Clinical and Paraclinical Characterization of Osseous Anomalies of the Long Bones of Patients With Klippel Trénaunay Syndrome

N/AActive Not RecruitingNCT07060703

University Hospital, Grenoble15 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

Purpose

OTHER

Masking

NONE

Enrollment

Conditions

Radiological Bone Phenotype Description of Patients With KTS ( Klippel-Trénaunay Syndrome )

Interventions

OsteodensitometryDIAGNOSTIC_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. \[Time Frame: 20 MIN\]

Eligibility

Sex: ALLMin age: 7 Years

Medical Language ↔ Plain English

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 \-

Locations (1)

Chu Grenoble Alpes

La Tronche, France

Outcomes

Primary Outcomes

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.

Time frame: 20 minutes

Secondary Outcomes

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.

Time frame: 30 MINUTES

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.

Time frame: 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.

Time frame: 20 MINUTES

description vascular of the KTS.

Cortical thickness (Ct.Th) expressed en mm and porosity (Ct.Po) expressed in percentage %.

Time frame: 20 minutes

Data from ClinicalTrials.gov

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