{"id":463,"date":"2020-04-07T14:58:05","date_gmt":"2020-04-07T14:58:05","guid":{"rendered":"https:\/\/www.cmtc.nl\/?page_id=463"},"modified":"2025-07-29T11:43:33","modified_gmt":"2025-07-29T09:43:33","slug":"klippel-trenaunay","status":"publish","type":"page","link":"https:\/\/www.cmtc.nl\/en\/pedia\/conditions\/klippel-trenaunay\/","title":{"rendered":"Klippel-Trenaunay"},"content":{"rendered":"\t\t
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The medical term for Klippel-Trenaunay syndrome is angio-osteohypertrophy syndrome.<\/p>\n

Klippel-Trenaunay syndrome has three main characteristics: varicose veins, port-wine stains, and abnormal growth of soft tissues or bones. Soft tissues are the tissues 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 above-mentioned abnormalities are present in the same arm or leg. Other parts of the body may also be affected.<\/p>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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Symptoms<\/h2>

Children with this syndrome typically have two or more abnormalities in one or more limbs (arms or legs): varicose veins, port-wine stains, and abnormal growth of soft tissues and bones. The port-wine stain is often visible from birth. The varicose veins and abnormal growth usually develop later, often at a young age. The port-wine stain is most commonly located on an arm or leg, but it can also appear on the hip, abdomen, buttocks, shoulders, chest, or back.<\/p>

Due to excessive growth, there may be a difference in length and\/or thickness between the two legs or arms. It also occurs that children have more than ten fingers or toes (polydactyly) or that fingers or toes are fused together (syndactyly). The severity of symptoms varies from patient to patient.<\/p>

Treatment<\/h2>

Klippel-Trenaunay syndrome cannot be cured. However, the symptoms can be reduced through specific treatments. Laser therapy can help reduce the appearance of port-wine stains. Treating varicose veins in people with Klippel-Trenaunay syndrome is more challenging than in those with regular varicose veins. Therefore, compression stockings are usually the preferred option.<\/p>

A difference in leg length can lead to posture problems and back pain, and is treated with a heel lift or surgery.
This syndrome is classified as a complex combined malformation because it involves a capillary-venous malformation, in which lymphatic vessels may also be involved (see ISSVA classification<\/a>).<\/p>

KTS & other vascular malformations \u2026 about genetics and semantics<\/h2>

Vascular anomaly syndromes with eponyms such as Sturge-Weber syndrome and Klippel-Trenaunay syndrome, were described approximately 100 years ago. At that time,\u00a0 diagnoses were
primarily based on clinical observations, as imaging studies were\u00a0limited and knowledge of genetics was not as advanced as it is\u00a0today. Relying solely on clinical examination made achieving an\u00a0accurate diagnosis challenging then and continues to pose\u00a0difficulties today.
Currently, many vascular anomaly syndromes can be characterized\u00a0genetically, with genetics as the most important clue to come to a\u00a0diagnosis. Despite this advancement, eponyms continue to be\u00a0used. Increasingly, these eponyms are associated with genetic\u00a0diagnoses, offering a new perspective on the naming and genetic\u00a0classification of disorders. This evolution brings the possibility of\u00a0targeted therapeutic options, suggesting that completely \u2018saying\u00a0goodbye\u2019 to well-known and frequently used eponyms might not\u00a0be desirable yet.<\/p>

Link to full article<\/a><\/p>

Articles & videos<\/h2>

Articles<\/h3>

Klippel-Trenaunay syndrome or not? An exploration of atypical presentations<\/a><\/p>

Phakomatosis pigmentovascularis type IIb with Klippel-Trenaunay syndrome: Association with GNAQ mutation in vascular endothelial cells<\/a><\/p>

A rare case of Klippel-Trenaunay syndrome presenting with chronic myeloid leukemia<\/a><\/p>

Application of endovascular microwave ablation in infants: A new treatment consideration for Klippel-Trenaunay syndrome<\/a><\/p>

Targeted next-generation sequencing for detection of PIK3CA mutations in archival tissues from patients with Klippel-Trenaunay syndrome in an Asian population : List the full names and institutional addresses for all authors<\/a><\/p>

Klippel-Trenaunay syndrome and pregnancy: A Case-Report<\/a><\/p>

The Prospective Natural History Study of Patients with Intractable Venous Malformation and Klippel-Trenaunay Syndrome to Guide Designing a Proof-of-Concept Clinical Trial for Novel Therapeutic Intervention<\/a><\/p>

Options in the treatment of superficial and deep venous disease in patients with Klippel-Trenaunay syndrome<\/a><\/p>

A Patient with Klippel-Trenaunay Syndrome and Mild Ophthalmic Manifestations<\/a><\/p>

Deep Vein Thrombosis in Klippel-Tr\u00e9naunay Syndrome<\/a><\/p>

Ophthalmic Alterations in the Sturge-Weber Syndrome, Klippel-Trenaunay Syndrome, and the Phakomatosis Pigmentovascularis: An Independent Group<\/a> of Conditions?<\/a><\/p>

Ophthalmic Alterations in the Sturge-Weber Syndrome, Klippel-Trenaunay Syndrome, and the Phakomatosis Pigmentovascularis: An Independent Group of Conditions?<\/a><\/p>

Management of pregnant woman with Klippel-Trenaunay syndrome: A rare and complex situation, about a case report<\/a><\/p>

Klippel-Trenaunay syndrome with gastrointestinal involvement and portal hypertension-evaluation and management<\/a><\/p>

A neonate with Klippel-Tr\u00e9naunay syndrome<\/a><\/p>

Breast Hemangioma: Unique Presentation in a Patient with Klippel- Trenaunay-syndrome<\/a><\/p>

Options in the treatment of superficial and deep venous disease in patients with Klippel-Trenaunay syndrome<\/a><\/p>

Klippel-Trenaunay syndrome with gastrointestinal involvement and portal hypertension-evaluation and management<\/a><\/p>

Management of pregnant woman with Klippel-Trenaunay syndrome: A rare and complex situation, about a case report<\/a><\/p>

Sirolimus for neonatal Klippel-Trenaunay syndrome with chylothorax<\/a>Chiari I malformation with Klippel-Trenaunay syndrome: case report and review of the literature<\/a><\/p>

Gastrointestinal bleeding with Klippel-Trenaunay syndrome: a case report<\/a><\/p>

Is there a place for prophylaxis with DOACs in Klippel-Trenaunay syndrome and other low-flow vascular malformations with intravascular coagulopathy and thromboembolic events?<\/a><\/p>

Breast Hemangioma: Unique Presentation in a Patient with Klippel- Trenaunay-syndrome<\/a><\/p>

Intraneural hemangioma in Klippel-Trenaunay syndrome: role of musculo-skeletal ultrasound in diagnosis-case report and review of the literature<\/a><\/p>

Diagnosis and management of the venous malformations of Klippel-Tr\u00e9naunay syndrome<\/a><\/p>

Ophthalmic Alterations in the Sturge-Weber Syndrome, Klippel-Trenaunay Syndrome, and the Phakomatosis Pigmentovascularis: An Independent Group of Conditions?<\/a><\/p>

Klippel-Trenaunay syndrome with anorectum involvement and portal hypertension<\/a><\/p>

Management of postpartum haemorrhage in a patient with Klippel-Tr\u00e8naunay syndrome<\/a><\/p>

Klippel-Tr\u00e9naunay syndrome<\/a><\/p>

Diffuse capillary malformation with overgrowth contains somatic PIK3CA variants A cross-sectional survey of long-term outcomes for patients with diffuse capillary malformation with overgrowth Wilms tumor screening in diffuse capillary malformation with overgrowth and macrocephaly-capillary malformation: A retrospective study<\/a><\/p>

Safety and efficacy of low-dose PI3K inhibitor taselisib in adult patients with CLOVES and Klippel-Trenaunay syndrome (KTS): the TOTEM trial, a phase 1\/2 multicenter, open-label, single-arm study<\/a><\/p>

Sirolimus for neonatal Klippel-Trenaunay syndrome with chylothorax<\/a><\/p>

Tracheal Varicose Veins Associated with Klippel-Trenaunay Syndrome<\/a><\/p>

A neonate with Klippel-Tr\u00e9naunay syndrome: a case report<\/a><\/p>

Emerging Role of Sirolimus in the Treatment of Diffuse Venous Malformation in a Woman with Klippel-Trenaunay Syndrome<\/a><\/p>

Large abdominal cutaneous bypass that developed naturally in Klippel-Tr\u00e9naunay syndrome<\/a><\/p>

Safety and efficacy of low-dose PI3K inhibitor taselisib in adult patients with CLOVES and Klippel-Trenaunay syndrome (KTS): the TOTEM trial, a phase 1\/2 multicenter, open-label, single-arm study<\/a><\/p>

Tracheal Varicose Veins Associated with Klippel-Trenaunay Syndrome<\/a><\/p>

A neonate with Klippel-Tr\u00e9naunay syndrome: a case report<\/a><\/p>

Intralesional Bleomycin Injection in Management of Localized Venous Problems in Children with Klippel-Trenaunay Syndrome<\/a><\/p>

Cerebral and spinal cavernomas associated with Klippel-Trenaunay syndrome: case report and literature review<\/a><\/p>

Emerging Role of Sirolimus in the Treatment of Diffuse Venous Malformation in a Woman with Klippel-Trenaunay Syndrome<\/a><\/p>

Large abdominal cutaneous bypass that developed naturally in Klippel-Tr\u00e9naunay syndrome<\/a><\/p>

Orthostatic intolerance with Klippel-Trenaunay syndrome<\/a><\/p>

Pregnancy with Klippel-Trenaunay syndrome<\/a><\/p>

Intraneural hemangioma in Klippel-Trenaunay syndrome: role of musculo-skeletal ultrasound in diagnosis-case report and review of the literature<\/a><\/p>

Diagnosis and management of the venous malformations of Klippel-Tr\u00e9naunay syndrome<\/a><\/p>

Ophthalmic Alterations in the Sturge-Weber Syndrome, Klippel-Trenaunay Syndrome, and the Phakomatosis Pigmentovascularis: An Independent Group of Conditions?<\/a><\/p>

Letter: Spinal Neurovascular Malformations in Klippel-Trenaunay Syndrome: A Single Center Study<\/a><\/p>

In Reply: Spinal Neurovascular Malformations in Klippel-Trenaunay Syndrome: A Single Center Study<\/a><\/p>

Extensive Cellulitis and Bacteremia Due to Streptococcus Pseudoporcinus in a Child With Klippel-Trenaunay Syndrome<\/a><\/p>

Tracheal Varicose Veins Associated with Klippel-Trenaunay Syndrome<\/a><\/p>

A neonate with Klippel-Tr\u00e9naunay syndrome: a case report<\/a><\/p>

Intralesional Bleomycin Injection in Management of Localized Venous Problems in Children with Klippel-Trenaunay Syndrome<\/a><\/p>

Emerging Role of Sirolimus in the Treatment of Diffuse Venous Malformation in a Woman with Klippel-Trenaunay Syndrome<\/a><\/p>

Large abdominal cutaneous bypass that developed naturally in Klippel-Tr\u00e9naunay syndrome<\/a><\/p>

Orthostatic intolerance with Klippel-Trenaunay syndrome<\/a><\/p>

Mechanochemical Endovenous Ablation of Varicose Veins in Pediatric Patients with Klippel-Tr\u00e9naunay Syndrome: Feasibility, Safety, and Initial Results<\/a><\/p>

Case Series of Concomitant Klippel-Trenaunay Syndrome and May-Thurner Syndrome<\/a><\/p>

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

Potential Utilization of Lymphoscintigraphy in Patients With Klippel-Trenaunay Syndrome<\/a><\/p>

Intraneural hemangioma in Klippel-Trenaunay syndrome: role of musculo-skeletal ultrasound in diagnosis-case report and review of the literature<\/a><\/p>

Diagnosis and management of the venous malformations of Klippel-Tr\u00e9naunay syndrome<\/a><\/p>

Ophthalmic Alterations in the Sturge-Weber Syndrome, Klippel-Trenaunay Syndrome, and the Phakomatosis Pigmentovascularis: An Independent Group of Conditions?<\/a><\/p>

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

Potential Utilization of Lymphoscintigraphy in Patients With Klippel-Trenaunay Syndrome<\/a><\/p>

Spinal Neurovascular Malformations in Klippel-Trenaunay Syndrome: A Single Center Study<\/a><\/p>

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

Sclerotherapy treatment of a large venous malformation invading the bladder wall related to Klippel-Trenaunay syndrome<\/a><\/p>

Large abdominal cutaneous bypass that developed naturally in Klippel-Tr\u00e9naunay syndrome<\/a><\/p>

Inverse Klippel-Trenaunay<\/a><\/p>

National Organization for Rare Diseases USA<\/a><\/p>

VideosISSVA classification<\/a><\/h3>

Klippel-Trenaunay video<\/a><\/p>

Bron:\u00a0https:\/\/www.huidhuis.nl\/huidaandoening\/syndroom-van-klippel-trenaunay<\/a><\/em><\/p>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t","protected":false},"excerpt":{"rendered":"

KTS-Folder The medical term for Klippel-Trenaunay syndrome is angio-osteohypertrophy syndrome. Klippel-Trenaunay syndrome has three main characteristics: varicose veins, port-wine stains, and abnormal growth of soft tissues or bones. Soft tissues are the tissues located under the skin, around the organs and bones, or in the spaces between them. The diagnosis is made when at least […]<\/p>\n","protected":false},"author":2,"featured_media":3460,"parent":72878,"menu_order":9,"comment_status":"closed","ping_status":"closed","template":"aw-modules\/awcf_create_page_templates_with_elementor_content\/page-templates\/page-aw-page-template-67351.php","meta":{"_acf_changed":false,"footnotes":""},"class_list":["post-463","page","type-page","status-publish","has-post-thumbnail","hentry"],"acf":[],"yoast_head":"\nKlippel-Trenaunay - CMTC-OVM<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.cmtc.nl\/en\/pedia\/conditions\/klippel-trenaunay\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Klippel-Trenaunay - CMTC-OVM\" \/>\n<meta property=\"og:description\" content=\"KTS-Folder The medical term for Klippel-Trenaunay syndrome is angio-osteohypertrophy syndrome. 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