Articles

Articles are a collaborative effort to provide a single canonical page on all topics relevant to the practice of radiology. As such, articles are written and edited by countless contributing members over a period of time. A global group of dedicated editors oversee accuracy, consulting with expert advisers, and constantly reviewing additions.

69 results found
Article

VEXAS syndrome

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a severe, treatment-refractory, monogenic, multiorgan, autoinflammatory condition with vasculitic and hematological complications. Epidemiology VEXAS syndrome is likely to be rare, but also likely to be underdiagnosed...
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PIK3CA-related overgrowth spectrum (PROS)

PIK3CA-related overgrowth spectrum (PROS) is a broad group of segmental overgrowth disorders that can occur with or without associated vascular anomalies, caused by activating mutations in the PIK3CA gene, which are also responsible for the vast majority of isolated lymphatic malformations and s...
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Multisystemic smooth muscle dysfunction syndrome

Multisystemic smooth muscle dysfunction syndrome is a rare genetic disorder caused by mutations in the ACTA2 gene, resulting in intracranial steno-occlusive disease and aortic dissection or aneurysm, among other complications. Epidemiology Most cases are diagnosed in childhood 1. Clinical pre...
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McCleery syndrome

McCleery syndrome is a subtype of venous thoracic outlet syndrome with symptoms resulting from compression of the subclavian vein without thrombosis (in contrast to Paget-Schroetter syndrome).  Clinical presentation Patients with McCleery syndrome present with intermittent obstruction of the s...
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Arterial tortuosity syndrome

Arterial tortuosity syndrome (ATS) is a very rare autosomal recessive connective tissue disease. It is similar to Ehlers-Danlos syndrome (EDS), the major differentiator being the general tortuous nature of the larger arteries. Clinical presentation It presents similarly to other collagen disor...
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Posterior nutcracker syndrome

Posterior nutcracker syndrome is a rare vascular compression disorder, in which a retroaortic left renal vein is trapped between the aorta and the vertebral column. Clinical presentation The most reported clinical manifestation is microscopic or macroscopic hematuria. See also vascular compr...
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Tumor necrosis factor receptor-associated periodic syndrome

Tumor necrosis factor receptor-associated periodic syndrome (or sometimes abbreviated as TRAPS) is a condition characterized by recurrent (periodic) episodes of fever as well as a spectrum of dermatologic findings that includes migratory patches, edematous plaques, periorbital edema, and/or conj...
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Adductor canal syndrome

Adductor canal syndrome (also known as adductor canal compression syndrome) is a rare, non-atherosclerotic cause of arterial occlusion and limb ischemia 1. There is compression of the superficial femoral artery (SFA) in the adductor canal. Epidemiology External compression of the superficial f...
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Abdominal compartment syndrome

Abdominal compartment syndrome (ACS) is a disease defined by the presence of new end-organ dysfunction secondary to elevated intra-abdominal pressure (IAP). Radiological diagnosis is difficult and usually suggested when a collection of imaging findings are present in the appropriate clinical set...
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Metabolic syndrome

The metabolic syndrome, also known as syndrome X, is a set of five conditions, which together increase a patient's risk of developing cardiovascular disease 1. Clinical presentation There are five central components of metabolic syndrome: hyperinsulinemia impaired glucose tolerance dyslipid...
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Neurovascular compression syndromes

Neurovascular compression syndromes are a form of vascular compression disorders where there is usually compression or distortion of a cranial nerve due to a redundant or aberrant vascular structure. Clinical presentation Not all cases of neurovascular contact are clinically symptomatic. Prese...
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Phlegmasia cerulea dolens

Phlegmasia cerulea dolens (PCD) is an uncommon complication of deep venous thrombosis, which results from extensive thrombotic occlusion of the major and collateral veins of an extremity (usually the legs). Epidemiology Left leg is more commonly affected than the right 4. Risk factors Risk f...
Article

Rhupus syndrome

Rhupus syndrome is a term traditionally used to describe patients uncommonly having the coexistence of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA)
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Raynaud phenomenon

Raynaud phenomenon (also known as Raynaud syndrome) describes a localized vasculopathy whereby there is an exaggerated vascular response to cold temperature or emotional stresses. Terminology Raynaud phenomenon is classified as being either 'primary' or idiopathic, or 'secondary' to another un...
Article

Post-thrombotic syndrome

Post-thrombotic syndrome (PTS) is a syndrome of chronic venous insufficiency following deep vein thrombosis (DVT) due to valvular incompetence, which results in chronic reflux and chronic venous hypertension. Epidemiology PTS is a common complication following extensive DVT of the limbs. Up to...
Article

LUMBAR syndrome

LUMBAR, PELVIS, or SACRAL syndrome is the association of infantile hemangiomas in the lower body with other extracutaneous congenital abnormalities in the region. The syndrome may be incomplete.   Pathology LUMBAR 1 lower body hemangiomas urogenital anomalies and ulceration myelopathy bony...
Article

Cerebral hyperperfusion syndrome

Cerebral hyperperfusion syndrome is a rare complication seen after treatment of long-standing severe carotid stenosis by carotid endarterectomy or carotid artery stenting. It is believed to be the result of failure of normal cerebral blood flow autoregulation.  Epidemiology Hyperperfusion occu...
Article

Blue rubber bleb nevus syndrome

Blue rubber bleb nevus syndrome (BRBNS) or Bean syndrome, is a rare sporadic syndrome characterized by multifocal venous anomalies. Patients often have multiple soft blue skin lesions (blueberry muffin syndrome) associated with multiple bowel venous malformations, which could lead to lower gastr...
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Drug rash with eosinophilia and systemic symptoms syndrome

The drug rash with eosinophilia and systemic symptoms (DRESS) syndrome typically manifests as a skin rash, fever, lymph nodal enlargement with variable internal organ involvement, and represents a hypersensitivity reaction to medication. Clinical presentation  Clinical presentation can be vari...
Article

Pinch off syndrome

Pinch-off syndrome is a spontaneous catheter fracture, which is seen as a complication of subclavian venous catheterization. Epidemiology It is a known complication of central venous catheterization with a much-reduced incidence in current practice and is generally considered to be rare. Radi...
Article

Acute coronary syndrome

Acute coronary syndrome (ACS) is a group of cardiac diagnoses along a spectrum of severity due to the interruption of coronary blood flow to the myocardium, which in decreasing severity are: ST elevation myocardial infarction (STEMI) non-ST elevation myocardial infarction (NSTEMI) unstable an...
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Heyde syndrome

Heyde syndrome is an association between aortic valve stenosis and gastrointestinal hemorrhage. The etiology of the gastrointestinal bleeding in this setting is uncertain, but it is thought to be related to intestinal angiodysplasia. The strength of this association independent of age-related d...
Article

Chronic hereditary lymphedema

Chronic hereditary lymphedema (also known as Milroy disease) is a condition characterized by lower limb lymphedema. Patients typically present with pedal edema at or before birth or soon after. Occasionally, it develops later in life. Clinical presentation The commonest presentation is bilater...
Article

Pseudoxanthoma elasticum

Pseudoxanthoma elasticum (PXE), also known as Grönblad–Strandberg syndrome, is a systemic condition characterized by the degeneration of elastic fibers. It has multiorgan manifestations. Epidemiology Its prevalence is estimated to be around 1 in 25,000 9.  Clinical presentation Patients may ...
Article

Carotidynia

Carotidynia, also known as Fay syndrome, is a rare syndrome characterized by neck pain in the region of the carotid bifurcation. It was classified by the International Headache Society (IHS) in 1988 as an idiopathic neck pain syndrome associated with tenderness over the carotid bifurcation with...
Article

Rotational vertebral artery occlusion syndrome

Rotational vertebral artery occlusion syndrome, also known as bow hunter's syndrome, is a rare form of vertebrobasilar insufficiency secondary to dynamic compression of the usually-dominant vertebral artery.  Pathology It has many predisposing etiologies, but is most often due to large osteoph...
Article

Segmental arterial mediolysis

Segmental arterial mediolysis (SAM) is an increasingly recognized vascular disease of the middle-aged and elderly and a leading cause of spontaneous intra-abdominal hemorrhage. It is characterized by fusiform aneurysms, stenoses, dissections and occlusions within splanchnic arterial branches. Im...
Article

Anterior choroidal artery syndrome

Anterior choroidal artery syndrome is a rare entity characterized by the triad of hemiplegia hemianaesthesia and contralateral hemianopia This occurs as a result of cerebral infarction in the anterior choroidal artery territory. The syndrome may also be associated with neuropsychological di...
Article

Yellow nail syndrome

The yellow nail syndrome (YNS) is a rare disorder principally affecting the lymphatic system. It is characterized by a clinical triad: nail discolouration (chromonychia): yellow to dark green slow-growing dystrophic nails (scleronychia) 9 lymphedema (peripheral/primary) pulmonary disease: se...
Article

Antiphospholipid syndrome

Antiphospholipid syndrome is a systemic autoimmune disorder. It is usually defined as the clinical complex of vascular occlusion and ischemic events occurring in patients who have circulating antiphospholipid antibodies. Clinical presentation Antiphospholipid syndrome is characterized by venou...
Article

Idiopathic retroperitoneal fibrosis

Idiopathic retroperitoneal fibrosis, also known as Ormond disease or occasionally Albarran-Ormond syndrome 6, is a subtype of retroperitoneal fibrosis where no cause is found. It includes a spectrum of diseases which are characterized by fibroinflammatory tissue encasing the abdominal aorta and ...
Article

Ovarian vein syndrome

Ovarian vein syndrome is a relatively rare condition where a dilated ovarian vein (ovarian venous varix) causes notching, dilatation, or obstruction of the ureter. This is usually secondary to varicosities of the ovarian vein or ovarian vein thrombosis and occurs at the point where the ovarian v...
Article

Superior vena cava obstruction

Superior vena cava obstruction (SVCO) can occur from extrinsic compression, intrinsic stenosis, or thrombosis of the superior vena cava. Malignancies are the main cause and are considered an oncologic emergency. Superior vena cava syndrome (SVCS) refers to the clinical syndrome with symptoms tha...
Article

Midaortic syndrome

Midaortic syndrome is an uncommon entity affecting children and young adults. It is characterized by progressive narrowing of the abdominal aorta and its major branches. Clinical presentation The onset of symptoms is usually during childhood or young adulthood 2: hypertension (most common) i...
Article

Proteus syndrome

Proteus syndrome is a rare congenital, multisystemic, hamartomatous condition characterized by asymmetrical overgrowth of almost any part of the body and a broad spectrum of manifestations. It can affect tissue from any germinal layer. Clinical presentation Affected patients usually appear nor...
Article

F P Weber syndrome

F P Weber syndrome (FPWS) is a traditional eponymous denomination of a certain type of angiodysplasia, that would nowadays rather be called a mixed hemolymphatic congenital vascular malformation (CVM) with arteriovenous (AV) shunting, based on the Hamburg classification of CVMs. In his original...
Article

Post-embolization syndrome

Post-embolization syndrome (PES) is one of the most common complications of transarterial embolization and chemoembolisation. The condition comprises a constellation of symptoms including pain, fever, nausea and vomiting. PES usually occurs within the first 72 hours after solid organ embolizatio...
Article

Hughes-Stovin syndrome

Hughes-Stovin syndrome (HSS) is a vasculitis that predominantly affects large vessels. The disease bears some resemblance to Behçet disease. Epidemiology Hughes-Stovin syndrome is very rare 7. It occurs predominantly between the 2nd to 4th decades. There is a recognized male predilection.  Cl...
Article

Acute aortic syndrome

Acute aortic syndrome describes the presentation of patients with one of a number of life-threatening aortic pathologies that give rise to similar clinical presentations. Exactly which entities are included under the umbrella term acute aortic syndrome varies somewhat from publication to public...
Article

Ehlers-Danlos syndrome

Ehlers-Danlos syndrome comprises a heterogeneous group of collagen disorders (hereditary connective tissue disease). Epidemiology The combined prevalence for all types of Ehlers-Danlos syndrome is estimated to be at least 1 of every 5000 individuals. There is no significant gender predominance...
Article

Sulcal artery syndrome

Sulcal artery syndrome is a rare cause of spinal cord infarction involving the territory of one of the sulcal arteries, which are penetrating branches of the anterior spinal artery, each vessel supplying either left or right side of the cord, but not both. The result is an incomplete Brown-Séqua...
Article

Superior mesenteric artery syndrome

Superior mesenteric artery (SMA) syndrome, also known as Wilkie syndrome, cast syndrome or aortomesenteric duodenal compression syndrome, is a rare acquired vascular compression disorder in which acute angulation of the superior mesenteric artery (SMA) results in compression of the third part of...
Article

Terson syndrome

Terson syndrome refers to vitreous hemorrhage associated with subarachnoid hemorrhage, however, some authors include retinal hemorrhage as well. The syndrome is a poor prognostic marker in patients with subarachnoid hemorrhage. Epidemiology Terson syndrome has been reported to occur in 13-50% ...
Article

Moyamoya syndrome

Moyamoya syndrome, also termed the moyamoya pattern or phenomenon, is due to numerous conditions that can cause arterial occlusion of the circle of Willis, with resultant collaterals, and appearances reminiscent of moyamoya disease. These conditions include 1-4 : vessel wall abnormalities athe...
Article

Cogan syndrome

Cogan syndrome is a rare vasculitis of young adults that is primarily characterized by 1,4,6: inflammatory eye disease (classically interstitial keratitis) 6 audiovestibular dysfunction (similar to Meniere disease) 6 Epidemiology Cogan syndrome is rare and can occur in people of any age and ...
Article

Popliteal artery entrapment syndrome

Popliteal artery entrapment syndrome refers to symptomatic compression or occlusion of the popliteal artery due to a developmentally abnormal positioning of the popliteal artery in relation to its surrounding structures such as with the medial head of gastrocnemius or less commonly with popliteu...
Article

Williams syndrome

Williams syndrome (WS), sometimes called Williams-Beuren syndrome, is characterized by some or all of the following features: craniofacial dysmorphism (e.g. elfin facies) oral abnormalities short stature (50% of cases) mild to moderate intellectual disability  supravalvular aortic stenosis ...
Article

Marfan syndrome

Marfan syndrome is a multisystem connective tissue disease caused by a defect in the protein fibrillin 1, encoded by the FBN1 gene. Cardiovascular involvement with aortic root dilatation and dissection is the most feared complication of the disease. Epidemiology The estimated prevalence is aro...
Article

Klippel-Trénaunay-Weber syndrome

Klippel-Trénaunay-Weber syndrome (KTWS) is a very rare congenital disorder that is characterized by a triad of venous malformations, cutaneous capillary malformations, and limb overgrowth. It is considered an angio-osteo-hypertrophic syndrome. Terminology The name Klippel-Trénaunay-Weber syndr...
Article

Familial multiple cavernous malformation syndrome

The familial multiple cavernous malformation syndrome, or familial cerebral cavernous malformation syndrome, is uncommon, accounting for only a minority of cavernous malformations. Epidemiology It has been more frequently reported in patients of Hispanic descent 1. Clinical presentation The ...
Article

Shone syndrome

Shone syndrome, also known as Shone complex, is a rare syndrome characterized by left-sided, obstructive congenital heart defects. Epidemiology Shone syndrome is thought to be very rare, accounting for less than 1% of all congenital heart disease 1. Clinical presentation Patients, usually ne...
Article

Top of the basilar syndrome

Top of the basilar syndrome, also known as rostral brainstem infarction, occurs when there is thromboembolic occlusion of the top of the basilar artery. This results in bilateral thalamic ischemia due to occlusion of perforator vessels. Clinical presentation Clinically, top of the basilar synd...
Article

Subclavian steal syndrome

Subclavian steal syndrome and subclavian steal phenomenon both result from severe proximal subclavian artery stenosis or occlusion resulting in retrograde flow in the ipsilateral vertebral artery. Terminology Subclavian steal phenomenon refers to steno-occlusive disease of the proximal subclav...
Article

Posterior choroidal artery stroke

Posterior choroidal artery occlusion uncommonly presents as an isolated stroke syndrome.  It usually coexists with posterior cerebral artery and often superior cerebellar artery involvement.  When seen in isolation damage is characteristically limited to the: lateral geniculate body pulvinar ...
Article

May-Thurner syndrome

May-Thurner syndrome refers to a chronic compression of the left common iliac vein (CIV) against the lumbar vertebrae by the overlying right common iliac artery (CIA), with or without deep venous thrombosis 2. Although both left and right CIVs lie deep to the right common iliac artery, the left...
Article

Wyburn-Mason syndrome

Wyburn-Mason syndrome (also known as Bonnet-Dechaume-Blanc syndrome) is a rare, nonhereditary neurocutaneous disorder that typically presents with unilateral vascular malformations that primarily involve the brain, orbits and facial structures. It is currently classified as a craniofacial arteri...
Article

Vascular syndromes

The are numerous vascular syndromes that can occur in the body. They include: Syndromes principally involving the vascular system Budd-Chiari syndrome celiac artery compression syndrome hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) hypothenar hammer syndrome Kasabach-M...
Article

Thoracic outlet syndrome

Thoracic outlet syndrome refers to a group of clinical syndromes caused by congenital or acquired compression of the brachial plexus or subclavian vessels as they pass through the superior thoracic aperture 11.  Clinical presentation Clinical presentation will depend on the structure compresse...
Article

Stewart-Treves syndrome

Stewart-Treves syndrome refers to an angiosarcoma seen in the setting of lymphedema 1. It was classically attributed to lymphedemas induced by radical mastectomy to treat breast cancer. Nowadays, we know that it can arise in chronically lymphedematous regions of any cause 2.  The incidence is ...
Article

Paget-Schrötter syndrome

Paget-Schrötter syndrome, alternatively spelled Paget-Schroetter syndrome and also known as effort thrombosis, refers to primary thrombosis of the axillary and/or subclavian vein. It can be thought of as a venous equivalent of thoracic outlet syndrome (i.e. venous thoracic outlet syndrome). Epi...
Article

PHACE syndrome

PHACE syndrome, also known as cutaneous hemangioma–vascular complex syndrome or Pascual-Castroviejo type II syndrome, is a phakomatosis that comprises of: P: posterior fossa malformations (e.g. Dandy-Walker malformation) H: hemangiomas A: arterial anomalies C: coarctation of the aorta and ca...
Article

Nutcracker syndrome

Nutcracker syndrome is a vascular compression disorder that refers to the compression of the left renal vein most commonly between the superior mesenteric artery (SMA) and aorta, although other variations can exist 1. This can lead to renal venous hypertension, resulting in rupture of thin-walle...
Article

Lemierre syndrome

Lemierre syndrome (also known as postanginal septicemia) refers to thrombophlebitis of the internal jugular vein(s) with distant metastatic sepsis in the setting of initial oropharyngeal infection such as pharyngitis/tonsillitis with or without peritonsillar or retropharyngeal abscess. Epidemio...
Article

Kasabach-Merritt syndrome

Kasabach-Merritt syndrome, also known as hemangioma thrombocytopenia syndrome, is a rare life-threatening disease found in infants in which a rapidly growing vascular tumor is responsible for thrombocytopenia, microangiopathic hemolytic anemia and consumptive coagulopathy. Pathology Vascular t...
Article

Hypothenar hammer syndrome

Hypothenar hammer syndrome occurs from trauma to the distal ulnar artery or proximal portion of superficial palmar arch as a result of repetitive trauma to the hypothenar eminence. Originally described in patients using hammers and screwdrivers, it is also seen in various athletes such as basket...
Article

Hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare inherited disorder characterized by abnormal blood vessel formation in the skin, mucous membranes, and organs including the lungs, liver, and central nervous system. Epidemiology Worldwide prevalen...
Article

Celiac artery compression syndrome

Celiac artery compression syndrome, also known as median arcuate ligament syndrome, Dunbar syndrome, or Harjola-Marable syndrome, is a rare condition characterized by upper abdominal pain in the setting of compression of the celiac trunk by the diaphragmatic crurae. Although well-recognized as ...
Article

Carney complex

Carney complex (not to be confused with the Carney triad) is a rare multiple endocrine neoplasia syndrome characterized by 1-4: cardiac myxoma often multiple seen in two-thirds of patients with Carney complex skin pigmentation (blue nevi): especially of the face, trunk, lips, and sclera   M...
Article

Budd-Chiari syndrome

Budd-Chiari syndrome, also known as hepatic venous outflow obstruction (HVOO), refers to the clinical picture that occurs when there is partial or complete obstruction of the hepatic veins.  There is no clear consensus regarding the number of occluded veins, some authors claim that there should...

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