scholarly journals Klippel–Trénaunay Syndrome – A Very Rare and Interesting Syndrome

Author(s):  
Deepak Sharma ◽  
Sachin Lamba ◽  
Aakash Pandita ◽  
Sweta Shastri

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.

PLoS ONE ◽  
2018 ◽  
Vol 13 (12) ◽  
pp. e0208954
Author(s):  
Heather C. Dwyer ◽  
David C. Baranowski ◽  
Perry V. Mayer ◽  
Simona Gabriele

2019 ◽  
Vol 34 (8) ◽  
pp. 543-551 ◽  
Author(s):  
Tjun Y Tang ◽  
Harsha P Rathnaweera ◽  
Jia W Kam ◽  
Tze T Chong ◽  
Edward C Choke ◽  
...  

Objectives The aim of this prospective single-centre study is to assess the effectiveness and patient experience of the VenaSeal™ Closure System, a novel non-thermal, non-tumescent catheter technique, which uses cyanoacrylate glue to occlude the refluxing truncal superficial veins to treat varicose veins and chronic venous insufficiency, in a multi-ethnic Asian population from Singapore. Methods Seventy-seven patients (93 legs; 103 procedures) underwent VenaSeal™ Closure System ablation. Forty-nine (63.6%) for great saphenous vein incompetence, 16 (20.8%) bilateral great saphenous vein, 2 (2.6%) small saphenous vein and 10 (13.0%) combined unilateral great saphenous vein and small saphenous vein/anterior thigh vein reflux. In addition, 65/93 legs (69.9%) had C4–C6 disease. Patients were reviewed at 2 weeks, 3, 6 and 12 months post-procedure. Results There was 100% technical success. 28/77 (36.4%) underwent concomitant phlebectomies. All procedures were well tolerated with a mean post-operative pain score of 3.0 (range: 0–5). After three months, median patient satisfaction was 9.0 (interquartile range: 7.0–10.0). At two-week follow-up, the great saphenous vein was completely occluded in 88/88 (100%) veins and small saphenous vein completely closed in 11/11 (100%) veins. At three-month follow-up, the great saphenous vein was occluded in 51/53 (96.2%) veins and small saphenous vein completely closed in 5/5 (100%) veins. At six-month follow-up, the great saphenous vein was completely occluded in 42/45 (93.3%) veins and small saphenous vein completely closed in 5/7 (71.4%) veins. At one year, great saphenous vein and small saphenous vein occlusion rates were 54/59 (91.5%) and 5/8 (62.5%), respectively. There was one deep vein thrombosis. Transient superficial phlebitis was reported in 10/93 (10.8%) legs, which were all self-limiting. There were 9/103 (8.7%) anatomical recurrences, but no patients required re-intervention as they were asymptomatic. Conclusions Cyanoacrylate glue is a safe and efficacious modality to ablate refluxing saphenous veins in Asian patients in the short term. There is a high satisfaction rate and peri-procedural pain is low. Early results are promising but further evaluation and longer term follow-up are required.


Ultrasound ◽  
2019 ◽  
Vol 28 (2) ◽  
pp. 91-102
Author(s):  
Olga Ivanitskaya ◽  
Elena Andreeva ◽  
Natalia Odegova

Klippel–Trenaunay syndrome is a rare disease with a classic triad of port wine stains, varicose veins, and bony and soft tissue hypertrophy of an extremity. The quality of life in these patients is significantly affected, making the prenatal diagnosis of Klippel–Trenaunay syndrome important. We present four prenatally diagnosed cases of this anomaly with a unique case of ectrodactyly of the hand in foetus with Klippel–Trenaunay syndrome. Such a combination has not been previously reported prenatally. A review of the literature for similar cases is also presented.


Author(s):  
Balaji Zacharia ◽  
Jittu Alex ◽  
Ashwin Rajmohan

AbstractWe present a case of a 14-year-old girl who was first treated when she was 4 years old. She had progressive limping of the left lower limb from the age of 3 years. She was diagnosed to have developmental coxa vara of the left hip and treated by a subtrochanteric valgus osteotomy of the left femur. Later, she developed hypertrophy of the left upper and lower limbs. There were port-wine stains over the left lower limbs with multiple superficial varicosities. Her diagnosis was Klippel–Trénaunay syndrome (KTS). She is asymptomatic at the final follow-up. Both developmental coxa vara and KTS are rare conditions. We present this case to demonstrate the rarest combination of two rare conditions occurring in the same limb.


Phlebologie ◽  
2002 ◽  
Vol 31 (03) ◽  
pp. 63-68
Author(s):  
E. Jahnová ◽  
P. Labasˇ ◽  
S. Weissová ◽  
M. Horváthová ◽  
M. Ferencík ◽  
...  

SummaryAim: We studied if the expression and release of adhesion molecules is changed in peripheral lymphocytes obtained from patients with chronic venous insufficiency (CVI) of the lower limbs. Methods: 26 patients with CVI (19 primary varicose veins, 7 ulcus cruris) and 39 clinically healthy persons were included in our study. The expression of adhesion molecules on granulocytes, monocytes, and lymphocytes was analysed by flow cytometry. In serum soluble sCD31 and sCD54 was assayed by ELISA, the CD profile was estimated on lymphocytes. Results: In patients suffering from CVI the increased expression of CD11b, CD18, CD54, and CD49d on granulocytes and lymphocytes, of CD62L and CD49d on granulocytes and monocytes, and of CD15 on monocytes and lymphocytes was demonstrated. The highest difference in the expression of adhesion molecules between patients and controls were found on lymphocytes. In patients with CVI lower mean counts of cytotoxic (CD8+) lymphocytes and higher counts of NK cells (CD16+ + CD56+) were seen. The concentration of activated T lymphocytes (CD3 HLA-DR+) and of sCD54 was much higher in the circulation of patients with ulcus cruris than in those with varicose veins or in the controls. Conclusion: Lymphocytes and leukocytes of patients with CVI express elevated concentrations of various adhesion molecules, especially in patients with ulcus cruris. Additionally, significant changes in their CD profile were observed.


2016 ◽  
Vol 4 (1) ◽  
pp. 294
Author(s):  
Krishnappa Santosh Kumar ◽  
Nanjappa Mohan Kumar

Klippel Trenaunay Syndrome (KTS) is a cutaneous vascular malformation in combination with bone and soft tissue overgrowth, with or without lymphatic malformation. Symptoms appear by birth and approximately 90% of the cases are diagnosed by the age of 12 years. Prompt diagnosis at the earliest and conservative management with regular follow up is crucial in prognosis of KTS.


2015 ◽  
Vol 31 (4) ◽  
pp. 264-274 ◽  
Author(s):  
Eberhard Rabe ◽  
Stefania Ballarini ◽  
Lorenz Lehr

Objective To show the superiority of 500 mg calcium dobesilate vs. placebo in reduction of edema of the lower limbs in patients with chronic venous insufficiency, Clinical, Etiological, Anatomical and Pathophysiological classes C3/C4. Methods A total of 351 patients were randomized (n = 174 calcium dobesilate, n = 177 placebo). Active treatment was 500 mg calcium dobesilate, three times daily for 12 weeks, with a 12-week follow-up. Results At the end of treatment, the relative volume change in the most pathological leg was −0.6 ± 4.8% with calcium dobesilate compared to −0.3 ± 3.3% with placebo (p = 0.09). At the end of follow-up, this was −1.01 ± 5.4% for calcium dobesilate vs. −0.08 ± 3.5% for placebo (p = 0.002). Conclusions Calcium dobesilate treatment resulted in no significant volume change in the most pathological leg between baseline and end of treatment. However, the calcium dobesilate group showed a significantly greater volume decrease in the most pathological leg at the end of follow-up. Calcium dobesilate was well-tolerated, with a safety profile consistent with previously published data.


2021 ◽  
pp. 026835552110251
Author(s):  
YL Linn ◽  
CJQ Yap ◽  
SXY Soon ◽  
SL Chan ◽  
VBX Khoo ◽  
...  

Background The Venablock© Venous Closure System (Invamed, Ankara, Turkey) is a novel cyanoacrylate-based non-thermal non-tumescent embolization device to block refluxing truncal veins for chronic venous insufficiency and varicose veins. The aim was to prospectively evaluate the safety and 6 months efficacy of Venablock© for the treatment of primary great saphenous vein (GSV) and small saphenous vein (SSV) incompetency in a multi-ethnic cohort from Singapore. Methods This was a single arm, single investigator prospective study of 29 patients (39 limbs, 39 truncal veins) recruited over a 5-month period (August 2019 to February 2020), who were treated with the Venablock© device at a tertiary vascular unit in Singapore. Patients with symptomatic varicose veins (C2–6) and had truncal reflux > 0.5 second on venous Duplex ultrasound were included. Follow-up occurred at 2 weeks, 3 and 6 months with dedicated quality of life questionnaires and a targeted Duplex ultrasound performed to check for continued venous occlusion. Result Mean age was 61.4 (±11.0) years and mean BMI was 26.2 (±5.7) kg/m2. 11/29 (37.9%) were males. Most common CEAP class treated was 2 (12/29, 41.3%). Mean diameter of treated GSV was 5.7 (±2.0) mm, 4.8 (±1.7) mm and 4.2 (±1.3) mm for the proximal, mid and distal above knee segments respectively. Mean time from access puncture to sheath removal was 23.4 (±10.0) mins. Vein occlusion at 2 weeks, 3 and 6 months was 39/39 (100%), 39/39 (100%) and 36/37 (97.2%) respectively. 5/29 (17.2%) developed puncture site infections, of which 3/29 (7.7%) required formal surgical drainage. 3/29 (7.7%) developed phlebitis. At 6 months, revised Venous Clinical Severity Score improved from 5.2 (±3.5) to 2.1 (±2.9; p < .001); EuroQol-5 Dimension score, from 7.4 (±2.1) to 5.7 (±1.4; p < .001); Aberdeen Varicose Vein Questionnaire score, from 18.1 (±15.5) to 7.9 (±8.9; p = .007); and Chronic Venous Insufficiency Questionnaire, from 18.6 (±16.2) to 4.5 (±6.3; p < .001). Conclusion Venablock© is a safe and efficacious option of treating truncal venous insufficiency in a multi-ethnic Asian cohort from Singapore in the short term. There is a significant improvement in QoL. Longer follow-up is required to assess the durability of this technique, in particular the higher puncture site infection rates observed compared to other glue-based therapies.


2019 ◽  
Vol 10 (2) ◽  
pp. 225-233
Author(s):  
Sarangam Venkata Satya Narayana Rao ◽  
◽  
Palaka Srinivasa Rao ◽  
K. Phani Jotsna ◽  
U. Grace Lalitha Priya ◽  
...  

2020 ◽  
Vol 19 ◽  
Author(s):  
Fanny Rodríguez Santos ◽  
Victoria Loson ◽  
Agustín Coria ◽  
Hugo Martínez

Abstract Klippel-Trenaunay syndrome (KTS) is a rare vascular malformation characterized by capillary malformation, venous malformations, and soft tissue or bone hypertrophy that affect the extremities in most cases. Knee or hip arthropathy are common associated conditions and cause serious disability. We present the case of a patient with a diagnosis of KTS and severe knee arthropathy. A 34-year-old man with KTS was referred to our hospital with severe knee arthropathy, with the joint fixed in a 90° position. CT Angiography and MRI of the left leg showed important varicose development of the superficial venous system with intraarticular vessels. After discussion of the case by a multidisciplinary committee, the patient was enrolled on a physiotherapy program and had achieved significant improvements in movement and quality of life at 12-month follow-up. Treatment of KTS is primarily conservative and a multidisciplinary approach is necessary.


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