25 results found
Article
Loeys-Dietz syndrome
Loeys-Dietz syndrome is an autosomal dominant connective tissue disorder which has many features similar to Marfan syndrome.
The disease is characterized by skeletal manifestations and vasculopathies. Although Loeys-Dietz syndrome shares many similarities with Marfan syndrome, the course is oft...
Case
Loeys-Dietz syndrome
Published
18 Oct 2022
95% complete
CT
MRI
Case
Loeys-Dietz syndrome
Published
19 Feb 2015
68% complete
MRI
Article
Aortic dissection
Aortic dissection is the prototype and most common form of acute aortic syndromes and a type of arterial dissection. It occurs when blood enters the medial layer of the aortic wall through a tear or penetrating ulcer in the intima and tracks longitudinally along with the media, forming a second ...
Article
Hypertelorism
Hypertelorism refers to an abnormal increase in distance between any two organs although some authors use the term synonymously with orbital hypertelorism meaning an abnormal increase in distance between the two eyes. The article mainly focuses on the latter. The abnormality is similar to teleca...
Article
Posterior vertebral scalloping (mnemonic)
A useful mnemonic to remember the differential diagnoses for posterior vertebral scalloping is:
SALMON
Mnemonic
S: spinal cord tumor (e.g. astrocytoma, ependymoma, schwannoma)
A: achondroplasia, acromegaly
L: Loeys-Dietz syndrome (and other connective tissue disorders)
M: Marfan's syndrome...
Article
Tarlov cyst
Tarlov cysts, also called perineural cysts, are CSF-filled dilatations of the nerve root sheath at the dorsal root ganglion (posterior nerve root sheath). These are type II spinal meningeal cysts that are, by definition, extradural but contain neural tissue.
Most Tarlov cysts are asymptomatic, ...
Article
Vertebral scalloping
Vertebral scalloping is a concavity to the posterior (or less commonly anterior) aspect of the vertebral body when viewed in a lateral projection. A small amount of concavity is normal, as is concavity of the anterior vertebral body (see vertebral body squaring).
Posterior scalloping
Causes of...
Article
Arachnodactyly
Arachnodactyly refers to the physical finding of elongated, thin "spider-like" fingers, which are a classic feature of Marfan disease, but by no means pathognomonic 1.
Radiographic features
The metacarpal index was historically-used as a radiographic criterion for arachnodactyly. To obtain it,...
Article
Spinal meningeal cyst
Spinal meningeal cysts are diverticulae of the arachnoid or dura mater or of the nerve root sheath. They are uncommon, usually asymptomatic and typically found incidentally on MRI.
Clinical presentation
They cysts are usually asymptomatic, but if they are large, they may cause mass effect and...
Article
Aortic dissection detection risk score
The aortic dissection detection risk score (ADD-RS) is a clinical decision tool that aids in grading the pretest probability of an acute aortic dissection. Scores range from 0-3, where 0 is classed as low risk, 1 is moderate risk and 2-3 is high risk 1.
Criteria
The three domains in which pati...
Article
Pulmonary trunk dilatation
Dilatation of the pulmonary trunk or dilatation of the main pulmonary artery (mPA) has a range of causes, and is often (though not always) associated with pulmonary hypertension. Most publications suggest an upper limit of normal in the region of 29-33 mm 1, which can be higher in males 7. Other...
Article
Ascending aortic aneurysm
Ascending aortic aneurysms are the most common subtype of thoracic aortic aneurysms and may be true or false aneurysms.
Epidemiology
Ascending aortic aneurysms represent 60% of thoracic aortic aneurysms.
Clinical presentation
Typically ascending aortic aneurysms are an incidental finding a...
Article
Thoracic aortic aneurysm
Thoracic aortic aneurysms are a type of thoraco-abdominal aneurysms and are relatively uncommon compared to abdominal aortic aneurysms. There is a wide range of causes, and the ascending aorta is the segment most commonly affected. Both CT-angiography and MR-angiography are the modalities of cho...
Article
Myxomatous mitral valve degeneration
Myxomatous mitral valve degeneration, myxomatous degeneration of the mitral valve, myxomatous mitral valve disease or simply myxomatous mitral valve is a non-inflammatory progressive alteration of the mitral valve structure associated with mitral valve prolapse and mitral insufficiency.
Termino...
Article
Dural ectasia
Dural ectasia refers to ballooning or widening of the dural sac which can result in posterior vertebral scalloping and is associated with herniation of nerve root sleeves.
Clinical presentation
Patients with dural ectasia may present with low back pain or radicular pain in the buttocks or legs...
Article
Uvula
The uvula is a small, conical, pendulous process projected inferiorly from the midline posterior margin of the soft palate. It is primarily formed from the insertions of the two muscles of the uvula and their covering mucosa.
Summary
location: posterior midline soft palate
blood supply: from ...
Article
Aortic arch aneurysm
An aortic arch aneurysm is a less common form of thoracic aortic aneurysm and may account for around 10% of such aneurysms.
Epidemiology
Aortic arch aneurysms have a recognized male predilection, with most patients presenting around the 6th to 7th decades of life.
Clinical presentation
It ca...
Article
Connective tissue disease
The connective tissue diseases or disorders (CTDs), also known as collagen vascular diseases, form a large heterogeneous group of conditions that are linked by a disease process that primarily involves the connective soft tissues of the body and often the vasculature too, due to shared structura...
Article
Pulmonary arterial ectasia
Pulmonary arterial ectasia refers to more diffuse dilatation of the pulmonary arteries without a focal pulmonary arterial aneurysm. At the time of writing, the upper limit of normal (90th percentile) for the main pulmonary arteries is taken at just under 29 mm for males and just under 27 mm for ...
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