Klippel-Trenaunay-Weber syndrome: Oro-dental manifestations and management

Daume, L.; Renz, C.; Trento, G.; Kleinheinz, J.

doi:10.1016/j.ijscr.2023.108690

Cited by 1 publication

(3 citation statements)

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“…Soft tissue overgrowth and connected deformations can sometimes affect the exterior genital area, where it can result in cryptorchidism, phimosis and problems with micturition [12]. Cases of facial soft tissue overgrowth and bone malformations have also been described, mostly in the areas of the mandible and maxilla, which can also be restricted laterally [2].…”

Section: Resultsmentioning

confidence: 99%

“…The last element of the Klippel-Trénaunay triad are portwine stains. There are sources referring to as 'flat angiomata' [8], although most of the newly-published papers prefer the term 'port-wine stains' while describing haemangiomas that do not shrink with age and (in most cases) cover the hypertrophic limb, gluteal region of the same side if the lower limb is affected, and the trunk -both anterior and posterior sides of the corpus can be affected [11]), and sometimes even the neck with the unilateral stain formation on both limbs and trunk [2]. They can appear in 98% of patients according to one study [9] or 32% in the other cases, and referred to as the previously 'flat angiomata' [8].…”

Section: Resultsmentioning

confidence: 99%

“…Klippel-Trénaunay Syndrome (KTS) is a rare congenital disease, occurring with the frequency of 2-5/100 000 births, regardless of gender [1][2][3]. It is characterised by the presence of vascular malformations, mostly located on the limbs.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Diversity and frequency of symptoms in Klippel–Trénaunay syndrome

Potępa,

Małyska,

Samczuk

et al. 2024

J Pre Clin Clin Res.

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Introduction and Objective. Klippel-Trénaunay syndrome is a rare congenital disease consisting of vascular malformations, limb overgrowth, large varicose veins and port-wine stains. It is related to the mosaic variants of PIK3CA gene. Patients with KTS usually receive symptomatic treatment, such as orthopaedic correction of limb overgrowth or varicose vein treatment. This systematic review aims to summarise knowledge about the most common of anomalies presented by Klippel-Trénaunay patients. The literature review describes the anomalies present in Klippel-Trénaunay syndrome. Review Methods. This review was created based on 21 papers found in PubMed and PubMedCentral databases after searching for'klippel trenaunay' and 'hyperthrophy/anomaly/anatomical/malformation', published between 1978-2023, as well as on Dove Press articles from 2012. Brief description of the state of knowledge. Limb overgrowth is a dominant symptom of KTS, appearing in 67% -100% of patients. More often it affects lower limbs, although it can also appear in the upper limbs. Overgrowth is caused by vascular malformations which, on their own, are another symptom of KTS. Patients with KTS present malformations of deep veins (e.g. popliteal vein) or superficial veins (e g. superficial femoral vein), together with embryonal veins (e.g. persisting sciatic vein). Port-wine stains can appear to up to 98% of patients, and are located mostly on the lower limbs and/or torso. Summary. The variety of anomalies appearing in Klippel-Trénaunay syndrome is large and diversified. Collecting and summing-up information about anatomical anomalies of Klippel-Trénaunay syndrome can help both doctors and patients in the search for more meticulous diagnosis and treatment of the symptoms.

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Section: Resultsmentioning

confidence: 99%