Klippel-Trenaunay

Klippel-Trenaunay syndrome has three characteristics
Klippel-Trenaunay

The medical term for Klippel-Trenaunay syndrome is angio-osteohypertrophy syndrome.

Klippel-Trenaunay syndrome has three characteristics, namely varicose veins, wine stains and abnormal growth of soft tissue or bones. Soft tissue are tissues that are located under the skin, around the organs and bones or in the spaces between them. The diagnosis is made when at least two of the abovementioned deviations occur in the same arm or leg. Other parts of the body can also be affected.

Symptoms

Children suffering from this syndrome therefore have two or more abnormalities in one or more limbs (arms / legs); varicose veins, wine stains and a deviant growth of soft tissues and bones. The wine stain is often visible from birth. The varicose veins and the deviant growth only occur later, but often at a young age. The wine stain is usually on an arm or leg, but sometimes the wine stain is present on the hip, stomach, buttocks or the shoulders, chest and back. Due to the excessive growth, there is sometimes a difference between the length and / or thickness of the two legs or arms. It also happens that children have more than ten fingers / toes (polydactyly) or that fingers / toes are fused together (syndactyly). The severity of the symptoms varies per patient.

Therapy

Klippel-Trenaunay’s syndrome can not be cured. However, the symptoms can be reduced by specific treatment. Laser symptoms can reduce the symptoms of a wine stain. Treatment of varicose veins in people with Klippel-Trenaunay syndrome is more difficult than in people with common varicose veins. That is why it is preferable to choose a support stocking. Leg length difference can cause postural problems and back problems and will be treated with heel elevation or surgery.
This syndrome falls under the complex mixed malformations because there is a capillary venous malformation in which lymph vessels sometimes play a role (see ISSVA classification).

Source: https://www.huidhuis.nl/huidaandoening/syndroom-van-klippel-trenaunay

Articles & video

Articles

Intraneural hemangioma in Klippel-Trenaunay syndrome: role of musculo-skeletal ultrasound in diagnosis-case report and review of the literature

Diagnosis and management of the venous malformations of Klippel-Trénaunay syndrome

Ophthalmic Alterations in the Sturge-Weber Syndrome, Klippel-Trenaunay Syndrome, and the Phakomatosis Pigmentovascularis: An Independent Group of Conditions?

Letter: Spinal Neurovascular Malformations in Klippel-Trenaunay Syndrome: A Single Center Study

In Reply: Spinal Neurovascular Malformations in Klippel-Trenaunay Syndrome: A Single Center Study

Extensive Cellulitis and Bacteremia Due to Streptococcus Pseudoporcinus in a Child With Klippel-Trenaunay Syndrome

Tracheal Varicose Veins Associated with Klippel-Trenaunay Syndrome

A neonate with Klippel-Trénaunay syndrome: a case report

Intralesional Bleomycin Injection in Management of Localized Venous Problems in Children with Klippel-Trenaunay Syndrome

Emerging Role of Sirolimus in the Treatment of Diffuse Venous Malformation in a Woman with Klippel-Trenaunay Syndrome

Large abdominal cutaneous bypass that developed naturally in Klippel-Trénaunay syndrome

Orthostatic intolerance with Klippel-Trenaunay syndrome

Pregnancy with Klippel-Trenaunay syndrome

Mechanochemical Endovenous Ablation of Varicose Veins in Pediatric Patients with Klippel-Trénaunay Syndrome: Feasibility, Safety, and Initial Results

Case Series of Concomitant Klippel-Trenaunay Syndrome and May-Thurner Syndrome

Diagnosis of Klippel-Trenaunay syndrome and extensive heterotopic ossification in a patient with a femoral fracture: a case report and literature review

Potential Utilization of Lymphoscintigraphy in Patients With Klippel-Trenaunay Syndrome

Intraneural hemangioma in Klippel-Trenaunay syndrome: role of musculo-skeletal ultrasound in diagnosis-case report and review of the literature

Diagnosis and management of the venous malformations of Klippel-Trénaunay syndrome

Ophthalmic Alterations in the Sturge-Weber Syndrome, Klippel-Trenaunay Syndrome, and the Phakomatosis Pigmentovascularis: An Independent Group of Conditions?

Diagnosis of Klippel-Trenaunay syndrome and extensive heterotopic ossification in a patient with a femoral fracture: a case report and literature review

Potential Utilization of Lymphoscintigraphy in Patients With Klippel-Trenaunay Syndrome

Spinal Neurovascular Malformations in Klippel-Trenaunay Syndrome: A Single Center Study

A pilot study of next generation sequencing-liquid biopsy on cell-free DNA as a novel non-invasive diagnostic tool for Klippel-Trenaunay syndrome

Sclerotherapy treatment of a large venous malformation invading the bladder wall related to Klippel-Trenaunay syndrome

Large abdominal cutaneous bypass that developed naturally in Klippel-Trénaunay syndrome

Inverse Klippel-Trenaunay

National Organization for Rare Diseases USA

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Klippel-Trenaunay

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