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Clinical and paraclinical characterization of osseous anomalies of the long bones of patients with Klippel Trénaunay syndrome
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Radiological Bone Phenotype Description of Patients With KTS | Other |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Osteodensitometry | Diagnostic Test | 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] |
| Measure | Description | Time Frame |
|---|---|---|
| Quantification of limb-length asymmetry on lower-limb telemetry (absolute values, in mm) compared with the contralateral side | The assessment of bone damage includes an X-ray of a weight-bearing pelvis and limb telemetry to detect any limb length abnormalities. | 20 minutes |
| Measure | Description | Time Frame |
|---|---|---|
| description vascular of the KTS. | 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. | 30 MINUTES |
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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.
Exclusion Criteria:
Pregnant or breast-feeding woman Protected person
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| Name | Affiliation | Role |
|---|---|---|
| SOPHIE M BLAISE, INVESTOGATOR | University Hospital, Grenoble | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Chu Grenoble Alpes | La Tronche | 38700 | France |
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|
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| measurement of bone density (BMD) in the spine and femoral necks, with calculation of the FRAX index. |
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. |
| 20 MINUTES |
| 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. | 20 MINUTES |
| description vascular of the KTS. | Cortical thickness (Ct.Th) expressed en mm and porosity (Ct.Po) expressed in percentage %. | 20 minutes |