Materiały źródłowe

• #1 Klippel-Trenaunay Syndrome (KTS): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17152-klippel-trenaunay-syndrome-kts

  Because KTS happens unpredictably, theres no way to prevent it. But treatment can help people with KTS live a higher quality of life. […] Ongoing treatment with your healthcare provider can lower your risk of complications.

• #2 Klippel-Trenaunay syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/klippel-trenaunay/symptoms-causes/syc-20374152

  Although there is no cure for KTS, treatment goals are to improve symptoms and prevent complications. […] It’s important to get a prompt, accurate diagnosis and appropriate care to treat symptoms and prevent complications.

• #3 (PDF) Use of Rivaroxaban for Prophylaxis of Superficial Venous Thrombosis in Klippel-Trenaunay-Weber Syndrome

  https://www.academia.edu/57288028/Use_of_Rivaroxaban_for_Prophylaxis_of_Superficial_Venous_Thrombosis_in_Klippel_Trenaunay_Weber_Syndrome

  Klippel-Trenaunay-Weber syndrome (KTWS) is a congenital malformation syndrome involving blood and lymph vessels and disturbed bone and soft tissue growth. […] He was ultimately maintained on prophylactic rivaroxaban to prevent recurrent thrombotic events. […] We evaluate the risk of pulmonary embolism in patients with KTWS, being aware of the risk of deep venous thrombosis in progress of the disease and the importance of early diagnosis in prognosis. […] Early diagnosis and treatment are important because of possible serious complications such as deep vein thrombosis and pulmonary embolism. […] Therefore, deep venous system must be evaluated definitely before determining the treatment strategy. […] 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.

• #4 Klippel-Trenaunay syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/klippel-trenaunay/symptoms-causes/syc-20374152

  Although there is no cure for KTS, treatment goals are to improve symptoms and prevent complications. […] It’s important to get a prompt, accurate diagnosis and appropriate care to treat symptoms and prevent complications.

• #5 (PDF) Use of Rivaroxaban for Prophylaxis of Superficial Venous Thrombosis in Klippel-Trenaunay-Weber Syndrome

  https://www.academia.edu/57288028/Use_of_Rivaroxaban_for_Prophylaxis_of_Superficial_Venous_Thrombosis_in_Klippel_Trenaunay_Weber_Syndrome

  Klippel-Trenaunay-Weber syndrome (KTWS) is a congenital malformation syndrome involving blood and lymph vessels and disturbed bone and soft tissue growth. […] He was ultimately maintained on prophylactic rivaroxaban to prevent recurrent thrombotic events. […] We evaluate the risk of pulmonary embolism in patients with KTWS, being aware of the risk of deep venous thrombosis in progress of the disease and the importance of early diagnosis in prognosis. […] Early diagnosis and treatment are important because of possible serious complications such as deep vein thrombosis and pulmonary embolism. […] Therefore, deep venous system must be evaluated definitely before determining the treatment strategy. […] 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.

• #6 Klippel–Trenaunay Syndrome with Hearing Loss: Is it the Cause? A Rare Case Report

  https://www.ijhns.com/abstractArticleContentBrowse/IJHNS/33974/JPJ/fullText

  An appropriate multidisciplinary approach to treatment and prevention of possible complications of KTS provides optimal care for the patient. […] The management of KTS has been largely conservative. […] The patient receives the best care when a proper multidisciplinary approach is used in the treatment and prevention of potential KTS complications. […] As a result, we recommend that hearing screenings be performed on these patients to learn more about the etiology of hearing loss.

• #7 Compression therapy in a patient with Klippel-Trénaunay syndrome :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/jwm/volume-24-number-3/compression-therapy-patient-klippel-trenaunay-syndrome

  Klippel-Trnaunay syndrome (KTS) is a rare congenital vascular malformation syndrome defined as capillary and venous malformations, and hypertrophy of the limb with or without lymphatic malformation. Currently there is no cure for KTS but the majority of patients are advised to wear compression garments on the affected limb to control swelling and discomfort. […] Compression therapy is essential in the treatment of Klippel-Trnaunay syndrome (KTS). […] Individualised compression therapy is beneficial for alleviating the swelling and lymphoedema associated with KTS. […] The majority of the KTS patients are advised to wear compression garments on the affected limb to control lymphoedema, venous insufficiency and discomfort. […] Static compression decreases the fluid escaping from the venous system and improves the proximal lymph flow which promotes lymph drainage into lymphatic-venous anastomoses and slows adipose deposition.

• #8 Klippel-Trénaunay Syndrome | Doctor

  https://patient.info/doctor/klippel-trenaunay-syndrome

  There is no curative therapy. Management requires a multidisciplinary and individualised approach. Symptomatic treatment is used to improve quality of life. Relative indications for intervention include pain, functional impairment, swelling, limb asymmetry, or cosmetic reasons. Absolute indications include haemorrhage, infections, refractory ulcers, or acute thromboembolism. […] Graduated compression garments help to reduce the effect of chronic venous insufficiency in the affected limb. Intermittent pneumatic compression pumps may also be used to the same effect. These help to reduce the effects of venous insufficiency but do not affect the ultimate size of the limb. […] Prophylaxis for VTE may be appropriate. […] Contraception: oestrogen-containing contraceptives are contra-indicated where there is a history of VTE and should probably be avoided in KT syndrome because of the increased VTE risk (although there is no specific UK contraception guideline for this condition). […] Pregnant women with KT syndrome need careful monitoring due to a range of haematological, obstetric and anaesthetic complications.

• #9 Compression therapy in a patient with Klippel-Trénaunay syndrome :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/jwm/volume-24-number-3/compression-therapy-patient-klippel-trenaunay-syndrome

  Klippel-Trnaunay syndrome (KTS) is a rare congenital vascular malformation syndrome defined as capillary and venous malformations, and hypertrophy of the limb with or without lymphatic malformation. Currently there is no cure for KTS but the majority of patients are advised to wear compression garments on the affected limb to control swelling and discomfort. […] Compression therapy is essential in the treatment of Klippel-Trnaunay syndrome (KTS). […] Individualised compression therapy is beneficial for alleviating the swelling and lymphoedema associated with KTS. […] The majority of the KTS patients are advised to wear compression garments on the affected limb to control lymphoedema, venous insufficiency and discomfort. […] Static compression decreases the fluid escaping from the venous system and improves the proximal lymph flow which promotes lymph drainage into lymphatic-venous anastomoses and slows adipose deposition.

• #10 Compression therapy in a patient with Klippel-Trénaunay syndrome :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/jwm/volume-24-number-3/compression-therapy-patient-klippel-trenaunay-syndrome

  Even though lymphoedema is incurable, compression therapy has been consistently described in the management of paediatric lymphoedema. […] Static compression applies continuous pressure to the affected extremity most effectively. […] Compression garments can also protect the affected area from incidental trauma and skin breaks, as well as prevent infections. […] Manual lymphatic drainages, intermittent pneumatic compressions and custom-made compression garments played a role in our patients comprehensive treatment. […] None of the treatment methods sufficiently reduced the patients limb oedema, but our case still highlights the importance of continuous individualised compression therapy in the treatment of lymphoedema combined with congenital vascular malformations. […] Symptoms may be alleviated substantially with custom-fitted garments, manual lymphatic drainages and intermittent pneumatic compressions, improving the patients quality of life as KTS is a lifelong disease requiring frequent, continuous follow-up visits and multimodal treatments. […] Research on the use of compression therapy for paediatric patients is needed to systemically determine the benefits and drawbacks.

• #11

  https://journals.lww.com/wajr/fulltext/2013/20020/unusual_age_at_presentation_of_klippel_trenaunay.6.aspx

  Klippel-Trenaunay syndrome is a congenital vascular disorder which is rare and affects one or both limbs. […] The management of this syndrome consists mainly of early diagnosis, prevention and treatment of complications. […] The management of this syndrome mainly include careful diagnosis, prevention and treatment of complications. […] Conservative treatment was given to the patient using elastic compression stockings. Patient was also advised to keep the limb elevated wherever possible and asked to come for yearly reviews. […] The effect of chronic venous insufficiency in the affected limb can be reduced by using graduated compression garments or intermittent pneumatic compression pumps may also be used. […] In cases with recurrent thrombophlebitis or before surgery, aspirin anticoagulants may be used as a prophylactic measure.

• #12 Compression therapy in a patient with Klippel-Trénaunay syndrome :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/jwm/volume-24-number-3/compression-therapy-patient-klippel-trenaunay-syndrome

  Even though lymphoedema is incurable, compression therapy has been consistently described in the management of paediatric lymphoedema. […] Static compression applies continuous pressure to the affected extremity most effectively. […] Compression garments can also protect the affected area from incidental trauma and skin breaks, as well as prevent infections. […] Manual lymphatic drainages, intermittent pneumatic compressions and custom-made compression garments played a role in our patients comprehensive treatment. […] None of the treatment methods sufficiently reduced the patients limb oedema, but our case still highlights the importance of continuous individualised compression therapy in the treatment of lymphoedema combined with congenital vascular malformations. […] Symptoms may be alleviated substantially with custom-fitted garments, manual lymphatic drainages and intermittent pneumatic compressions, improving the patients quality of life as KTS is a lifelong disease requiring frequent, continuous follow-up visits and multimodal treatments. […] Research on the use of compression therapy for paediatric patients is needed to systemically determine the benefits and drawbacks.

• #13

  https://journals.lww.com/ijvs/fulltext/2018/05030/management_of_klippel_trenaunay_syndrome_from_a.5.aspx

  Once a diagnosis of KTS was confirmed, each component (VM, LM, CM) was sub-classified as truncular or extra-truncular. Parents of children in the pediatric age group were advised symptomatic treatment, until they were at least 18-year-old. The importance of good skin care, height correction footwear, compression (either short stretch bandage or 2533 mm of Hg stockings), manual decongestive therapy (MLD), and need for regular follow-up was impressed on them. […] To reduce the edema (in adult patients) a short course therapy of tablet daflon 1 g once a day 3 months, tablet chlorthalidone 12.5 mg alternate days for a month were added to the above-mentioned plan. This we found improved compliance with the use of compression hosiery and MLD, as they saw a reduction in limb girth. […] Venous thromboembolism (VTE) prophylaxis was used in patients with abnormal D-dimer values pre- and post-operatively. […] The use of LMWH preoperatively and postoperatively in patients undergoing intervention, if their D-dimer was elevated has prevented any VTE event, thus far.

• #14 Klippel-Trenaunay syndrome | Children’s Health Queensland

  https://www.childrens.health.qld.gov.au/health-a-to-z/klippel-trenaunay-syndrome

  Compression therapy is the main treatment for children presenting with KTS. It assists in minimising swelling in the affected limbs, in turn reducing tissue overgrowth. […] The main purpose of this treatment is to enable your child to participate in activities as much as possible, as this is important for their development. […] There are a few small steps you can take at home to help relieve your child’s discomfort: Assist your child in completing their prescribed therapy regimen, Assist your child in caring for the affected limb with care as they can be easily damaged, Keep the limb clean and well moisturised, Encourage your child to participate in age-appropriate activities where possible.

• #15 Compression therapy in a patient with Klippel-Trénaunay syndrome :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/jwm/volume-24-number-3/compression-therapy-patient-klippel-trenaunay-syndrome

  Even though lymphoedema is incurable, compression therapy has been consistently described in the management of paediatric lymphoedema. […] Static compression applies continuous pressure to the affected extremity most effectively. […] Compression garments can also protect the affected area from incidental trauma and skin breaks, as well as prevent infections. […] Manual lymphatic drainages, intermittent pneumatic compressions and custom-made compression garments played a role in our patients comprehensive treatment. […] None of the treatment methods sufficiently reduced the patients limb oedema, but our case still highlights the importance of continuous individualised compression therapy in the treatment of lymphoedema combined with congenital vascular malformations. […] Symptoms may be alleviated substantially with custom-fitted garments, manual lymphatic drainages and intermittent pneumatic compressions, improving the patients quality of life as KTS is a lifelong disease requiring frequent, continuous follow-up visits and multimodal treatments. […] Research on the use of compression therapy for paediatric patients is needed to systemically determine the benefits and drawbacks.

• #16

  https://journals.lww.com/ijvs/fulltext/2018/05030/management_of_klippel_trenaunay_syndrome_from_a.5.aspx

  Once a diagnosis of KTS was confirmed, each component (VM, LM, CM) was sub-classified as truncular or extra-truncular. Parents of children in the pediatric age group were advised symptomatic treatment, until they were at least 18-year-old. The importance of good skin care, height correction footwear, compression (either short stretch bandage or 2533 mm of Hg stockings), manual decongestive therapy (MLD), and need for regular follow-up was impressed on them. […] To reduce the edema (in adult patients) a short course therapy of tablet daflon 1 g once a day 3 months, tablet chlorthalidone 12.5 mg alternate days for a month were added to the above-mentioned plan. This we found improved compliance with the use of compression hosiery and MLD, as they saw a reduction in limb girth. […] Venous thromboembolism (VTE) prophylaxis was used in patients with abnormal D-dimer values pre- and post-operatively. […] The use of LMWH preoperatively and postoperatively in patients undergoing intervention, if their D-dimer was elevated has prevented any VTE event, thus far.

• #17 Klippel-Trénaunay Syndrome | Doctor

  https://patient.info/doctor/klippel-trenaunay-syndrome

  There is no curative therapy. Management requires a multidisciplinary and individualised approach. Symptomatic treatment is used to improve quality of life. Relative indications for intervention include pain, functional impairment, swelling, limb asymmetry, or cosmetic reasons. Absolute indications include haemorrhage, infections, refractory ulcers, or acute thromboembolism. […] Graduated compression garments help to reduce the effect of chronic venous insufficiency in the affected limb. Intermittent pneumatic compression pumps may also be used to the same effect. These help to reduce the effects of venous insufficiency but do not affect the ultimate size of the limb. […] Prophylaxis for VTE may be appropriate. […] Contraception: oestrogen-containing contraceptives are contra-indicated where there is a history of VTE and should probably be avoided in KT syndrome because of the increased VTE risk (although there is no specific UK contraception guideline for this condition). […] Pregnant women with KT syndrome need careful monitoring due to a range of haematological, obstetric and anaesthetic complications.

• #18 (PDF) Use of Rivaroxaban for Prophylaxis of Superficial Venous Thrombosis in Klippel-Trenaunay-Weber Syndrome

  https://www.academia.edu/57288028/Use_of_Rivaroxaban_for_Prophylaxis_of_Superficial_Venous_Thrombosis_in_Klippel_Trenaunay_Weber_Syndrome

  Klippel-Trenaunay-Weber syndrome (KTWS) is a congenital malformation syndrome involving blood and lymph vessels and disturbed bone and soft tissue growth. […] He was ultimately maintained on prophylactic rivaroxaban to prevent recurrent thrombotic events. […] We evaluate the risk of pulmonary embolism in patients with KTWS, being aware of the risk of deep venous thrombosis in progress of the disease and the importance of early diagnosis in prognosis. […] Early diagnosis and treatment are important because of possible serious complications such as deep vein thrombosis and pulmonary embolism. […] Therefore, deep venous system must be evaluated definitely before determining the treatment strategy. […] 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.

• #19

  https://journals.lww.com/ijvs/fulltext/2018/05030/management_of_klippel_trenaunay_syndrome_from_a.5.aspx

  Once a diagnosis of KTS was confirmed, each component (VM, LM, CM) was sub-classified as truncular or extra-truncular. Parents of children in the pediatric age group were advised symptomatic treatment, until they were at least 18-year-old. The importance of good skin care, height correction footwear, compression (either short stretch bandage or 2533 mm of Hg stockings), manual decongestive therapy (MLD), and need for regular follow-up was impressed on them. […] To reduce the edema (in adult patients) a short course therapy of tablet daflon 1 g once a day 3 months, tablet chlorthalidone 12.5 mg alternate days for a month were added to the above-mentioned plan. This we found improved compliance with the use of compression hosiery and MLD, as they saw a reduction in limb girth. […] Venous thromboembolism (VTE) prophylaxis was used in patients with abnormal D-dimer values pre- and post-operatively. […] The use of LMWH preoperatively and postoperatively in patients undergoing intervention, if their D-dimer was elevated has prevented any VTE event, thus far.

• #20

  https://journals.lww.com/wajr/fulltext/2013/20020/unusual_age_at_presentation_of_klippel_trenaunay.6.aspx

  Klippel-Trenaunay syndrome is a congenital vascular disorder which is rare and affects one or both limbs. […] The management of this syndrome consists mainly of early diagnosis, prevention and treatment of complications. […] The management of this syndrome mainly include careful diagnosis, prevention and treatment of complications. […] Conservative treatment was given to the patient using elastic compression stockings. Patient was also advised to keep the limb elevated wherever possible and asked to come for yearly reviews. […] The effect of chronic venous insufficiency in the affected limb can be reduced by using graduated compression garments or intermittent pneumatic compression pumps may also be used. […] In cases with recurrent thrombophlebitis or before surgery, aspirin anticoagulants may be used as a prophylactic measure.

• #21 A multidisciplinary management approach for patients with Klippel-Trenaunay syndrome and multifetal gestation with successful outcomes

  https://www.degruyter.com/document/doi/10.1515/crpm-2017-0070/html?lang=en

  Management of pregnancies affected by KTS and multiple gestations should be personalized with a multi-disciplinary approach to avoid morbidity and improve maternal and neonatal outcomes. […] Anticoagulation should be addressed early and patients should be counseled on the importance of compliance. In both cases, patients received prophylactic dose LMWH daily antepartum with resumption of LMWH for 6 weeks postpartum. […] Given increased bleeding risks associated with KTS, extra precautions should be taken in preparation for postpartum hemorrhage, especially in multiple pregnancies. […] If not previously done, imaging studies should be performed to evaluate extent of vascular disease and to aid decision making for route of anesthesia as well as mode of delivery. […] Given the significant involvement of perineum with port-wine staining and baseline increased risk of bleeding within capillary stains, hematology recommended against vaginal delivery to avoid trauma to the perineum.

• #22 (PDF) Use of Rivaroxaban for Prophylaxis of Superficial Venous Thrombosis in Klippel-Trenaunay-Weber Syndrome

  https://www.academia.edu/57288028/Use_of_Rivaroxaban_for_Prophylaxis_of_Superficial_Venous_Thrombosis_in_Klippel_Trenaunay_Weber_Syndrome

  Klippel-Trenaunay-Weber syndrome (KTWS) is a congenital malformation syndrome involving blood and lymph vessels and disturbed bone and soft tissue growth. […] He was ultimately maintained on prophylactic rivaroxaban to prevent recurrent thrombotic events. […] We evaluate the risk of pulmonary embolism in patients with KTWS, being aware of the risk of deep venous thrombosis in progress of the disease and the importance of early diagnosis in prognosis. […] Early diagnosis and treatment are important because of possible serious complications such as deep vein thrombosis and pulmonary embolism. […] Therefore, deep venous system must be evaluated definitely before determining the treatment strategy. […] 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.

• #23

  https://journals.lww.com/ijvs/fulltext/2018/05030/management_of_klippel_trenaunay_syndrome_from_a.5.aspx

  Once a diagnosis of KTS was confirmed, each component (VM, LM, CM) was sub-classified as truncular or extra-truncular. Parents of children in the pediatric age group were advised symptomatic treatment, until they were at least 18-year-old. The importance of good skin care, height correction footwear, compression (either short stretch bandage or 2533 mm of Hg stockings), manual decongestive therapy (MLD), and need for regular follow-up was impressed on them. […] To reduce the edema (in adult patients) a short course therapy of tablet daflon 1 g once a day 3 months, tablet chlorthalidone 12.5 mg alternate days for a month were added to the above-mentioned plan. This we found improved compliance with the use of compression hosiery and MLD, as they saw a reduction in limb girth. […] Venous thromboembolism (VTE) prophylaxis was used in patients with abnormal D-dimer values pre- and post-operatively. […] The use of LMWH preoperatively and postoperatively in patients undergoing intervention, if their D-dimer was elevated has prevented any VTE event, thus far.

• #24

  https://www.indianpediatrics.net/aug2020/aug-754-755.htm

  Klippel Trenaunay syndrome (KTS) is a sporadic disorder that belongs to PIK3CA-related overgrowth spectrum of disorders. […] The patient was transfused and was put on prophylaxis for portal hypertension and referred to vascular surgery department for further intervention. […] Prophylactic anticoagulant therapy can be considered in patients with complex vascular malformations prior to radiological or surgical procedure.

• #25 (PDF) Use of Rivaroxaban for Prophylaxis of Superficial Venous Thrombosis in Klippel-Trenaunay-Weber Syndrome

  https://www.academia.edu/57288028/Use_of_Rivaroxaban_for_Prophylaxis_of_Superficial_Venous_Thrombosis_in_Klippel_Trenaunay_Weber_Syndrome

  A multidisciplinary approach to management of KTS is warranted. […] Compression is the hallmark of conservative management; laser can be used to treat port wine stains. […] Techniques for ablation of superficial veins and malformations are individualized and may include sclerotherapy with alcohol or foam, endovenous thermal ablation or, as used most frequently in our practice, surgical stripping and phlebectomy. […] Selective use of an inferior vena cava filter will prevent pulmonary embolism. […] Management of this syndrome includes careful diagnosis, prevention and treatment of complications.

• #26

  https://www.indianpediatrics.net/aug2020/aug-754-755.htm

  Klippel Trenaunay syndrome (KTS) is a sporadic disorder that belongs to PIK3CA-related overgrowth spectrum of disorders. […] The patient was transfused and was put on prophylaxis for portal hypertension and referred to vascular surgery department for further intervention. […] Prophylactic anticoagulant therapy can be considered in patients with complex vascular malformations prior to radiological or surgical procedure.

• #27 Klippel-Trénaunay Syndrome | Doctor

  https://patient.info/doctor/klippel-trenaunay-syndrome

  There is no curative therapy. Management requires a multidisciplinary and individualised approach. Symptomatic treatment is used to improve quality of life. Relative indications for intervention include pain, functional impairment, swelling, limb asymmetry, or cosmetic reasons. Absolute indications include haemorrhage, infections, refractory ulcers, or acute thromboembolism. […] Graduated compression garments help to reduce the effect of chronic venous insufficiency in the affected limb. Intermittent pneumatic compression pumps may also be used to the same effect. These help to reduce the effects of venous insufficiency but do not affect the ultimate size of the limb. […] Prophylaxis for VTE may be appropriate. […] Contraception: oestrogen-containing contraceptives are contra-indicated where there is a history of VTE and should probably be avoided in KT syndrome because of the increased VTE risk (although there is no specific UK contraception guideline for this condition). […] Pregnant women with KT syndrome need careful monitoring due to a range of haematological, obstetric and anaesthetic complications.

• #28 A multidisciplinary management approach for patients with Klippel-Trenaunay syndrome and multifetal gestation with successful outcomes

  https://www.degruyter.com/document/doi/10.1515/crpm-2017-0070/html?lang=en

  Management of pregnancies affected by KTS and multiple gestations should be personalized with a multi-disciplinary approach to avoid morbidity and improve maternal and neonatal outcomes. […] Anticoagulation should be addressed early and patients should be counseled on the importance of compliance. In both cases, patients received prophylactic dose LMWH daily antepartum with resumption of LMWH for 6 weeks postpartum. […] Given increased bleeding risks associated with KTS, extra precautions should be taken in preparation for postpartum hemorrhage, especially in multiple pregnancies. […] If not previously done, imaging studies should be performed to evaluate extent of vascular disease and to aid decision making for route of anesthesia as well as mode of delivery. […] Given the significant involvement of perineum with port-wine staining and baseline increased risk of bleeding within capillary stains, hematology recommended against vaginal delivery to avoid trauma to the perineum.

• #29 A multidisciplinary management approach for patients with Klippel-Trenaunay syndrome and multifetal gestation with successful outcomes

  https://www.degruyter.com/document/doi/10.1515/crpm-2017-0070/html?lang=en

  Management of pregnancies affected by KTS and multiple gestations should be personalized with a multi-disciplinary approach to avoid morbidity and improve maternal and neonatal outcomes. […] Anticoagulation should be addressed early and patients should be counseled on the importance of compliance. In both cases, patients received prophylactic dose LMWH daily antepartum with resumption of LMWH for 6 weeks postpartum. […] Given increased bleeding risks associated with KTS, extra precautions should be taken in preparation for postpartum hemorrhage, especially in multiple pregnancies. […] If not previously done, imaging studies should be performed to evaluate extent of vascular disease and to aid decision making for route of anesthesia as well as mode of delivery. […] Given the significant involvement of perineum with port-wine staining and baseline increased risk of bleeding within capillary stains, hematology recommended against vaginal delivery to avoid trauma to the perineum.

• #30 A multidisciplinary management approach for patients with Klippel-Trenaunay syndrome and multifetal gestation with successful outcomes

  https://www.degruyter.com/document/doi/10.1515/crpm-2017-0070/html?lang=en

  Management of pregnancies affected by KTS and multiple gestations should be personalized with a multi-disciplinary approach to avoid morbidity and improve maternal and neonatal outcomes. […] Anticoagulation should be addressed early and patients should be counseled on the importance of compliance. In both cases, patients received prophylactic dose LMWH daily antepartum with resumption of LMWH for 6 weeks postpartum. […] Given increased bleeding risks associated with KTS, extra precautions should be taken in preparation for postpartum hemorrhage, especially in multiple pregnancies. […] If not previously done, imaging studies should be performed to evaluate extent of vascular disease and to aid decision making for route of anesthesia as well as mode of delivery. […] Given the significant involvement of perineum with port-wine staining and baseline increased risk of bleeding within capillary stains, hematology recommended against vaginal delivery to avoid trauma to the perineum.

• #31 Klippel-Trénaunay Syndrome | Doctor

  https://patient.info/doctor/klippel-trenaunay-syndrome

  There is no curative therapy. Management requires a multidisciplinary and individualised approach. Symptomatic treatment is used to improve quality of life. Relative indications for intervention include pain, functional impairment, swelling, limb asymmetry, or cosmetic reasons. Absolute indications include haemorrhage, infections, refractory ulcers, or acute thromboembolism. […] Graduated compression garments help to reduce the effect of chronic venous insufficiency in the affected limb. Intermittent pneumatic compression pumps may also be used to the same effect. These help to reduce the effects of venous insufficiency but do not affect the ultimate size of the limb. […] Prophylaxis for VTE may be appropriate. […] Contraception: oestrogen-containing contraceptives are contra-indicated where there is a history of VTE and should probably be avoided in KT syndrome because of the increased VTE risk (although there is no specific UK contraception guideline for this condition). […] Pregnant women with KT syndrome need careful monitoring due to a range of haematological, obstetric and anaesthetic complications.

• #32

  https://journals.lww.com/ijvs/fulltext/2018/05030/management_of_klippel_trenaunay_syndrome_from_a.5.aspx

  Once a diagnosis of KTS was confirmed, each component (VM, LM, CM) was sub-classified as truncular or extra-truncular. Parents of children in the pediatric age group were advised symptomatic treatment, until they were at least 18-year-old. The importance of good skin care, height correction footwear, compression (either short stretch bandage or 2533 mm of Hg stockings), manual decongestive therapy (MLD), and need for regular follow-up was impressed on them. […] To reduce the edema (in adult patients) a short course therapy of tablet daflon 1 g once a day 3 months, tablet chlorthalidone 12.5 mg alternate days for a month were added to the above-mentioned plan. This we found improved compliance with the use of compression hosiery and MLD, as they saw a reduction in limb girth. […] Venous thromboembolism (VTE) prophylaxis was used in patients with abnormal D-dimer values pre- and post-operatively. […] The use of LMWH preoperatively and postoperatively in patients undergoing intervention, if their D-dimer was elevated has prevented any VTE event, thus far.

• #33 Klippel-Trenaunay syndrome | Children’s Health Queensland

  https://www.childrens.health.qld.gov.au/health-a-to-z/klippel-trenaunay-syndrome

  Compression therapy is the main treatment for children presenting with KTS. It assists in minimising swelling in the affected limbs, in turn reducing tissue overgrowth. […] The main purpose of this treatment is to enable your child to participate in activities as much as possible, as this is important for their development. […] There are a few small steps you can take at home to help relieve your child’s discomfort: Assist your child in completing their prescribed therapy regimen, Assist your child in caring for the affected limb with care as they can be easily damaged, Keep the limb clean and well moisturised, Encourage your child to participate in age-appropriate activities where possible.

• #34 Klippel-Trenaunay syndrome | Children’s Health Queensland

  https://www.childrens.health.qld.gov.au/health-a-to-z/klippel-trenaunay-syndrome

  Compression therapy is the main treatment for children presenting with KTS. It assists in minimising swelling in the affected limbs, in turn reducing tissue overgrowth. […] The main purpose of this treatment is to enable your child to participate in activities as much as possible, as this is important for their development. […] There are a few small steps you can take at home to help relieve your child’s discomfort: Assist your child in completing their prescribed therapy regimen, Assist your child in caring for the affected limb with care as they can be easily damaged, Keep the limb clean and well moisturised, Encourage your child to participate in age-appropriate activities where possible.

• #35 Klippel-Trenaunay syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/klippel-trenaunay/diagnosis-treatment/drc-20374155

  Although there’s no cure for KTS, your doctor can help you manage symptoms and prevent complications. […] In addition, treatment may be needed for complications such as bleeding, infection, blood clots or skin ulcers. Special care may be needed during pregnancy to prevent complications. […] Strategies to help manage KTS symptoms include: Regularly scheduled appointments can help your doctor identify and address problems early. […] Work with your doctor to manage your symptoms and prevent complications. Contact your doctor if you have symptoms of blood clots or an infection or if you experience increased pain or swelling.

• #36 Klippel-Trenaunay syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/klippel-trenaunay-syndrome?content_id=CON-20374143

  Although there is no cure for KTS, treatment goals are to improve symptoms and prevent complications. […] It’s important to get a prompt, accurate diagnosis and appropriate care to treat symptoms and prevent complications. […] Although there’s no cure for KTS, your doctor can help you manage symptoms and prevent complications. […] In addition, treatment may be needed for complications such as bleeding, infection, blood clots or skin ulcers. Special care may be needed during pregnancy to prevent complications. […] Strategies to help manage KTS symptoms include: Regularly scheduled appointments can help your doctor identify and address problems early. […] Work with your doctor to manage your symptoms and prevent complications.

• #37 Klippel-Trenaunay syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/klippel-trenaunay/diagnosis-treatment/drc-20374155

  Although there’s no cure for KTS, your doctor can help you manage symptoms and prevent complications. […] In addition, treatment may be needed for complications such as bleeding, infection, blood clots or skin ulcers. Special care may be needed during pregnancy to prevent complications. […] Strategies to help manage KTS symptoms include: Regularly scheduled appointments can help your doctor identify and address problems early. […] Work with your doctor to manage your symptoms and prevent complications. Contact your doctor if you have symptoms of blood clots or an infection or if you experience increased pain or swelling.

• #38 Klippel-Trenaunay syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/klippel-trenaunay-syndrome?content_id=CON-20374143

  Although there is no cure for KTS, treatment goals are to improve symptoms and prevent complications. […] It’s important to get a prompt, accurate diagnosis and appropriate care to treat symptoms and prevent complications. […] Although there’s no cure for KTS, your doctor can help you manage symptoms and prevent complications. […] In addition, treatment may be needed for complications such as bleeding, infection, blood clots or skin ulcers. Special care may be needed during pregnancy to prevent complications. […] Strategies to help manage KTS symptoms include: Regularly scheduled appointments can help your doctor identify and address problems early. […] Work with your doctor to manage your symptoms and prevent complications.

• #39 Klippel-Trenaunay syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/klippel-trenaunay/diagnosis-treatment/drc-20374155

  Although there’s no cure for KTS, your doctor can help you manage symptoms and prevent complications. […] In addition, treatment may be needed for complications such as bleeding, infection, blood clots or skin ulcers. Special care may be needed during pregnancy to prevent complications. […] Strategies to help manage KTS symptoms include: Regularly scheduled appointments can help your doctor identify and address problems early. […] Work with your doctor to manage your symptoms and prevent complications. Contact your doctor if you have symptoms of blood clots or an infection or if you experience increased pain or swelling.

• #40 Klippel-Trénaunay Syndrome | Doctor

  https://patient.info/doctor/klippel-trenaunay-syndrome

  There is no curative therapy. Management requires a multidisciplinary and individualised approach. Symptomatic treatment is used to improve quality of life. Relative indications for intervention include pain, functional impairment, swelling, limb asymmetry, or cosmetic reasons. Absolute indications include haemorrhage, infections, refractory ulcers, or acute thromboembolism. […] Graduated compression garments help to reduce the effect of chronic venous insufficiency in the affected limb. Intermittent pneumatic compression pumps may also be used to the same effect. These help to reduce the effects of venous insufficiency but do not affect the ultimate size of the limb. […] Prophylaxis for VTE may be appropriate. […] Contraception: oestrogen-containing contraceptives are contra-indicated where there is a history of VTE and should probably be avoided in KT syndrome because of the increased VTE risk (although there is no specific UK contraception guideline for this condition). […] Pregnant women with KT syndrome need careful monitoring due to a range of haematological, obstetric and anaesthetic complications.

• #41 Klippel-Trenaunay Syndrome (KTS): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17152-klippel-trenaunay-syndrome-kts

  Because KTS happens unpredictably, theres no way to prevent it. But treatment can help people with KTS live a higher quality of life. […] Ongoing treatment with your healthcare provider can lower your risk of complications.

• #42 Compression therapy in a patient with Klippel-Trénaunay syndrome :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/jwm/volume-24-number-3/compression-therapy-patient-klippel-trenaunay-syndrome

  Klippel-Trnaunay syndrome (KTS) is a rare congenital vascular malformation syndrome defined as capillary and venous malformations, and hypertrophy of the limb with or without lymphatic malformation. Currently there is no cure for KTS but the majority of patients are advised to wear compression garments on the affected limb to control swelling and discomfort. […] Compression therapy is essential in the treatment of Klippel-Trnaunay syndrome (KTS). […] Individualised compression therapy is beneficial for alleviating the swelling and lymphoedema associated with KTS. […] The majority of the KTS patients are advised to wear compression garments on the affected limb to control lymphoedema, venous insufficiency and discomfort. […] Static compression decreases the fluid escaping from the venous system and improves the proximal lymph flow which promotes lymph drainage into lymphatic-venous anastomoses and slows adipose deposition.

• #43 Compression therapy in a patient with Klippel-Trénaunay syndrome :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/jwm/volume-24-number-3/compression-therapy-patient-klippel-trenaunay-syndrome

  Even though lymphoedema is incurable, compression therapy has been consistently described in the management of paediatric lymphoedema. […] Static compression applies continuous pressure to the affected extremity most effectively. […] Compression garments can also protect the affected area from incidental trauma and skin breaks, as well as prevent infections. […] Manual lymphatic drainages, intermittent pneumatic compressions and custom-made compression garments played a role in our patients comprehensive treatment. […] None of the treatment methods sufficiently reduced the patients limb oedema, but our case still highlights the importance of continuous individualised compression therapy in the treatment of lymphoedema combined with congenital vascular malformations. […] Symptoms may be alleviated substantially with custom-fitted garments, manual lymphatic drainages and intermittent pneumatic compressions, improving the patients quality of life as KTS is a lifelong disease requiring frequent, continuous follow-up visits and multimodal treatments. […] Research on the use of compression therapy for paediatric patients is needed to systemically determine the benefits and drawbacks.

• #44 (PDF) Use of Rivaroxaban for Prophylaxis of Superficial Venous Thrombosis in Klippel-Trenaunay-Weber Syndrome

  https://www.academia.edu/57288028/Use_of_Rivaroxaban_for_Prophylaxis_of_Superficial_Venous_Thrombosis_in_Klippel_Trenaunay_Weber_Syndrome

  A multidisciplinary approach to management of KTS is warranted. […] Compression is the hallmark of conservative management; laser can be used to treat port wine stains. […] Techniques for ablation of superficial veins and malformations are individualized and may include sclerotherapy with alcohol or foam, endovenous thermal ablation or, as used most frequently in our practice, surgical stripping and phlebectomy. […] Selective use of an inferior vena cava filter will prevent pulmonary embolism. […] Management of this syndrome includes careful diagnosis, prevention and treatment of complications.

• #45

  https://journals.lww.com/wajr/fulltext/2013/20020/unusual_age_at_presentation_of_klippel_trenaunay.6.aspx

  Klippel-Trenaunay syndrome is a congenital vascular disorder which is rare and affects one or both limbs. […] The management of this syndrome consists mainly of early diagnosis, prevention and treatment of complications. […] The management of this syndrome mainly include careful diagnosis, prevention and treatment of complications. […] Conservative treatment was given to the patient using elastic compression stockings. Patient was also advised to keep the limb elevated wherever possible and asked to come for yearly reviews. […] The effect of chronic venous insufficiency in the affected limb can be reduced by using graduated compression garments or intermittent pneumatic compression pumps may also be used. […] In cases with recurrent thrombophlebitis or before surgery, aspirin anticoagulants may be used as a prophylactic measure.

• #46 Klippel-Trenaunay syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/klippel-trenaunay/symptoms-causes/syc-20374152

  Although there is no cure for KTS, treatment goals are to improve symptoms and prevent complications. […] It’s important to get a prompt, accurate diagnosis and appropriate care to treat symptoms and prevent complications.

• #47 Compression therapy in a patient with Klippel-Trénaunay syndrome :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/jwm/volume-24-number-3/compression-therapy-patient-klippel-trenaunay-syndrome

  Even though lymphoedema is incurable, compression therapy has been consistently described in the management of paediatric lymphoedema. […] Static compression applies continuous pressure to the affected extremity most effectively. […] Compression garments can also protect the affected area from incidental trauma and skin breaks, as well as prevent infections. […] Manual lymphatic drainages, intermittent pneumatic compressions and custom-made compression garments played a role in our patients comprehensive treatment. […] None of the treatment methods sufficiently reduced the patients limb oedema, but our case still highlights the importance of continuous individualised compression therapy in the treatment of lymphoedema combined with congenital vascular malformations. […] Symptoms may be alleviated substantially with custom-fitted garments, manual lymphatic drainages and intermittent pneumatic compressions, improving the patients quality of life as KTS is a lifelong disease requiring frequent, continuous follow-up visits and multimodal treatments. […] Research on the use of compression therapy for paediatric patients is needed to systemically determine the benefits and drawbacks.

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