Klippel and Trenaunayʼs Syndrome

Abstract

Since 1945, we have operated on 786 patients with Klippel and Trénaunay's syndrome. Elongation of the impaired limb was invariably found while edema was present in 84%, varicose veins in 36%, and flat angiomata in 32%. Venography and surgical exploration have demonstrated malformation of the deep veins involving the popliteal vein in 51%; superficial femoral vein, 16%; both popliteal and superficial femoral veins; 29%; iliac veins, three per cent; and lower vena cava, one per cent. Good clinical results have been achieved following the surgical release of these deep veins in the lower limb. During childhood, when the difference in limb length is noteworthy, ligature of the popliteal vein of the shorter limb induces a compensating elongation. Klippel and Trénaunay's syndrome may be associated with lymphatic malformations, including lymphedema and malformation of the lymph vessels. Knowledge of the pathophysiology of these malformations of the deep veins enables a better understanding of the clinical manifestations of the condition, as well as the improved treatment of the serious vesical or rectal hemorrhage which occurs in one per cent of these patients.

Klippel-Trenaunay Syndrome and Spinal Arteriovenous Malformations: An Erroneous Association

“…30 KTS typically occurs in the lower extremity and may occasionally be bilateral or affect the upper extremity. 31 Unlike the Parkes Weber syndrome, arteriovenous communications are not a feature of KTS. 32,33 Unfortunately, KTS has often been used as a generic diagnosis referring to a heterogeneous group of vascular anomalies with overgrowth.…”

“…The triple eponym Klippel-Trenaunay-Weber syndrome should thus be abandoned. On a review of large cohort of 786 patients with KTS, Servelle 31 distinguished between the pattern of venous ectasia noted in Parkes Weber syndrome, which is due to arteriovenous shunts, and the inherent venous anomalies of KTS. Recently, some patients with a clinical pattern of Parkes Weber syndrome have been found to have a mutation in the RASA1 gene (CM-AVM).…”

Objawy wstrząsu hipowolemicznego podczas wprowadzenia do znieczulenia dziewczynki z rozległą malformacją naczyniową — niepożądane następstwo zastosowania propofolu i sewofluranu?

“…Dopiero wtedy możliwa jest próba przewidzenia zagrażających powikłań i wybór optymalnej metody znieczulenia dla danego chorego. Kolejny problem to współistnienie anomalii naczyniowych z innymi zaburzeniami i wadami, tworzące zespoły chorobowe, takie jak zespół Klippel-Trenaunaya [1], czy zespół Sturge-Webera. W takich przypadkach o doborze metody znieczulenia decydują także schorzenia towarzyszące [2,3].…”

Klippel Trenaunay Syndrome, Inverse Klippel Trenaunay Syndrome: Hypertrophy of Lower Limbs and Atrophy of the Upper Limbs and Facial Muscles: Case Report and Literature Review

“…The prevalence of deep venous aplasia or hypoplasia, as detected with venographic techniques (ascending venography and varicography), ranges from 18% [27] to 40% [26]. Lymphatic malformations have also been common in up to 70% of cases [1], that include primary lymphaedema, cystic hygroma or lymphangiectasia associated with reflux of chyle [29].…”

“…The cutaneous vascular lesion is generally a capillary malformation and usually involves the enlarged limb, although involvement of the whole side of the body or of the contralateral limb may be seen [9,27].In addition, other bone deformities can be seen such as macrodactyly, syndactyly, split hand deformity, phalangeal agenesis and hip joint dislocation [35]. Soft tissue hypertrophy may be limited to a localized mass on the back or chest, or it can be diffuse involving an entire arm or leg [29].…”