Acute aortic syndrome (AAS) describes the presentation of patients with one of a number of life threatening aortic pathologies that give rise to aortic symptoms.
The spectrum of these aortic emergencies include:
aortic intramural haematoma
penetrating atherosclerotic ulcer
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 (non-STEMI)
Anterior choroidal artery syndrome is a rare entity characterised by the triad of hemiplegia, hemianaesthesia and contralateral hemianopia as a result of cerebral infarction in the anterior choroidal artery territory.
The syndrome may also be associated with neuropsychological disorders, includ...
Antiphospholipid syndrome is a systemic autoimmune disorder. It is usually defined as the clinical complex of vascular occlusion and ischaemic events occurring in patients who have circulating antiphospholipid antibodies.
Patients have circulating antiphospholipid antibodies cross-re...
Blue rubber bleb nevus syndrome (BRBNS) or Bean syndrome, is a rare sporadic syndrome characterised by multifocal venous anomalies. Patients often have multiple soft blue skin lesions associated with multiple bowel venous malformations, which could lead to lower gastrointestinal bleeding.
Budd-Chiari syndrome refers to the clinical picture that occurs when there is partial or complete hepatic venous outflow obstruction.
Budd-Chiari syndrome is rare. A Japanese study estimated the prevalence to be in the region of 2.4 cases/million 4. In Western populations, the mo...
Carney complex (not to be confused with the Carney triad) is a rare multiple endocrine neoplasia syndrome, which is autosomal dominant and characterised by 1-4:
seen in two-thirds of patients with Carney complex
skin pigmentation (blue naevi): especially of the ...
Carotidynia or (Fay syndrome) is a rare syndrome characterised by neck pain in the region of the carotid bifurcation. There is confusion in the literature as to what this term actually refers to, with some authors suggesting that the term should be reserved for a pain syndrome with no structural...
Chronic hereditary lymphedema or Milroy disease is a condition characterised by lower-limb lymphedema. Patients typically present with pedal oedema at or before birth or soon after. Occasionally, it develops later in life.
Mutations in the FLT4 gene is thought to be present...
Cogan syndrome is a rare vasculitis of children and young adults which primarily characterised by 1,4,6:
inflammatory eye disease (ocular keratitis, uveitis, scleritis, optic neuritis) 6
audiovestibular symptoms (similar to Meniere disease) 6
However, it can potentially affect a multitude of ...
Ehlers-Danlos syndrome comprises a heterogeneous group of collagen disorders (hereditary connective tissue disease).
There is a recognised male predominance.
Clinically manifests by skin hyperelasticity and fragility, joint hypermobility and blood vessel fr...
Familial multiple cavernous malformation syndrome(s) are uncommon, accounting for only a minority of cavernous malformations.
It has been more frequently reported in patients of Hispanic descent 1.
The presentation is most commonly with seizures (38-55%) 1 ...
Hereditary haemorrhagic telangiectasia (HHT) is also known as Osler-Weber-Rendu syndrome.
Worldwide prevalence ~1.5 per 100,000. Wide geographic variability with much higher incidence in certain regions, e.g. 1 in 200 in Dutch Antilles, 1 in 3500 in France.
Heyde syndrome is an association between aortic valve stenosis and gastrointestinal haemorrhage.
The aetiology 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...
Hyperperfusion syndrome after carotid endarterectomy (CEA) or carotid artery stenting (CAS) is a rare complication.
Hyperperfusion occurs in ~7.5% (range 1-14%) of patients but only a minority (~1.5%) of patients are symptomatic 1,2, with incidence being reported slightly more af...
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...
Kasabach-Merritt syndrome, also known as haemangioma thrombocytopaenia syndrome, is a rare life-threatening disease found in infants in which a rapidly growing vascular tumour is responsible for thrombocytopaenia, microangiopathic haemolytic anaemia and consumptive coagulopathy.
Klippel-Trénaunay-Weber syndrome (KTWS) is a syndrome combination of capillary malformations, soft-tissue or bone hypertrophy, and varicose veins or venous malformations. It is considered an angio-osteo-hypertrophic syndrome.
KTS classically comprises a triad of:
port wine nevi
bony or soft t...
Lemierre syndrome refers to thrombophlebitis of the jugular veins with distant metastatic sepsis in the setting of initial oropharyngeal infection such as pharyngitis/tonsillitis with or without peritonsillar or retropharyngeal abscess.
Patients typically present unwell...
Marfan syndrome is a multisystem connective tissue disease with autosomal dominant inheritance of defect in fibrillin 1 gene. The affected patients are tall with long disproportionate extremities and have pectus excavatum, arachnodactyly, and may also experience upward and lateral optic lens dis...
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...
Midaortic syndrome (MAS) is an uncommon entity affecting children and young adults. It is characterised by progressive narrowing of the abdominal aorta and its major branches.
The onset of symptoms is usually during young adulthood or childhood 2:
hypertension (most comm...
Nutcracker syndrome is a vascular compression disorder and refers to the compression of the left renal vein between the superior mesenteric artery (SMA) and aorta. This can lead to renal venous hypertension, resulting in rupture of thin-walled veins into the collecting system with resultant haem...
Ovarian vein syndrome is a relatively rare condition where a dilated ovarian vein causes notching, dilatation, or obstruction of the ureter. This is usually secondary to varicoses of the ovarian vein or ovarian vein thrombosis and occurs at the point where the ovarian vein crosses the ureter.
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.
It is associated with forced...
Popliteal artery entrapment syndrome (PAES) 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 (MHG) or less commonly ...
Post thrombotic syndrome (PTS) is a syndrome of chronic venous insufficiency following deep vein thrombosis due to valvular incompetence which results in chronic reflux and chronic venous hypertension.
PTS is a common complication following extensive DVT of the limbs. Up to half o...
Post-embolisation syndrome (PES) is one of the commonest side effects of transarterial embolisation and chemoembolisation. It comprises of a constellation of fever, nausea/vomiting, and pain. It usually occurs within the first 72 hours after embolisation (liver lesion or uterine fibroids) and th...
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
Proteus syndrome is a rare congenital, multisystemic, hamartomatous condition characterised by asymmetrical overgrowth of almost any part of the body and a broad spectrum of manifestations. It can affect tissue from any germinal layer. It is suspected to be a genetic condition, but a particular ...
Pseudoxanthoma elasticum (PXE), also known as Grönblad–Strandberg syndrome is a systemic condition charaterised by degeneration of elastic fibers. It has multi-organ manifestations.
Its prevalence is estimated to be around 1 in 25,000 9.
Patients may prese...
Rotational occlusion of the vertebral artery, also known as Bowhunter syndrome, is a rare form of posterior cerebral circulation ischemia secondary to dynamic compromise of the dominant vertebral artery.
It has many aetiologies, but is most often due to large osteophytes or atlanto-axial hyperm...
Segmental arterial mediolysis (SAM) is an increasingly recognised vascular disease of the middle-aged and elderly and a leading cause of spontanoeus intra-abdominal haemorrhage. It is characterised by fusiform aneurysms, stenoses, dissections and occlusions within splanchnic arterial branches. I...
Shone syndrome is characterised by four congenital heart defects, largely multiple left sided obstructions:
supravalvular mitral membrane (SVMM)
subaortic stenosis (membranous or muscular)
parachute mitral valve
coarctation of the aorta
Stewart-Treves syndrome refers to an angiosarcoma seen in the setting of post-mastectomy lymphoedema. It however only accounts for 10% of all angiosarcomas seen in the setting of chronic post-mastectomy lymphoedema.
Subclavian steal syndrome (SSS) and subclavian steal phenomenon both result from occlusion or severe stenosis of the proximal subclavian artery resulting in retrograde flow in the ipsilateral vertebral artery.
Subclavian steal phenomenon refers to steno-occlusive disease of the pro...
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...
Superior mesenteric artery (SMA) syndrome, also known as Wilkie syndrome, is a rare acquired vascular compression disorder in which acute angulation of superior mesenteric artery (SMA) results in compression of the third part of the duodenum leading to obstruction.
It should not be confused wit...
Superior vena cava (SVC) obstruction can occur from extrinsic compression, intrinsic stenosis or thrombosis. Malignancies are the main cause and are considered an oncologic emergency. Superior vena cava syndrome (SVCS) refers to the clinical syndrome with symptoms that results from this obstruct...
von Hippel-Lindau disease
Terson syndrome refers to vitreous haemorrhage associated with subarachnoid haemorrhage, however some authors include retinal haemorrhage as well. The syndrome is a poor prognostic marker in patients with subarachnoid haemorrhage.
Terson syndrome has been reported to occur in 13-5...
Thoracic outlet syndrome (TOS) 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.
Clinical presentation will depend on the structure compre...
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 ischaemia due to occlusion of perforator vessels.
Clinically, top of the basilar syn...
The are numerous vascular syndromes that can occur in the body. There include
Syndromes principally involving the vascular system
coeliac artery compression syndrome
hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)
hypothenar hammer syndrome
Williams syndrome (WS) is characterised by some or all or the following features:
craniofacial dysmorphism (e.g. elfin facies)
short stature (50% of cases)
mild to moderate mental retardation
supravalvular aortic stenosis 2
pulmonary artery stenosis 3
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...