Lymphaticovenular anastomosis for Klippel-Trenaunay-Weber syndrome

Onoda, Shigeki; Komagoe, Sho

doi:10.1016/j.ijscr.2019.04.023

Cited by 8 publications

(7 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Many studies demonstrated the use of LVA for extremity lymphedema treatment, and it is currently the standard lymphedema therapy 6–10 . Furthermore, as we previously reported, groin lymphorrhea, pelvic lymphocele, and chylous ascites caused by lymphatic vessel injuries can also be treated with LVA, as it reduces the amount of lymphatic fluid flowing into injured lymphatic vessels 11 .…”

Section: Discussionmentioning

confidence: 86%

Multiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome

Miyashita,

Kadota,

Hanada

et al. 2023

Ann Plast Surg

View full text Add to dashboard Cite

Klippel-Trenaunay syndrome (KTS) is characterized by port-wine stains, mixed vascular malformations, and soft tissue and bone hypertrophy. Klippel-Trenaunay syndrome is occasionally complicated by chyluria, for which there is no effective treatment currently. We report a case of KTS complicated by intractable chyluria and hematuria due to a lymphatic-ureteral fistula. The patient was successfully treated with multiple lymphaticovenular anastomoses (LVAs). A 66-year-old woman with an enlarged left lower extremity since childhood was diagnosed with KTS. At 60 years of age, she developed chyluria (urine albumin, 2224 μg/mL) and hematuria. Lymphoscintigraphy showed a lymphatic-ureteral fistula near the ureterovesical junction. Conservative treatment was ineffective. She also developed left lower extremity lymphedema, which gradually worsened. Leg cellulitis and purulent pericarditis developed because of hypoalbuminemia (minimum serum albumin level, 1.3 g/dL). We performed 14 LVAs in 2 surgeries to reduce lymphatic fluid flow through the lymphatic-ureteral fistula. The chyluria and hematuria resolved soon after the second operation, and the urine albumin level decreased (3 μg/mL). After 28 months, she had no chyluria or hematuria recurrence and her serum albumin level improved (3.9 g/dL). Multiple LVAs can definitively treat chyluria caused by a lymphatic-ureteral fistula in patients with KTS.

show abstract

Section: Discussionmentioning

confidence: 86%

Multiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome

Miyashita,

Kadota,

Hanada

et al. 2023

Ann Plast Surg

View full text Add to dashboard Cite

show abstract

“…20 Lymphaticovenular anastomosis (LVA) has also proven to be effective in the treatment of cellulitis in these patients. 21 Gastrointestinal involvement in KTWS disease is rare but very life-threatening, so in a study by Servelle et al, of 588 KTWS patients, only six had gastrointestinal bleeding and in cases where the patient has severe bleeding, it may be necessary to remove a part of the digestive system. 22 Usually, in KTWS patients, vascular abnormalities do not involve all parts of the digestive system.…”

Section: Discussionmentioning

confidence: 99%

KTWS (Klippel‐Trenaunay‐Weber syndrome): A systematic presentation of a rare disease

Mofarrah,

Gooranorimi

et al. 2024

J of Cosmetic Dermatology

View full text Add to dashboard Cite

BackgroundKlippel‐Trenaunay‐Weber syndrome (KTWS) is a rare disease with a wide range of manifestations. KTWS is characterized by a clinical triad of varicosities of the extremities, cutaneous vascular malformations, and hypertrophy of soft tissues and long bones. The diagnosis is made clinically supplemented with magnetic resonance imaging and computed tomography.AimHereby we aim to highlight the significance of the possible life‐threatening first‐time presentations associated with the GI system in previously undiagnosed KTWS patients.PatientWe report the case of a 47‐year‐old male with KTWS, who presented with various symptoms such as rectorrhagia since childhood, digestive problems and abnormal lateral vascular malformations of the left buttock which extended all the way to the leg, vascular malformations of the left fourth and fifth toes as well as soft tissue swelling of the left foot. There was no evidence of other clinical presentations. The patient was hospitalized with severe rectorrhagia and a hemoglobin level of 3/9. Physical examination revealed a blood pressure of 85/55 and pulse rate of 115. Ruptured aneurysm of the superior mesenteric artery was found on angiography and subsequently treated with embolization. Dermatologic evaluation showed pitting edema of the left leg and foot and multiple vascular lesions. Thus a diagnosis of KTWS was established. Pulsed dye laser therapy and compression bandage was performed for the patient. The patient's follow‐up was done 3 months after discharge for which the patient was again consulted by a dermatologist and gastroenterologist. Lymphedema of the left leg had improved to a great extend so treatment with compression bandage was continued. Colonoscopy was repeated for the patient to evaluate and control possible active sources of bleeding, due to potential life‐threating complications.ResultsAccording to previous findings, there have been few case reports of KTWS presenting with gastrointestinal manifestations, fewer of which have covered acute life‐threatening bleedings associated with this system.

show abstract

“…There are many ways to remove malformed veins. [7][8][9]15] In this case, due to the man lymphedema of the leg, frequent cellulitis, and skin nutritional disorders, any direct puncture or incision may result in non-healing of the incision or ulceration. Therefore, in the first operation, we performed foam sclerotherapy through the right femoral vein approach into the malformed vein of the left lower extremity.…”

Section: Discussionmentioning

confidence: 99%

“…[5,6] Surgical, endovascular laser or radiofrequency ablation of superficial varicose veins or venous lymphatic anastomosis have been reported to reduce lymphedema, but there is a risk of nonunion of the surgical incision. [7][8][9] In our case, Doppler ultrasonography (DUS)combined with CT venography (CTV) was used to evaluate the clinical and hemodynamic characteristics of KTS, and foam sclerotherapy was applied according to the characteristics of KTS, which successfully prevented frequent cellulitis in the patient with KTS and significantly improved his quality of life.…”

Section: Introductionmentioning

confidence: 99%

Foam sclerotherapy for patients with Klipple-Trenaunary syndrome complicated by frequent cellulitis of lower extremity: A case report

Li,

Wu,

et al. 2023

Medicine

View full text Add to dashboard Cite

Rationale: Klipple-Trenaunary Syndrome (KTS) complicated by frequent cellulitis of lower extremity seriously affects a patient quality of life. The hemodynamic characteristics of the disease are still unclear. Direct skin incision or puncture to remove malformed veins at the lesion site carries the risk of non-healing of the surgical incision. Our aim is to explore initial management strategies based on the hemodynamic characteristics of this disease. Patient concerns: A 29-year-old Manchu man was affected by KTS from childhood, characterized by an increase of the circumference and superficial varicose veins of the lower extremity. In the past 5 years, he suffered from frequent cellulitis in the left leg every 15 days or so. Diagnoses: KTS complicated by frequent cellulitis of lower extremity. Interventions: The clinical and hemodynamic characteristics of KTS were evaluated by Doppler ultrasonography (DUS) combined with CT venography (CTV), and foam sclerotherapy and postoperative elastic bandage compression were performed accordingly. Outcomes: Based on evaluations, the reason for frequent cellulitis was the continuous increase of venous hypertension in the calf caused by the malformed superficial vein and its penetrating vein. After 3 operations, the patient had no recurrence of cellulitis of the leg. Follow-up for 1 year showed no recurrence of left leg cellulitis. Lessons: This report emphasizes that foam sclerotherapy can significantly improve the clinical symptoms of KTS, such as cellulitis, and provide a safe skin environment for the implementation of other surgical methods, based on the evaluation of the pathological characteristics of KTS by DUS combined with CTV.

show abstract

Lymphaticovenular anastomosis for Klippel-Trenaunay-Weber syndrome

Cited by 8 publications

References 8 publications

Multiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome

Multiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome

KTWS (Klippel‐Trenaunay‐Weber syndrome): A systematic presentation of a rare disease

Foam sclerotherapy for patients with Klipple-Trenaunary syndrome complicated by frequent cellulitis of lower extremity: A case report

Contact Info

Product

Resources

About