Klippel trenaunay

INTRODUCTION: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital systemic disease characterized by a classic typical triad: cutaneous haemangioma, arterio-venous fistulas or varicosities (or both) and unilateral hypertrophy of hard and soft tissue with different localizations. First described by the French physicians Marcel Klippel and Paul Trenaunay, in 1900, KTWS has a clinically incidence of 2-5/100000. The complete triad (port-wine stains, varicose veins, and soft tissue and/or bony hypertrophy) occurs in almost 2/3 of the patients...

Skeletal remains of two prehispanic male adult individuals (antiquity ≈ 550 BP) recovered from a burial cave located in Montaña Blanca (Las Cañadas del Teide) at an altitude of 2450 m above sea level, in the highlands of Tenerife (Canary Islands) showed some unusual features. Femora and tibiae of both individuals showed increased bone density, with irregular thickening of the midshaft diaphyses. One individual showed a cystic lesion in the distal third of the left femoral diaphysis, surrounded by a subtle sclerotic reaction of the spongiosa and a thin cortex that was partially fractured...

INTRODUCTION AND IMPORTANCE: Servelle-Martorell syndrome (SMS) is a rare congenital anomaly that is frequently mistaken for Klippel-Trenaunay syndrome (KTS) or Parkes-Weber syndrome (PWS). SMS usually involves venous dilatations, soft tissue hypertrophy, and bone hypotrophy, while KTS and PWS usually have bone hypertrophy. The management of SMS is primarily conservative, and surgery should be done selectively. This study aimed to report a case of SMS and its management to relieve a painful aneurysm on the right knee by excision...

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterized by a combination of capillary malformations, soft-tissue or bone hypertrophy, and varicose veins or venous malformations. The syndrome predisposes patients to hypercoagulable states, including venous thromboembolism and pulmonary embolism (PE). CASE SUMMARY: A 12-year-old girl with KTS was scheduled excision of verrucous hyperkeratosis in the left foot and posterior aspect of the left leg and left thigh and excision of a cutaneous hemangioma in the right buttock...

Klippel-Trénaunay syndrome (KTS) is a rare and complex congenital syndrome defined as the triad of cutaneous capillary malformation, bone and soft tissue hypertrophy, and venous and lymphatic malformations. KTS is thought to be due to a somatic mutation in phosphatidyl-inositol 3 kinase. It belongs to a group of syndromes termed the PI3CA-Related Overgrowth Spectrum (PROS) disorders. Because of the rarity and clinical heterogeneity of these disorders, management is patient specific, and best evidence guidelines are lacking...

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INTRODUCTION: Klippel-Trenaunay Syndrome (KTS) is a rare genetic disorder which is characterised by vascular nevi, venous varicosity, and hyperplasia of soft tissue or bone. Renovascular involvement is considered uncommon in KTS. PRESENTATION OF CASE: A 79-year-old man presented with a left sided varicocele, lymphedema, hydrocele, and microscopic haematuria. After a series of investigations his imaging and clinical features were suggestive KTS. His images, which showed a 2...

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BACKGROUND: We report the anesthetic management of an external iliac artery transection in a morbidly obese patient with Klippel-Trenaunay-Weber syndrome (KTWS). CASE PRESENTATION: A 47-year-old man with KTWS was scheduled for a right external iliac artery transection. Preoperative CT showed a right external iliac artery aneurysm, a right superficial femoral artery aneurysm, and developed collateral vessels. General anesthesia was maintained with desflurane, remifentanil, and rocuronium bromide...

Klippel-Trenaunay syndrome is a rare congenital anomaly that is associated with abnormalities in the deep venous system. Operative intervention is often used only when patients fail conservative management for chronic venous insufficiency. We present a case of a deep venous abnormality requiring a saphenous vein crossover Palma procedure, in combination with a left femoral arteriovenous PTFE fistula to manage a non-healing wound from chronic venous insufficiency in a 22-year-old man. This case highlights updates for modern treatment tips for technical and medical management decisions to avoid early graft thrombosis...

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is an overgrowth syndrome associated with capillary/venous/ lymphatic malformations with limb hypertrophy and cancer risk. Various cancers, mostly Wilms tumor, have been reported in patients with KTS, but not leukemia. Chronic myeloid leukemia (CML) is also a rare disease in children, where there is no known disease or syndrome to predispose to CML. CASE: We report a case of CML incidentally diagnosed in a child with KTS when he was bleeding from surgery of the left groin for vascular malformation...

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a congenital vascular malformation with a complicated etiology. It is sporadic and clinically rare in occurrence. The typical characteristics are capillary malformation (also known as port-wine stain), varicose veins and malformations, and bony and/or soft tissue hypertrophy with or without lymphatic malformation, which are known as the "classic clinical triad". Herein, a rare case of KTS characterized by crossed-bilateral limb hypertrophy accompanied by intermittent hematochezia and hematuria is reported...

Abdominal wall and spinal soft tissue findings are frequently encountered on CT or MR imaging of the abdomen and pelvis. Many of these entities have specific imaging findings, for which a definitive diagnosis can be made without the need for further work up. These abdominal wall and spinal findings may be diagnostically challenging for sub-specialized abdominal radiologists who are unfamiliar with their appearance and appropriate management. This review article describes and illustrates pathognomonic or characteristic abdominal wall and spinal pathologies, which reside outside the abdominopelvic cavity...

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INTRODUCTION: The etiology of Klippel-Trenaunay syndrome (KTS) has a significant impact on the management of patient requiring surgical interventions. We report the strategies employed to address an infected non-union in a patient with KTS. CASE REPORT: The patient was transported to an initial treating hospital with femoral shaft fracture after experiencing a fall. The patient was diagnosed with KTS due to vascular malformations identified after severe intraoperative hemorrhaging...

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BACKGROUND: Capillary lymphatic venous malformations (CLVM) and associated syndromes, including Klippel-Trenaunay syndrome (KTS) and congenital lipomatous overgrowth, vascular malformation, epidermal nevi, skeletal, and spinal syndrome (CLOVES), are underrecognized disorders associated with high morbidity from chronic pain, recurrent infections, bleeding, and clotting complications. The rarity of these disorders and heterogeneity of clinical presentations make large-scale randomized clinical drug trials challenging...

Cystic lymphatic malformations (LMs) are rare chronic conditions which management differs according to the type (macrocystic LMs, microcystic LMs or both). Studies are lacking due to rarity of the pathology. We aimed to establish a French National Diagnosis and Care Protocol (PNDS: Protocole National de Diagnostic et de Soins), to provide health professionals with free open access synthesis on optimal management and care of patients with LMs ( https://www.has-sante.fr/upload/docs/application/pdf/2021-03/malformations_lymphatiques_kystiques_-_pnds...

A fracture of the affected extremity in patients with Klippel-Trenaunay-Weber syndrome can be fatal due to massive bleeding and show poor results. A 42-year-old male presented with an old fracture of the right femoral shaft with metal failure. We planned an operation to remove the previously fixed plate and to perform re-fixation using an intra-medullary nail. Preoperative angiography was performed and the arteriovenous malformations were embolized in order to reduce the risk of bleeding. After angiography, the previously fixed plate was removed...