Klippel–Trénaunay Syndrome - a Rare Entity (original) (raw)
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Klippel–Trénaunay Syndrome – A Very Rare and Interesting Syndrome
Clinical Medicine Insights: Circulatory, Respiratory and Pulmonary Medicine, 2015
Klippel–Trénaunay syndrome (KTS or KT) is an infrequently seen dermatological syndrome, which is often viewed as a triad of vascular malformation (capillary malformations or port-wine brands), venous varicosity, and soft tissue and/or bony hypertrophy. We report a case of a 12-year-old male who presented to us with the symptoms of varicose plaques over both lower limbs and was diagnosed as a case of KTS. Management is normally conservative and includes stockings for compression of the branches to reduce edema because of chronic venous insufficiency; modern devices that cause on and off pneumatic compression; and rarely, surgical correction of varicose veins with lifelong follow-up. The orthopedic abnormalities are treated with epiphysiodesis in order to prevent (stop) overgrowing of limb and correction of bone deformity.
Egyptian Journal of Radiology and Nuclear Medicine
Background: We present a series of three cases of Klippel-Trenaunay syndrome (KTS) presenting with unilateral lower limb involvement demonstrating a wide range of radiological findings. Case presentation: Case 1: A 61-year-old male presented with varicosities in the right lower limb, which was atrophic with a complete absence of deep venous system in the right limb. Case 2: A 23-year-old woman presented with hypertrophied left lower limb with multiple varicosities showing unusual distribution and intraosseous extension into the distal femur, patella, and tibia. Case 3: A 14-year-old male presented with hypertrophy of the right lower limb overlying macular cutaneous lesions. Capillary malformation was seen underneath the cutaneous lesion and multiple subcutaneous and intramuscular venous malformations were seen. Also noted was persistent embryological vein, i.e., lateral marginal vein of Servelle. Conclusion: The three cases in our series demonstrate common and unusual findings of KTS and highlight the role of CT and MR venography in addition to color Doppler sonography to identify the complete extent of the involvement.
Clinical and imaging findings of klippel-trenaunay syndrome
International journal of health & medical sciences, 2022
Klippel-Trenaunay syndrome (KTS) is characterized by the classic triad of capillary malformations manifesting as a "port-wine stain", venous varicosities, and bone and/or soft tissue hypertrophy. Imaging could help to diagnose and evaluation of KTS. Case Presentation: We report a 12-year-old with progressive enlargement of the right lower limb with geographic stain and venous varicosities. The patient also underwent MRI and angiography, which revealed multiple nidus with multiple feeding arteries. Conclusion: Klippel-Trenaunay Syndrome is a rare condtion. MRI and Arteriography could help diagnose KTS for confirmation, monitoring progression, or identification of complications.
The Klippel-Trenaunay syndrome (KTS): A case report in an infant
Journal of Rawalpindi Medical College
Background: Klippel-Trenaunay syndrome (KTS) is a rare congenital disease having an incidence of 1/20-40,000, live births. KTS is a congenital, vascular disorder affecting usually one or rarely more than one limbs. It is characterized by port wine stain (capillary malformation), limb hemihypertrophy and other vascular anomalies. Case presentation: We present a case of this rare disorder in an infant who presented at an early stage of this disorder. The index case presented to us with a port wine stain and soft tissue edema of right lower limb. The Doppler scan showed venous malformation of right leg. The diagnosis was made on the basis of clinical and radiological criteria. Conclusion: The case is being reported in order to increase awareness among medical professionals.
Surgical treatment of venous malformations in Klippel-Trénaunay syndrome
Journal of Vascular Surgery, 2000
Purpose: Klippel-Trénaunay syndrome (KTS) is a complex congenital anomaly, characterized by varicosities and venous malformations (VMs) of one or more limbs, port-wine stains, and soft tissue and bone hypertrophy. Venous drainage is frequently abnormal because of embryonic veins, agenesis, hypoplasia, valvular incompetence, or aneurysms of deep veins. We previously reported on the surgical management of KTS. In this article, we update our experience. Methods: Twenty patients with KTS underwent surgical treatment for VMs between July 1, 1987, and January 1, 2000. This group represented 6.9% of 290 patients with KTS who were seen at our institution during this 12.5-year study period. Surgical indications, venous anatomy (determined with duplex scan, contrast phlebography, magnetic resonance imaging or magnetic resonance phlebography), operative procedures, and complications were reviewed, and outcomes were recorded. Results: Twelve male and eight female patients (mean age, 23.4 years; range, 7.7-40.6 years) underwent 30 vascular surgical procedures in 21 lower limbs. All 20 patients (100%) had varicose veins or VMs, 13 (65%) had port-wine stains, and 18 (90%) had limb hypertrophy. Pain was the most common complaint, which was present in 16 patients (80%), followed by swelling in 15 (75%), bleeding in 8 (40%), and superficial thrombophlebitis and cellulitis in 3 (15%). Imaging confirmed patent deep veins in 18 patients, hypoplastic femoral vein in 1, and entrapped popliteal veins bilaterally in 1. Four patients (20%) had large persistent sciatic veins (PSVs). The CEAP clinical classification was C-3 for 17 patients (85%), C-4 for 1 patient (5%), and C-6 for 2 patients (10%). Stripping of large lateral veins, avulsion, and excision of varicosities or VMs were performed on all limbs. Three patients required staged resections. The release of entrapped popliteal veins was performed in both limbs of one patient; another underwent a popliteal-saphenous bypass graft. One patient underwent excision of a PSV. Open and endoscopic perforator vein ligation was performed in one patient each. Two patients (12%) had hematomas that required evacuation. No patients had caval filter placement; none had postoperative deep venous thrombosis or pulmonary embolus. The mean follow-up was 63.6 months (range, 0-138 months). All patients reported initial improvement, but some varicosities recurred in 10 patients (50%), an ulcer did not heal in one, and a new ulcer developed in one, 8 years after surgery. Three patients underwent reoperation for recurrent varicosities. Follow-up CEAP scores were C-2 in 10 patients (50%), C-3 in 6 patients (30%), C-4 and C-5 in 1 patient each (5%), and C-6 in 2 patients (10%). Clinical scores improved from 4.3 ± 2.2 to 3.1 ± 2.3. (P = .03).
A Series of Cases of Klippel Trenaunay Syndrome with Unusual Presentations
IOSR Journals , 2019
Klippel trenaunay syndrome (KTS) is characterized by triad of portwine stain, venous varicosities and bone and soft tissue hypertrophy. It is not commonly seen in dermatological practise. Hypertrophy and vascular malformations are usually confined to single limbs. We report a series of 2 cases with different presentations and they attended to our OPD in a span of 6 months from June 2018 to December 2018. Management is usually conservative and surgical management for varicosities with lifelong follow-up.
A rare presentation of Klippel-Trenaunay syndrome
Indian Dermatology Online Journal, 2014
The Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by capillary malformation, varicosities and bony and soft tissue hypertrophy. This disease has several morbidities like bleeding, deep venous thrombosis, embolic complications and in some cases enlargement of limb that may require amputation. Vascular malformations are segmented and never cross midline. However, we came across a case, a 45-year-old male, who presented with varicosity of veins and deformity of left lower limb besides cavernous hemangiomas (port-wine stains) scattered all over his face, chest, back, gluteal region, groin and legs since birth. Multiple paravertebral soft tissue masses and bladder hypertrophy were also noted due to involving neurofibromatosis. Simultaneous occurrence of KTS and neurofibromatosis is rarely seen in clinical practice.
Klippel Trenaunay Syndrome: Report of a Rare, Mild Form of Syndrome
Journal of Evolution of medical and Dental Sciences, 2012
Klippel Trenaunay Syndrome (KTS) is a congenital vascular disease characterized by malformations of capillary, venous and lymphatic vessels with bony and soft tissue hypertrophy. KTS is a sporadic disease with unknown etiology, and there is no predilection for gender or any particular ethnicity. The disease appears more frequently at birth, childhood or adolescence. It generally affects only one extremity and lesions are present at birth or appear by the age of 12 years. Clinical presentation of this syndrome is protean ranging from minimal asymptomatic disease to life threatening bleeding and embolism. Management of this syndrome includes careful diagnosis, prevention and treatment of complications. We report a case of KTS in a 25 year old female who presented with mild form of disease.