Multiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome.

ABSTRACT: 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.

Anaesthetic management of an abdominal aortic aneurysmorrhaphy in Klippel-Trenaunay-Weber syndrome: a case report.

BACKGROUND: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital malformation. Although there have been few reports on anaesthetic management of patients with KTWS, there is a lack of data on anaesthetic management for abdominal aortic aneurysm (AAA) surgeries in these patients. CASE PRESENTATION: A 74-year-old man (height, 160 cm and body weight, 51.5 kg) with KTWS was scheduled for AAA replacement. Abdominal computed tomography (CT) showed prominent tortuosity below the abdominal aorta with an infrarenal abdominal aortic aneurysm, right common iliac artery aneurysm, and right external iliac artery aneurysm. Moreover, a remarkably noted arteriovenous fistula had developed between the aneurysm and peripheral artery. General anaesthesia was induced. Furthermore, a central venous catheter and an 8.5 French sheath in the left internal jugular vein were inserted. During the operation, bleeding from a collateral vessel in the cross-clamped aorta led the surgeon to decide to perform aneurysmorrhaphy. Intraoperatively, blood loss was 1500 ml, and 20 units of red blood cell concentrate were used. CONCLUSIONS: Regarding AAA procedures in patients with KTWS, aortic cross-clamping may not sufficiently intercept blood flow due to collateral vessels. In these patients, the anaesthesiologist must be prepared to transfuse blood more rapidly and frequently than during normal AAA procedures.

Complex Total Hip Arthroplasty in a Patient with Klippel-Trénaunay Syndrome: A Case Report.

CASE: Klippel-Trénaunay syndrome (KTS) carries manifestations including skeletal dysplasia and prominent vascular malformations. This report details a case of hip dysplasia in the setting of KTS treated with total hip arthroplasty (THA) requiring preoperative embolization, intraoperative angiography for placement of an iliac artery occlusive balloon, and modular hip arthroplasty components for femoral and acetabular dysplasia. Perioperatively, the patient rehabilitated well and was walking painlessly and unassisted at 3 and 12 months postoperatively. CONCLUSION: Successful THA for dysplasia and degenerative changes associated with KTS is possible but requires a complex multidisciplinary perioperative approach.

Clinical presentation and outcomes after endovascular management in a mixed pediatric and adult Klippel-Trenaunay syndrome population.

OBJECTIVE: We retrospectively studied the clinical presentations and outcomes of endovascular management in a mixed pediatric and adult Klippel-Trenaunay syndrome (KTS) population at a single academic medical center. METHODS: We performed a retrospective study of patients with KTS who had been referred for endovascular intervention after evaluation and diagnosis by a multidisciplinary team at a single academic medical center during a 10-year period. The patient demographics, areas affected, presenting symptoms, previous treatments, imaging modalities, endovascular treatment types, number of treatments, and complications were assessed. The technical and clinical success rates were calculated. RESULTS: Twenty-six patients with suspected KTS were evaluated. Of these 26 patients, 20, aged 2 to 75 years, had been diagnosed with KTS using the International Society for the Study of Vascular Anomalies criteria and referred for endovascular management. The left lower extremity was affected most often. The presenting symptoms were pain (80%), edema (70%), bleeding (10%), numbness (25%), and claudication (25%). Of the 20 patients, 16 (80%) had undergone treatment of KTS before presenting to our institution. Magnetic resonance imaging and ultrasound (US) were the most common imaging modalities. Fifteen patients underwent 46 endovascular treatments during the study period. The treatments included 5 endovenous ablations only, 4 US-guided sclerotherapies with endovenous ablation, 5 US-guided sclerotherapies only, and 32 catheter-directed venograms with additional interventions. Localized intravascular coagulopathy was the only procedure-related complication and occurred in one patient after three treatments. The technical success rate was 97.8%, and the clinical success rate was 100%. CONCLUSIONS: Endovascular intervention is safe and effective for KTS patients for whom conservative management has failed. Pain and edema were the most common presenting symptoms. Presenting symptoms may be related to pathology of anomalous veins, orthotopic superficial veins or deep veins. Venous claudication can be present in those with KTS despite patency of the deep venous system. Magnetic resonance imaging and duplex US are frequently used modalities for venous assessment. The complications of endovascular treatment are rare but include localized intravascular coagulopathy.

Undiagnosed Case of Klippel-Trenaunnay Syndrome Presenting as Extensive Heterotrophic Ossification and Flexion Deformity of Right Lower Limb Requiring Amputation : A Case Report.

Klippel-Trenaunnay Syndrome is a rare disease characterized by a clinical triad of capillary malformation, soft tissue and bony hypertrophy, and atypical varicosity. This syndrome ranges from asymptomatic disease to life-threatening bleeding, embolism, and deformities. Management includes early diagnosis, prevention, and treatment of complications. We present a case of a 43-year-old male presenting with pain, swelling and deformity of the right leg for 30 years. On examination, diffusely enlarged tender right limb with several dark patchy discolorations, multiple tortuous vessels were found. Right leg X-ray showed heterotrophic ossification and distortion of ankle joint. Due to chronic severe pain, recurrent infection, contracture and flexion deformity of right leg, the patient underwent above knee amputation. This case focuses on the variable presentation and multiple problems faced by patients with Klippel-Trenaununay Syndrome as they get diagnosed late and shows the importance of high index of suspicion for early diagnosis and prevention of complications.

Klippel-Trenaunay-Weber Syndrome Associated with Multiple Cerebral Arteriovenous Malformations: Usefulness of Gamma Knife Stereotactic Radiosurgery in This Syndrome.

BACKGROUND: Klippel-Trenaunay-Weber syndrome (KTWS) is characterized by the presence of a combined vascular malformation of capillaries, veins, and lymphatic vessels; congenital venous abnormalities; and limb hypertrophy. Its association with neurovascular abnormalities is infrequent, and the presence of intracranial arteriovenous malformations (AVMs) is extremely rare. CASE DESCRIPTION: We report a case of a 48-year-old male diagnosed with KTWS who spontaneously presented with a cerebral hemorrhage. Computed tomography scan and angio-computed tomography studies revealed bleeding associated with AVM rupture. In the conventional arteriography study, 10 small (<1 cm) AVMs were observed. The patient presented a good clinical recovery. These multiple small lesions were not considered susceptible to surgical or endovascular treatments. Therefore all lesions were treated with Gamma Knife stereotactic radiosurgery since it attains the highest dose drop and minimal irradiation of the healthy parenchyma. One year after the treatment, the lesions have shrunk. CONCLUSIONS: Cerebral AVMs are extremely rare in KTWS cases; however, their presence can have serious consequences if they are treated. We find it advisable to include brain imaging tests, such as nuclear magnetic resonance imaging, to diagnose and monitor KTWS. Furthermore, a Gamma Knife may be useful when multiple AVMs are present.

[Klippel-Trenaunay-Weber syndrome with vesical and uterine involvement treated by endoscopic and endovascular routes]. / Síndrome de Klippel-Trenaunay-Weber con compromiso vesical y uterino tratado por vía endoscópica y endovascular.

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare venous malformation that generally affects the lower limbs and, more infrequently, the upper limbs. It is characterized by cutaneous angiomatous formations, varicose veins and hypertrophy of the affected limb. The involvement of the genitourinary tract is extremely infrequent. We expose the case of a 14 years old female patient who was admitted for macroscopic hematuria of 48 hours of evolution and metrorrhagia with severe hemodynamic decompensation. The patient was under study for presenting a hemangioma in the lower left limb that extended to the pelvic region. Urethrocystofibroscopy showed the presence of multiple wide-spread angiomatous lesions in the bladder, some of them with active bleeding. The angio-resonance showed a voluminous hypervascular formation in contact with the bladder wall showing several arteriovenous fistulas at the pelvic level and in the left lower limb confirming the etiological diagnosis. A selective arterial embolization of the internal and external iliac territories was performed and then, a laser endocoagulation of the bleeding angiomatous foci was carried out. The hematuria completely stopped within 24 hours later of the procedure. The metrorrhagia associated with KTWS was controlled by the use of LHRH analogs and progestogens.

El síndrome de Klippel-Trenaunay-Weber (SKTW) es una rara malformación venosa que, en general afecta a los miembros inferiores y, más raramente, a los superiores. Se caracteriza por formaciones angiomatosas cutáneas, várices e hipertrofia del miembro afectado. El compromiso genitourinario es sumamente infrecuente. Se presenta el caso de una paciente de 14 años. Ingresó por hematuria macroscópica de 48 h de evolución y metrorragia con grave compromiso hemodinámico. Se encontraba en estudio por presentar un hemangioma en el miembro inferior izquierdo que se extendía hasta la región pelviana. La uretrocistofibroscopía demostró la presencia de múltiples lesiones angiomatosas diseminadas en forma amplia en la vejiga, algunas de ellas con sangrado activo. La angioresonancia mostró una voluminosa formación hipervascularizada en contacto con la pared vesical a la cual desplazaba y fístulas arteriovenosas a nivel pelviano y en el miembro inferior izquierdo confirmando el diagnóstico etiológico. Se realizó una embolización arterial selectiva de los territorios ilíacos interno y externo e inmediatamente después una endocoagulación láser de los focos angiomatosos sangrantes. La hematuria remitió completamente en las 24 h posteriores al procedimiento. La metrorragia asociada al SKTW fue controlada mediante la utilización de análogos LHRH y progestágenos.

Síndrome de Klipper-Trenaunay-Weber con compromiso vesical y uterino tratado por vía endoscópica y endovascular / Klippel-Trenaunay-Weber syndrome with vesical and uterine involvement treated by endoscopic and endovascular routes

El síndrome de Klippel-Trenaunay-Weber (SKTW) es una rara malformación venosa que, en general afecta a los miembros inferiores y, más raramente, a los superiores. Se caracteriza por formaciones angiomatosas cutáneas, várices e hipertrofia del miembro afectado. El compromiso genitourinario es sumamente infrecuente. Se presenta el caso de una paciente de 14 años. Ingresó por hematuria macroscópica de 48 h de evolución y metrorragia con grave compromiso hemodinámico. Se encontraba en estudio por presentar un hemangioma en el miembro inferior izquierdo que se extendía hasta la región pelviana. La uretrocistofibroscopía demostró la presencia de múltiples lesiones angiomatosas diseminadas en forma amplia en la vejiga, algunas de ellas con sangrado activo. La angioresonancia mostró una voluminosa formación hipervascularizada en contacto con la pared vesical a la cual desplazaba y fístulas arteriovenosas a nivel pelviano y en el miembro inferior izquierdo confirmando el diagnóstico etiológico. Se realizó una embolización arterial selectiva de los territorios ilíacos interno y externo e inmediatamente después una endocoagulación láser de los focos angiomatosos sangrantes. La hematuria remitió completamente en las 24 h posteriores al procedimiento. La metrorragia asociada al SKTW fue controlada mediante la utilización de análogos LHRH y progestágenos.

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare venous malformation that generally affects the lower limbs and, more infrequently, the upper limbs. It is characterized by cutaneous angiomatous formations, varicose veins and hypertrophy of the affected limb. The involvement of the genitourinary tract is extremely infrequent. We expose the case of a 14 years old female patient who was admitted for macroscopic hematuria of 48 hours of evolution and metrorrhagia with severe hemodynamic decompensation. The patient was under study for presenting a hemangioma in the lower left limb that extended to the pelvic region. Urethrocystofibroscopy showed the presence of multiple wide-spread angiomatous lesions in the bladder, some of them with active bleeding. The angio-resonance showed a voluminous hypervascular formation in contact with the bladder wall showing several arteriovenous fistulas at the pelvic level and in the left lower limb confirming the etiological diagnosis. A selective arterial embolization of the internal and external iliac territories was performed and then, a laser endocoagulation of the bleeding angiomatous foci was carried out. The hematuria completely stopped within 24 hours later of the procedure. The metrorrhagia associated with KTWS was controlled by the use of LHRH analogs and progestogens.

Laser ablation of embryonic veins in children.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) and related overgrowth syndromes are rare conditions that often present to academic pediatric units and multidisciplinary vascular anomaly clinics. These conditions are commonly associated with abnormalities of the superficial and deep venous structures and carry an increased risk of venous thromboembolism. METHODS: A retrospective chart review was completed of all patients treated at Johns Hopkins All Children's Hospital with endovenous laser ablation therapy (EVLT) for management of dilated embryonic veins in the setting of limb overgrowth. RESULTS: Three patients with limb overgrowth underwent EVLT between 2015 and 2017. All patients had successful occlusion of the targeted veins on post-procedure imaging. One patient developed a cellulitis successfully treated with oral antibiotics. CONCLUSIONS: Endovenous laser ablation therapy is a well-tolerated and safe procedure for prophylactic closure of abnormal superficial embryonic veins. This study supports the use of this technique in managing increased thromboembolic risk in this unique patient population and recognizes the need for a long-term study to determine its efficacy.

Relato de caso: síndrome de Klippel-Trénaunay- Weber / Case Report: Klippel-Trénaunay-Weber syndrome

Introdução: A síndrome de Klippel-Trénaunay-Weber (SKTW) é caracterizada pelo conjunto de sinais que consiste em malformações capilares, malformações venosas com ou sem malformações linfáticas associado ao supercrescimento de membros. Na maioria das vezes, envolve apenas uma extremidade com malformação arteriovenosa e cerca de 75% dos pacientes manifestam antes dos 10 anos de idade. Relato de Caso: Relatamos um caso de Klippel-Trénaunay- Weber em um paciente de 7 meses em acompanhamento na enfermaria da Cirurgia Plástica do Hospital de Clínicas da Universidade Federal de Uberlândia para o qual foi proposto tratamento cirúrgico da lesão. Conclusão: Como a SKTW é uma doença com morbidade progressiva e grave, o paciente deve ser acompanhado em um centro de referência com experiência e arsenal terapêutico diversificado para atuar da melhor forma possível no tratamento.

Introduction: The Klippel-Trénaunay-Weber syndrome (KTWS) is characterized by several signs, including capillary malformations and venous malformations with or without lymphatic malformations associated with limb overgrowth. In most cases, only one extremity is involved with arteriovenous malformation, and approximately 75% of the patients manifest symptoms before 10 years of age. Case Report: We report a case of a 7-month-old patient with KTWS followed-up at the Plastic Surgery Service of the Hospital de Clínicas, Federal University of Uberlândia; surgical treatment of the lesion was proposed for the patient. Conclusion: Since KTWS is a progressive disease with severe morbidity, the patient must be followed-up at a reference center by experienced staff with diverse therapeutic arsenal.

Spinal Cavernous Angioma Associated with Klippel-Trenaunay-Weber Syndrome: Case Report and Literature Review.

BACKGROUND: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital vascular disorder characterized by the classic triad of cutaneous nevi, venous varicosities, and osseous and soft tissue hypertrophy of the affected limb. Various vascular anomalies of the central nervous system have also been described in patients with KTWS. The English language literature to date contains 6 reports of associations between KTWS and spinal cord cavernous angioma (CA), but management of these patients has not been well described. CASE DESCRIPTION: A 23-year-old woman was admitted to our institution with acute onset of leg weakness accompanied by upper back pain. Thoracic magnetic resonance imaging of the spinal cord showed a heterogeneous mass with a slit component at the T1-2 level. The patient underwent left hemilaminectomy followed by removal of the tumor, and her neurologic symptoms improved postoperatively. Pathologic examination showed the spinal lesion was characterized by hemosiderin deposition and thin-walled vascular channels surrounded by fibrous tissue. CONCLUSIONS: This is the first report to provide a detailed pathologic description of the features of spinal CA in a patient with KTWS. Assessment of the clinical features and management of CA associated with KTWS are discussed. This syndrome is rare, and further experience in the treatment of these patients is needed. However, considering that the pathologic findings of spinal CA in patients with KTWS include the typical features of CA, the management of CA in patients with KTWS may be identical to management of isolated CA.

Growing skull hemangioma: first and unique description in a patient with Klippel-Trénaunay-Weber syndrome.

We present the first and unique case of a rapid-growing skull hemangioma in a patient with Klippel-Trénaunay-Weber syndrome. This case report provides evidence that not all rapid-growing, osteolytic skull lesions need to have a malignant character but certainly need a histopathological verification. This material offers insight into the list of rare pathological diagnoses in an infrequent syndrome.

Surgical treatment of varicose veins and venous malformations in Klippel-Trenaunay syndrome.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a mixed mesenchymal malformation characterised by varicose veins, venous and capillary malformations, and hypertrophy of soft tissue and bone. The purpose of this study was to evaluate the surgical outcomes in KTS patients to provide standards for comparison with endovenous therapy. METHODS: The clinical data of consecutive patient with KTS who underwent open venous surgical treatment between January 1987 and December 2008 were reviewed. Demographics, clinical presentation, operative data, and clinical outcomes were recorded. Follow-up information was obtained from the medical records, mailed questionnaires and phone calls. Descriptive statistics, the Kaplan-Meier method and Log-rank statistics were used where appropriate. RESULTS: Twenty-seven females and 22 males, (mean age 26.5 years, range 7.7-55.8) were included in this study. All had varicose veins, 36 (73%) had limb hypertrophy, and 33 (67%) had capillary malformations, with two of three clinical features present in all. The most frequent symptom was pain (N = 43, 88%). Forty-nine patients underwent operations on 53 limbs. Stripping of the GSV, small and accessory saphenous and lateral embryonic veins was performed in 17 (32%), 10 (19%), 9 (17%), and 15 (28%) limbs, respectively. Two patients developed deep vein thrombosis, one had pulmonary embolism (PE), and one patient had peroneal nerve palsy. Freedom from disabling pain at 1, 3 and 5 years was 95%, 77% and 59%, respectively, and freedom from secondary procedures was 78% at 3 years, and 74% at 5 years. At the last follow-up visit, the venous clinical severity score had decreased from 9.48 ± 3.27 to 6.07 ± 3.20 (P < 0.001). CONCLUSIONS: In selected symptomatic patients with KT syndrome, open surgical treatment is safe and durable. Three-fourths of the patients remain free of disabling pain at five years, but secondary procedures are required in one-fourth of the patients. These data can serve as standards for comparison of endovenous therapy for KT syndrome.