Radiological Bone Phenotype Description of Patients With KTS ( Klippel-Trénaunay Syndrome )
Conditions
Brief summary
Clinical and paraclinical characterization of osseous anomalies of the long bones of patients with Klippel Trénaunay syndrome
Detailed description
The objective is to better characterize bone damage in KTS as well as their risks in the shorter or medium term (potentially higher risks of long bone fractures, hemorrhagic risks in the event of a long bone fracture, osteoarthritis risks or risk osteoporosis) as well as the need for rheumatological care in the care pathway.
Interventions
DIAGNOSTIC_TESTOsteodensitometry
exploration of long bones using non-invasive Guided Wave Velocity (GWV) technology. comparison of ultrasound parameters (cortical thickness and porosity, among others) with BMD values (spine, right and left femoral necks) obtained by DXA. \[Time Frame: 20 MIN\]
Sponsors
University Hospital, Grenoble
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
OTHER
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
7 Years to No maximum
Healthy volunteers
No
Inclusion criteria
Vascular malformation such as Klippel-Trénaunay syndrome or Capillary malformations (CM) + venous malformations (VM) +/- Lymphatic malformations (LM) + limb overgrowth according to ISSVA (International Society for the Study of Vascular Anomalies) classification. * Signature of study consent by subject of legal age or by 2 legal guardians for minors Patient affiliated to a social security scheme or beneficiary of such a scheme * Age greater than or equal to 7 years
Exclusion criteria
Pregnant or breast-feeding woman Protected person \-
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Quantification of limb-length asymmetry on lower-limb telemetry (absolute values, in mm) compared with the contralateral side | 20 minutes | The assessment of bone damage includes an X-ray of a weight-bearing pelvis and limb telemetry to detect any limb length abnormalities. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| description vascular of the KTS. | 30 MINUTES | classification of vascular dysplasia using the International Classification of Vascular Anomalies (ISSVA), Hamburg classification, presence or absence of lymphatic involvement, presence or absence of localized or diffuse intravascular coagulopathy, vascular infiltrative bone involvement on MRI. |
| measurement of bone density (BMD) in the spine and femoral necks, with calculation of the FRAX index. | 20 MINUTES | quantitative analysis with measurement of bone density (BMD) in the spine and 2 femoral necks by bone densitometry, images obtained by two-photon X-ray absorptiometry or DXA combined with clinical risk factors using the FRAX tool. |
| exploration of long bones using non-invasive Guided Wave Velocity (GWV) technology. | 20 MINUTES | comparison of ultrasound parameters (cortical thickness and porosity, among others) with BMD values (spine, right and left femoral necks) obtained by DXA. |
Countries
France
Outcome results
None listed