AVAST Anomalies Vasculaires Associées au Syndrome de Turner (Vascular Abnormalities Associated With Turner Syndrome)
AVAST Anomalies Vasculaires Associées au Syndrome de Turner (Vascular Abnormalities Associated With Turner Syndrome)
Turner syndrome is a genetic condition, rare, due to the total or partial absence of one X
 chromosome, affecting 1/2500 newborn female. It combines almost constantly short stature and
 ovarian failure with infertility.

 Other anomalies are inconstant: morphological characteristics of varying intensity,
 associated malformations, and increased risk of acquired diseases ...

 The prognosis of patients reaching the Turner Syndrome is linked to cardiovascular
 complications (congenital heart disease, dilatation of the ascending aorta with risk of
 dissection or rupture of aneurysm), causing early mortality with reduction of life expectancy
 of at least 10 years.

 For these reasons, screening for heart disease and dilatation of the ascending aorta is
 established and is intended to prevent the complications associated with medical treatment
 and / or surgery to increase life expectancy and reduce the co-morbidities.

 On the vascular level, the recommendations other than those relating to the monitoring of the
 diameter of the ascending aorta include research of renal artery stenosis by doppler
 ultrasound if the patient is hypertensive and looking for lymphedema.

 However, other arterial lesions were described in the literature, outside of the aneurysm of
 the ascending aorta. These peripheral arterial lesions can also be life and / or functional
 prognosis of the patient. Ascending aorta dilation seems not to be exclusive in Turner
 syndrome.

 In addition, specific vascular lesions outside the affected artery are described: hepatic
 cirrhosis by vascular depletion, lymphedema and varicose veins. The prevalence of venous or
 lymphatic disease is unknown.

 A single-center review of 9 cases of patients followed at the University Hospital of
 Strasbourg showed the presence of vascular lesions discovered incidentally during assessments
 performed for reasons other than cardiovascular screening: cystic lymphangioma, internal
 carotid aneurysm, agenesis of the inferior vena cava, early varicose veins, embryonic
 cerebral artery, etc ... None of these patients showed any dilatation of the ascending aorta
 or heart disease. Peripheral vascular abnormalities in this patient group are exclusive.

 In this study, we seek to demonstrate that arterial disease in Turner syndrome involve the
 entire arterial territory and is not confined to the ascending aorta. Screening for arterial
 lesions should be performed on the entire arterial vascular tree and regularly in the course
 of time, especially as the presence of cardiovascular risk factors increases with the age of
 these patients.

 The venous and lymphatic vascular damage in the literature and in our series of cases in
 University Hospital of Strasbourg description should also lead to the detection of these
 lesions.

 These vascular complications can be alone responsible for the reduction in life expectancy or
 responsible for serious morbidity. Improved screening of associated vascular lesions is
 necessary to enable the best prevention of cardiovascular complications.

 It is also to establish the prevalence of vascular anomalies, whether arterial, venous or
 lymphatic, to better understand the disease and its management. By collecting systematically
 karyotype leading to diagnosis, it may be possible to make a link between the genetic defect
 and heart or vascular disease.
Inclusion Criteria:

 -  Any woman over 18 years with Turner Syndrome confirmed by karyotype

 -  Affiliated to a social security scheme

 -  Having signed an informed consent

 -  Having been informed of the results of the medical examination prior

 Exclusion Criteria:

 -  Inability to give informed patient information related to comprehension difficulties

 -  Topic featuring against-indications for MRI examination:

 -  pacemaker or automatic defibrillator, implanted pump

 -  auditory nerve stimulator, anal nerve stimulator, etc ...

 -  the ferromagnetic objects in the soft tissues, intraocular metallic objects,
 cerebral vascular clips

 -  claustrophobia

 -  morphotype not allowing access to MRI

 -  Patient under judicial protection, guardianship or trusteeship

 -  Patient in exclusion period (as determined by a previous study or in progress)

 -  Pregnancy at the time of inclusion