ACR Appropriateness Criteria® Clinically Suspected Vascular Malformation of the Extremities

Obara, Piotr; McCool, Justin; Kalva, Sanjeeva P.; Majdalany, Bill S.; Collins, Jeremy D.; Eldrup-Jørgensen, Jens; Ganguli, Suvranu; Gunn, Andrew J.; Kendi, Ayse T.; Khaja, Minhajuddin S.; Sutphin, Patrick D.; Vijay, Kanupriya; Dill, Karin E.

doi:10.1016/j.jacr.2019.05.013

Cited by 14 publications

(3 citation statements)

References 24 publications

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“…KTS is often diagnosed clinically with imaging modalities used to confirm the diagnosis [2]. Ultrasound and MRI of the involved lower extremity are recommended as the initial imaging for suspected KTS [4]. A biopsy is not typically necessary to confirm the diagnosis [4].…”

Section: Discussionmentioning

confidence: 99%

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Klippel-Trenaunay Syndrome With Atypical Presentation of Small Port-Wine Stain

Alazawi¹,

Wright²

2022

Cureus

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Klippel-Trenaunay Syndrome (KTS) is a rare congenital capillary/venous malformation (CVM) disorder associated with deep soft-tissue swelling. A seven-year-old Caucasian male with a history of Klippel-Trenaunay Syndrome presented to the dermatology clinic for evaluation and treatment of mildly edematous right lower extremity and varicose veins with multiple, small port-wine stains and nodules. Typically, cases of KTS report large port-wine stains, where a multidisciplinary approach is the mainstay for management. However, this case involves a clinical presentation of mild KTS with atypical, small port-wine stains on the affected limb.

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Section: Discussionmentioning

confidence: 99%

“…Ultrasound and MRI of the involved lower extremity are recommended as the initial imaging for suspected KTS [4]. A biopsy is not typically necessary to confirm the diagnosis [4]. This syndrome has an estimated prevalence of two to five per 100,000 people worldwide [5].…”

Section: Discussionmentioning

confidence: 99%

Klippel-Trenaunay Syndrome With Atypical Presentation of Small Port-Wine Stain

Alazawi¹,

Wright²

2022

Cureus

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“…Principalmente, su diagnóstico es clínico, sin embargo, con el advenimiento del genotipado de ADN, el KTS puede diagnosticarse específicamente en comparación con el espectro de sobrecrecimiento relacionado con PIK3CA. Se puede realizar una caracterización adicional de la severidad del síndrome con imágenes de resonancia magnética (RM), observando específicamente las alteraciones óseas, de los tejidos blando, las venosas, y linfáticas [5]. Dadas las alteraciones inherentes y específicas de cada paciente, el manejo debe ser multidisciplinario, que asegure el cuidado con medidas de soporte, prevención de coagulopatías e infecciones, y la corrección quirúrgica de la hiperplasia del tejido anómalo.…”

unclassified

Manejo de la paciente gestante con síndrome de Klippel Trenaunay

Daza¹,

Parra²,

Camacho³

et al. 2022

Rev. chil. anest.

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Klippel-Trenaunay Weber syndrome is a congenital vascular disease of unknown cause, in which the blood and/or lymphatic vessels do not form correctly. It is characterized by a triad of symptoms: port wine stains that cover one or more limbs, congenital vascular anomalies, usually varicose veins, absence or duplication of a venous structure, malformation or hypertrophy. Objective: To make an update on the Klippel-Trenaunay Weber Syndrome.

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