Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal

Karmacharya, Robin Man; Vaidya, Satish; Bhatt, Swechha; Tamang, Ashish; Shrestha, Rohit Bhasink; Bhandari, Niroj; Paudel, Bijaya; Shah, Manish A.; Nepal, Gaurav

doi:10.1016/j.amsu.2022.103732

Cited by 3 publications

(9 citation statements)

References 20 publications

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“…According to the International Society for the Study of Vascular Anomalies 2018, KTS consists of capillary and venous malformations, limb overgrowth, and lymphatic malformations [4,6,7]. In addition to hyperostosis frontalis interna, rectum and bladder bleeding are other manifestations, which are potentially fatal complications of these malformations and have been reported in less than 5% of cases [8]. The distal colon and rectum are the most frequently reported sites in the gastrointestinal tract.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Klippel-Trénaunay Syndrome

Arasu¹,

Khalil-Khan²,

G³

et al. 2022

Cureus

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The Klippel-Trénaunay syndrome (KTS) is a rare form of a birth disorder that includes capillary malformation, hypertrophy of bones and soft tissues, and tortuous varicosities, as well as hypertrophy of the capillaries resulting in hemangiomas and port wine discoloration. KTS is also known as angioosteohypertrophy syndrome and dysplastic angiopathy. In this case report, we describe the case of a 13year-old female with multiple superficial varicosities on the medial aspect of her left leg since birth. Computed tomography angiogram assessed and identified abnormal venous drainage in the lower limb. Klippel-Trénaunay-Weber syndrome (KTWS) differs from KTS in that KTWS involves arteriovenous malformations.

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Section: Discussionmentioning

confidence: 99%

A Rare Case of Klippel-Trénaunay Syndrome

Arasu¹,

Khalil-Khan²,

G³

et al. 2022

Cureus

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“…Conservative management includes non-invasive measures such as the use of compression stocking, and intake of analgesics, antibiotics and corticosteroids [1] , [4] . Surgery is usually performed for symptomatic cases and is guided by preoperative evaluation of deep venous system using computed tomography (CT) arteriography [1] , [11] .…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…There have been studies of this syndrome in Nepal [4] , however KTS in a child as young as 3 years treated with sclerotherapy has rarely been reported. Literature suggests that endovenous laser ablation adjunct with sclerotherapy is an effective management of KTS in pediatric patients [4] , [10] . However, in our case the lateral marginal vein was tortuous which could have created trouble in insertion of radiofrequency ablation (RFA) catheter.…”

Section: Discussionmentioning

confidence: 99%

“…At least two of the above features should be present to clinically diagnose KTS [3] . It is mostly unilateral involving lower extremities and rarely can involve bilateral lower extremities, upper extremities, trunk and internal organs [4] . The etiology of KTS is not clearly known; however, mesodermal abnormality during fetal development is said to be the cause for maintenance of arteriovenous communication in limb bud [5] .…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Klippel Trenaunay syndrome in a 3-year-old: A case report

Karmacharya

Vaidya

Yadav

et al. 2022

International Journal of Surgery Case Reports

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Large birth mark and unilateral swelling of the lower extremity in a young teenager

Foster,

Roy,

Kumar

2024

BMJ Case Rep

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We describe an early adolescent male who was diagnosed with vascular malformation associated with unilateral limb overgrowth based on the clinical findings of a persistent port-wine stain since birth and gradually progressing right lower limb oedema since early childhood. Clinicians should keep in mind to clinically evaluate such malformations in detail, as well as contemplate genetic testing in patients presenting with a large port-wine stain at birth, particularly if well demarcated and lateral in a lower extremity.

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Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal

Cited by 3 publications

References 20 publications

A Rare Case of Klippel-Trénaunay Syndrome

A Rare Case of Klippel-Trénaunay Syndrome

Klippel Trenaunay syndrome in a 3-year-old: A case report

Large birth mark and unilateral swelling of the lower extremity in a young teenager

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