) presented to the Emergency Department (ED) with acute coronary syndrome (ACS), more specifically NSTEMI. In ED, troponin levels were elevated and non-ST segment elevation myocardial infarction (NSTEMI) was detected on electrocardiogram (ECG). Computed tomography (CT) scan showed Internal Carotid artery (ICA) pseudoaneurysm. Cardiac catheterization (CATH) was performed and revealed occlusion of the Left anterior descending (LAD) artery. Spontaneous coronary artery dissection (SCAD) was suspected due to the history of FMD without a history of hypertension, smoking or substance use.
文摘Extrarenal fibromuscular dysplasia causing gastro- intestinal bleeding without other manifestations and especially sparing renal vasculature is uncommon. The diagnosis of this entity is usually made by radiographic appearance and the treatment is controversial. To our knowledge only seven cases of visceral fibromuscular dysplasia as a primary manifestation of the disease have been described, symptoms range from abdominal pain to gangrene. This is the first case of visceral fibromuscular dysplasia presenting with otherwise asymptomatic gastrointestinal bleeding, without bowel necrosis or ischemic changes. We provide a review of the literature.
文摘Renal artery stenosis is a common cause of secondary hypertension and chronic kidney disease. We present here a case of fibromuscular dysplasia that was treated with surgical revascularization, resulting in recovery of kidney function with eventual cessation of chronic dialysis. The case involves a 25-year-old female with coincidentally discovered hypertension, who underwent further investigations revealing a diagnosis of renal artery stenosis due to fibromuscular dysplasia. She subsequently developed two episodes of malignant hypertension, with flash pulmonary oedema and worsening renal failure that resulted in dialysis dependence. After evidence was obtained that the right kidney was still viable, a revascularization procedure was performed, improving blood pressure control and restoring kidney function, thereby allowing dialysis to be stopped. This case highlights the importance of evaluating patients with renal artery stenosis for revascularization before committing them to a life of chronic dialysis.
文摘Fibromuscular dysplasia(FMD)is a rare disease with a typical clinical manifestations.This article will describe the clinical features of a FMD case with severe headache misdiagnosed as cerebral artery dissection(CAD).Treatment remedies:we collected the clinical data of a patient with severe headache and performed brain MRI(magnetic resonance imaging)and cerebral arteriovenous MRA(magnetic resonance angiography).The preliminary diagnosis was considered as right vertebral artery dissection,and then cerebral DSA(digital subtraction angiography)was performed for further diagnosis.Post treatment evaluating:cerebral angiography showed that the vessel wall was stiff,with multiple(insect phagocytic)-like changes,and severe stenosis of local long segments.Some vessels showed“double lumen sign”,which was consistent with the performance of FMD.After anticoagulant treatment,the patient’s condition was improved.Conclusions:for severe headache,in addition to common diseases,the possibility of FMD should also be paid great attention.Early cerebrovascular screening is conducive to early diagnosis and timely treatment.
文摘The vascular diseases including aneurysm, occlusion, and thromboses in the mesenteric lesions could cause severe symptoms and appropriate diagnosis and treatment are essential for managing patients. With the development and improvement of imaging modalities, diagnostic frequency of these vascular diseases in abdominal lesions is increasing even with the small changes in the vasculatures. Among various vascular diseases, fibromuscular dysplasia(FMD) and segmental arterial mediolysis(SAM) are noninflammatory, nonatherosclerotic arterial diseases which need to be diagnosed urgently because these diseases could affect various organs and be lethal if the appropriate management is not provided. However, because FMD and SAM are rare, the cause, prevalence, clinical characteristics including the symptoms, findings in the imaging studies, pathological findings, management, and prognoses have not been systematically summarized. Therefore, there have been neither standard diagnostic criteria nor therapeutic methodologies established, to date. To systematically summarize the information and to compare these disease entities, we have summarized the characteristics of FMD and SAM in the gastroenterological regions by reviewing the cases reported thus far. The information summarized will be helpful for physicians treating these patients in an emergency care unit and for the differential diagnosis of other diseases showing severe abdominal pain.
文摘Segmental arterial mediolysis (SAM) is an uncommon non-inflammatory iatrogenic arteriopathy caused by alpha-1 adrenergic agonists or Beta-2 agonists able to release norepinephrine from the peripheral nervous system. Causative agents include adrenergic agonists used to control blood pressure, B-2 tocolytic agonists, and ractopamine used as a repartitioning agent in animal husbandry. The liberated norepinephrine both injures and stimulates a robust reparative response in the muscular arteries in the abdomen, brain base, and coronary arteries. This response may be augmented by endothelin-1 formed in the arterial adventitia. Three types of arterial lesions develop in the injurious stage: 1) apoptotic induced mediolysis, 2) separation of the outer media from the adventitia and 3) the formation of arterial gaps. The latter enlarge, particularly in elderly patients, to form gap-aneurysms complicated by dissections and dissecting an- eurysms that when ruptured cause the calamitous hemorrhages that clinically announce SAM. The other types of injury remain clinically silent but with repair develop sequelae and can metamorphose into fibromuscular dysplasia. The sequelae are mainly asymptomatic but may cause arterial stenosis and ischemic lesions. The definitive diagnosis of SAM re- quires histological conformation but misinterpreta- tion of smooth muscle vacuolar change has caused di- agnostic errors. Muscular artery cystic necrosis a newly named non-inflammatory muscular artery ar- teriopathy may be confused with SAM both clinically and pathologically. This arteriopathy represents the muscular artery equivalent of cystic media necrosis of the elastic arteries since it exhibits similar morphol- ogic features and can occur concomitantly with this entity. Adrenergic agents to counter hemorrhagic shock in SAM are contraindicated since they may intensify injury and create new lesions. The use of norepinehrine antagonists introduces a new, but as yet untested, treatment option for SAM.
文摘Background: Fibromuscular dysplasia (FMD) is a rare idiopathic segmental non-arteriosclerotic and non-inflammatory arterial disease of small to medium-sized vessels. Although it is considered a benign entity, FMD can lead to potentially severe cerebrovascular complications. We present an atypical clinical case of ischemic stroke (IS) caused by bilateral carotid dissection revealing multifocal FMD. Case Report: A 43-year-old right-handed female with no medical history suddenly developed somnolence with left-sided weakness. Clinical examination revealed left hemiplegia and left central facial paralysis with an NIHSS score: 22. Cerebral tomography (CT) with arterial angiogram revealed abilateralis of the middle cerebral arteries (MCA), a double dissection of the internal carotids (IC), aneurysmal ectasia of the left IC, and aneurysm of the M2 segment of the MCA. Brain magnetic resonance imaging (MRI) with arterial angiography confirmed the bilateral dissection. A cerebral angiography (CA) revealed a radiological pattern of multifocal FMD. The angiography of the aorta and its branches showed adiffuse dysplasia of the renal arteries. The patient was put under antiaggregants. The modified Rankin score (mRS) after three months was three. Conclusion: Cerebrovascular FMD has typical angiographic features, the “string of beads” appearance being the most common. Although the risk of a stroke in patients with cervical FMD is low, it can lead to a severe stroke and the long-term risks are unknown. Patient registries should be encouraged to better understand the mechanisms and evolutionary profile of FMD and alert clinicians to this potentially disabling condition.
文摘Segmental arterial mediolysis (SAM), an uncommon arteriopathy putatively caused by norepinephrine released by alpha-1 adrenergic agonists or some Beta-2 agonists capable of releasing norepinephrine from the peripheral sympathetic nervous system potentially can present ischemic and organ injury symptoms caused by sequelae created in its reparative phase in lieu of catastrophic hemorrhages announced in its injurious phase. The case documents this presentation—the patient presenting renal infarcts and ischemic lesions causing abdominal angina, hypertension and a nephrectomy event developing 10 years after prolonged ritodrine treatment for premature labor. This agent may have directly caused SAM or sensitized the patient to agonists causing SAM encountered at a latter date. A variety of lesions derived from injurious phase arterial injuries characterize reparative phase SAM. All were encountered in a hilar branch of the resected renal artery. These included side-by-side sequela aneurysms grossly forming a large fusiform aneurysm, granulation tissue filling adventitial medial tear spaces in which a dissecting hematomas developed, medial muscle loss centered to the outer media repaired with fibrous tissue, arterial stenosis created by reparative intimal plaques, and arterial thrombo-embolism. These lesions were mirrored in accompanying radiologic studies. The accompanying renal vein exhibited changes consistent with repair of the spastic venous angiopathy that often accompanies abdominal SAM. This angiopathy, putatively induced by Endothelin-1, suggested that this agent played a role in the genesis of the arterial lesions. Angiographic resolution of non-treated sequelae occurred in 5 months either spontaneously or due to treatment with bosentem. Conclusions: The histologic and angiographic changes demonstrate that the clinical onset of reparative SAM may be significantly delayed to produce ischemic lesions, renal infarction and in this case report, medial fibromuscular dysplasia in the hilar branch of the renal artery.
文摘A previously healthy 25-year-old man with no known risk factors was presented at the emergency room with a 3 h history of abdominal and loin pain.Physical examination and lab data showed no specific findings except tenderness,slight white cell count elevation and decreased haemoglobin level.The patient’s condition deteriorated over the following hours and he died despite resuscitation attempts.Autopsy revealed a 2.5-cm longitudinal tear in the intima of the right common iliac artery,which formed a thrombosed false lumen extending to the abdominal aorta proximally and to the left common iliac artery.Histopathologic examination revealed the characteristic changes of fibromuscular dysplasia(FMD).FMD involving the common iliac arteries is extremely rare;only six cases have been reported previously,and only two of those included forensic findings.The presented case is the first case of FMD with intimal tearing in the right common iliac artery,with propagation to the left common iliac artery and abdominal aorta.When a previously healthy young adult without hypertension or other risk factors presents with acute abdominal and loin pain,systemic vascular disease should be on the list of differential diagnoses.Careful and complete evaluation of multiple arteries can be critical.
基金the National Natural Science Foundation of China(Grant No.81471195)the second affiliated hospital of Soochow university preponderant clinic discipline group project funding(Grant No.XKQ2015002).
文摘Fibromuscular dysplasia(FMD)is a noninflammatory,nonatherosclerotic,and multifocal vascular disease,commonly involving the cerebral and renal arteries.Cerebrovascular stenosis and spontaneous dissection resulting from cerebrovascular FMD(cFMD)is one of the important causes of young stroke.Here,we reported the case of cFMD in a 28-year-old male patient with stroke.Digital subtraction angiogram demonstrated a dissecting aneurysm in the carotid artery and multiple stenoses in both vertebral arteries.Endovascular angioplasty with balloon predilation and stenting was successfully performed for the patient when the medical treatment was not adequate.The follow-up showed a remarkable improvement and no recurrence of stroke.
