Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 243.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 243.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 243.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\25572952
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 AJNR+Am+J+Neuroradiol
2015 ; 36
(5
): 863-70
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Neurovascular manifestations in hereditary hemorrhagic telangiectasia: imaging
features and genotype-phenotype correlations
#MMPMID25572952
Krings T
; Kim H
; Power S
; Nelson J
; Faughnan ME
; Young WL
; terBrugge KG
AJNR Am J Neuroradiol
2015[May]; 36
(5
): 863-70
PMID25572952
show ga
BACKGROUND AND PURPOSE: Hereditary hemorrhagic telangiectasia is an autosomal
dominant disease that presents in 10%-20% of patients with various brain vascular
malformations. We aimed to report the radiologic features (phenotype) and the
genotype-phenotype correlations of brain vascular malformations in hereditary
hemorrhagic telangiectasia. MATERIALS AND METHODS: Demographic, clinical,
genotypic, and imaging information of 75 patients with hereditary hemorrhagic
telangiectasia with brain arteriovenous malformations enrolled in the Brain
Vascular Malformation Consortium from 2010 to 2012 were reviewed. RESULTS:
Nonshunting, small, superficially located conglomerates of enhancing vessels
without enlarged feeding arteries or draining veins called "capillary vascular
malformations" were the most commonly observed lesion (46 of 75 patients; 61%),
followed by shunting "nidus-type" brain AVMs that were typically located
superficially with a low Spetzler-Martin Grade and a small size (32 of 75
patients; 43%). Direct high-flow fistulous arteriovenous shunts were present in 9
patients (12%). Other types of vascular malformations (dural AVF and
developmental venous anomalies) were present in 1 patient each. Multiplicity of
vascular malformations was seen in 33 cases (44%). No statistically significant
correlation was observed between hereditary hemorrhagic telangiectasia gene
mutation and lesion type or lesion multiplicity. CONCLUSIONS: Depending on their
imaging features, brain vascular malformations in hereditary hemorrhagic
telangiectasia can be subdivided into brain AVF, nidus-type AVM, and capillary
vascular malformations, with the latter being the most common phenotype in
hereditary hemorrhagic telangiectasia. No genotype-phenotype correlation was
observed among patients with this condition.