Manejo del Síndrome de Klippel-Trenaunay: reporte de caso / Management of Klippel-Trenaunay Syndrome: case report / Manejo da Síndrome de Klippel-Trenaunay: relato de caso

El Síndrome de Klippel-Trenaunay se constituye en una malformación vascular compleja con una incidencia de 2 a 3 casos por cada 100.000 nacidos vivos, clínicamente presenta una triada clásica: manchas cutáneas en vino de Oporto, venas varicosas de localización atípica e hipertrofia ósea y de tejidos blandos. Se presenta el caso de una paciente femenina de 33 años, sin antecedentes patológicos o quirúrgicos de importancia quien acude a consulta por aumento de volumen de la extremidad derecha, mancha violácea ipsilateral, además de dolor y parestesias. Al examen físico se evidencia aumento longitudinal de miembro inferior derecho, nevus hiperpigmentario en cara lateral de pierna derecha y muslo que se extiende al glúteo ipsilateral además de venas varicosas atípicas. Se practica ecografía Doppler venosa con transductor lineal que reporta incompetencia de vena Safena Mayor y perforantes suprageniculares. Se realizó manejo quirúrgico mediante safenectomía, corrección de deformidad y referencia a dermatología para terapia láser por el nevus hiperpigmentario. La paciente mostró evolución clínico - quirúrgica favorable con remisión de la sintomatología que motivó su consulta.

Klippel-Trenaunay syndrome is a complex vascular malformation with an incidence of 2 to 3 cases per 100,000 live births. Clinically, it presents a classic triad: port-wine stains, varicose veins of atypical location and bone and soft tissue hypertrophy. We present the case of a 33-year-old female patient, with no pathologic or surgical history of importance, who comes to the clinic for an increase in volume of the right extremity, ipsilateral violaceous spot, in addition to pain and paresthesia. Physical examination revealed longitudinal enlargement of the right lower limb, hyperpigmented nevus on the lateral aspect of the right leg and thigh extending to the ipsilateral buttock and atypical varicose veins. Venous Doppler ultrasound with linear transducer reported incompetence of the greater saphenous vein and supragenicular perforators. Surgical management was performed by saphenectomy, deformity correction and referral to dermatology for laser therapy for hyperpigmented nevus. The patient showed favorable clinical-surgical evolution with remission of the symptomatology that motivated her consultation.

A síndrome de Klippel-Trenaunay é uma malformação vascular complexa com uma incidência de 2 a 3 casos por 100.000 nascidos vivos. Clinicamente, apresenta uma tríade clássica: manchas vinho do porto, veias varicosas de localização atípica e hipertrofia óssea e de tecidos moles. Apresentamos o caso de uma paciente do sexo feminino, 33 anos, sem histórico patológico ou cirúrgico de importância, que consultou por aumento de volume do membro direito, mancha violácea ipsilateral, além de dor e parestesia. O exame físico revelou aumento longitudinal do membro inferior direito, nevo hiperpigmentado na face lateral da perna e coxa direitas, estendendo-se até a nádega ipsilateral e veias varicosas atípicas. Foi realizado ultrassom Doppler venoso com transdutor linear, que relatou incompetência da veia safena magna e das perfurantes suprageniculares. O tratamento cirúrgico foi realizado por meio de safenectomia, correção da deformidade e encaminhamento à dermatologia para terapia a laser para nevo hiperpigmentado. A paciente apresentou uma evolução clínico-cirúrgica favorável com remissão dos sintomas que a levaram à consulta.

Síndrome de Klippel Trenaunay y embarazo. Reporte de tres casos / Klippel-Trenaunay syndrome and pregnancy. Report of three cases

Resumen ANTECEDENTES: El síndrome de Klippel Trenaunay es una enfermedad congénita rara, caracterizada por malformaciones capilares y venosas, sobrecrecimiento de miembros y en algunos casos malformaciones linfáticas. Quienes lo padecen tienen un riesgo incrementado de hemorragia y tromboembolismo. CASOS CLÍNICOS: Tres pacientes primigestas con diagnóstico de síndrome de Klippel Trenaunay con complicaciones del embarazo que finalizaron mediante cesárea con el nacimiento de sus hijos sanos, de término, en todos los casos. CONCLUSIONES: Las embarazadas y con síndrome de Klippel-Trenaunay tienen un riesgo significativo de que sus síntomas se agraven, de tener hemorragia durante el nacimiento y de eventos tromboembólicos, incluso después del nacimiento. La atención individualizada y multidisciplinaria ayudará a mitigar las complicaciones asociadas y a conseguir desenlaces óptimos.

Abstract BACKGROUND: Klippel Trenaunay syndrome is a rare congenital disease characterized by capillary and venous malformations, limb overgrowth and in some cases lymphatic malformations. Sufferers have an increased risk of hemorrhage and thromboembolism. CLINICAL CASES: Three primigravid patients diagnosed with Klippel Trenaunay syndrome with pregnancy complications that were terminated by cesarean section with the birth of their healthy, full-term children in all cases. CONCLUSIONS: Women with Klippel-Trenaunay syndrome and pregnancy are at significant risk for aggravation of their symptoms, hemorrhage during birth, and thromboembolic events, even after birth. Individualized, multidisciplinary care will help mitigate associated complications and achieve optimal outcomes.

Síndrome Klippel-Trenaunay en el embarazo. Reporte de un caso / Klippel-Trenaunay syndrome in pregnancy. Case report

Resumen ANTECEDENTES: El síndrome Klippel-Trenaunay es neurocutáneo, con repercusión vascular. La triada característica la integran: nevo vascular cutáneo, venas varicosas e hipertrofia asimétrica de los tejidos blandos y huesos que afectan una o más extremidades. Durante el embarazo estas malformaciones se incrementan, con afectación pélvica e intraabdominal. En la bibliografía internacional están reportados menos de 100 casos de embarazos complicados con este síndrome. CASO CLÍNICO: Paciente de 16 años, primigesta, con síndrome Klippel-Trenaunay diagnosticado a los 15 años, enviada a nuestra unidad, en el tercer trimestre del embarazo, para finalización de éste. Se le practicaron estudios preoperatorios: biometría hemática, pruebas de coagulación, reportados sin alteraciones. El ultrasonograma Doppler del segmento uterino sin incremento en la vasculatura. Se programó para cesárea, que se llevó a cabo sin complicaciones intraoperatorias, con sangrado de 600 cc, incremento de la vascularidad en el colon. Permaneció en vigilancia durante 48 horas, luego de la operación, en cuidados intensivos, sin complicaciones hemorrágicas o isquémicas. Se dio de alta del hospital a las 72 horas, con tromboprofilaxis, analgésico y antibiótico. CONCLUSIÓN: El embarazo en pacientes con síndrome de Klippel-Trenaunay implica una elevada morbilidad y mortalidad, que pueden prevenirse con atención multidisciplinaria que disminuya las potenciales complicaciones.

Abstract BACKGROUND: Klippel-Trenaunay syndrome is a neurocutaneous syndrome with vascular repercussion whose characteristic triad is a cutaneous vascular nevus, varicose veins and asymmetric soft tissue and bone hypertrophy, which affect one or more limbs, during pregnancy these malformations increase, with pelvic and intra-abdominal repercussion. In the world literature, fewer than 100 cases of complicated pregnancies with this syndrome have been reported. OBJECTIVE: The second case of complicated pregnancy with Klippel-Trenaunay syndrome treated in our institution is reported, given its high morbidity, due to the high risk of severe complications such as venous thromboembolism or excessive bleeding in the intrapartum period. CLINICAL CASE: A 16-year-old, with a Klippel-Trenaunay syndrome diagnosed at fifteen, sent to our unit, with a third-trimester pregnancy, for resolution of pregnancy. Preoperative studies were performed blood count, coagulation tests, reported without alterations; as well as Doppler ultrasound of the uterine segment, without an increase in vasculature. It is scheduled for caesarean section, which is performed without intraoperative complications, with total bleeding of 600 cc, with an increase in vascularity at the colon level. She remains in immediate postoperative surveillance for 48 hours in an intensive care unit, without presenting haemorrhagic or ischemic complications. She is discharged at 72 hours, with thromboprophylaxis, analgesic and antibiotic. CONCLUSION: Pregnancy in patients with this syndrome implies high morbidity and mortality, which can be prevented with multidisciplinary management, anticipating potential complications.

Conservative management of knee arthropathy in a patient with Klippel Trenaunay syndrome / Manejo conservador da artropatia do joelho em paciente com síndrome de Klippel-Trenaunay

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.

Resumo A síndrome de Klippel-Trenaunay (SKT) é uma malformação vascular rara caracterizada por malformação capilar, malformações venosas e hipertrofia de tecidos moles ou ósseos que afetam as extremidades na maioria dos casos. A artropatia do joelho ou do quadril é uma condição comumente associada e causa sérias deficiências. Apresentamos o caso de um paciente com diagnóstico de SKT e artropatia grave do joelho. Um homem de 34 anos com SKT foi encaminhado ao nosso hospital com artropatia grave do joelho com articulação fixa na posição de 90 °. A angiotomografia e a ressonância magnética da perna esquerda mostraram importante desenvolvimento varicoso do sistema venoso superficial com vasos intra-articulares. Após o caso ser discutido em um comitê multidisciplinar, o paciente foi incluído em um programa de fisioterapia, obtendo uma melhora significativa nos movimentos e na qualidade de vida após 12 meses de acompanhamento. O tratamento da SKT é principalmente conservador e exige uma abordagem multidisciplinar.

Manejo anestésico para la cesárea en una paciente con síndrome de Klippel-Trenaunay. Reporte de un caso / Anesthetic management for cesarean section in a patient with Klippel-Trenaunay syndrome. Report of a case

Klippel-Trenaunay syndrome is a rare disease that includes the triad of malformation of vascular capillaries, varicose veins and/or venous malformation and hypertrophy of soft or bony tissues. Its presence during pregnancy poses an anesthetic challenge due to the high obstetric risk due to thromboembolic and/or hemorrhagic phenomena. We present the case of a pregnant woman with the diagnosis of Klippel-Trenaunay syndrome announced for elective cesarean section. Our objective is to expose the anesthetic management carried out and to propose a strategic guide for anesthesiologists.

El síndrome de Klippel-Trenaunay es una rara enfermedad que comprende la tríada de malformación de capilares vasculares, venas varicosas y/o malformación venosa e hipertrofia de tejidos blandos u óseos. Su presencia durante la gestación supone un reto anestésico por el alto riesgo obstétrico debido a fenómenos tromboembólicos y/o hemorrágicos. Presentamos el caso de una embarazada con el diagnóstico de síndrome de Klippel-Trenaunay anunciada para cesárea electiva. Nuestro objetivo es exponer el manejo anestésico realizado y proponer una guía estratégica para los anestesiólogo

Anesthesia for cesarean delivery in a patient with Klippel-Trenaunay syndrome / Anestesia para parto cesáreo em paciente portadora de síndrome de Klippel-Trenaunay

Abstract Introduction: Klippel-Trenaunay syndrome is a rare congenital vascular disease characterized by cutaneous hemangiomas, varicosities, and limb asymmetry, which may evolve with coagulation disorders and hemorrhage as those more frequent complications in pregnant patients. Pregnancy is not advised in women with this syndrome due to increased obstetrical risk. Case report: Female patient, 29 years old, 99 kg, 167 cm, BMI 35.4 kg.m−2, physical status ASA III, with 27 weeks of gestational age and diagnosis of Klippel-Trenaunay syndrome. She was admitted to attempt inhibition of preterm labor. As manifestations of Klippel-Trenaunay syndrome, the patient presented with cerebral and cutaneous hemangioma mainly in the trunk and lumbar region, paresis in the left upper and lower limbs, and limb asymmetry requiring the use of a walking stick. Physical examination revealed absence of airway vascular malformations and Mallampati class 3. Laboratory tests were normal and abdominal angiotomography showed irregular uterus, with multiple varices and vessels of arterial origin and bilateral periadnexal varices. She evolved with failure in preterm labor inhibition, and cesarean section under total intravenous anesthesia was indicated. Monitoring, central and peripheral venous access, radial artery catheterization, and diuresis were secured. Cesarean section was performed with median incision and longitudinal uterine body section for fetal extraction. Two episodes of arterial hypotension were seen intraoperatively. The postoperative evolution was uneventful. The choice of anesthesia was dependent on the clinical manifestations and the lack of imaging tests proving the absence of neuraxial hemangiomas.

Resumo Introdução: A síndrome de Klippel-Trenaunay é uma doença vascular congênita rara caracterizada por hemangiomas cutâneos, varicosidades e assimetria de membros, que pode evoluir com distúrbios de coagulação e hemorragia como complicações mais frequentes na paciente grávida. A gestação é desaconselhada nas mulheres portadoras dessa síndrome devido ao aumentado risco obstétrico. Relato de caso: Paciente com 29 anos, 99 kg e 167 cm, IMC 35,4 kg.m-2, estado físico ASA III, com 27 semanas de idade gestacional, com diagnóstico de síndrome de Klippel-Trenaunay, foi internada para tentativa de inibição de trabalho de parto prematuro. Como manifestações da síndrome de Klippel-Trenaunay apresentava hemangiomas cerebral e cutâneos, principalmente em tronco e região lombar, paresia em membros superior e inferior esquerdos e assimetria de membros, necessitando de bengala para locomoção. Ao exame físico: ausência de malformações vasculares em vias aéreas, escore 3 pela classificação de Mallampati; exames laboratoriais normais; angiotomografia computadorizada de abdômen mostrava útero irregular, com múltiplas varizes e vasos de permeio de origem arterial e varizes perianexiais bilaterais. Evoluiu com falha de inibição do trabalho de parto prematuro e foi indicada a cesariana sob anestesia geral venosa total. Monitoração, acesso venoso central e periférico, cateterização de artéria radial, diurese. Operação cesariana com incisão mediana e secção corporal longitudinal uterina para extração fetal. No intraoperatório, observaram-se dois episódios de hipotensão arterial. A evolução pós-operatória seguiu sem complicações ou intercorrências. A escolha da anestesia dependeu das manifestações clínicas e da falta de exames de imagem que comprovassem a ausência de hemangiomas no neuroeixo.

Klippel-Trenaunay syndrome complicated by spinal arteriovenous fistula and subarachnoid hemorrhage: report of one case with literature review / 介入放射学杂志

Objective To discuss the clinical value of interventional treatment for Klippel-Trenaunay syndrome complicated by spinal arteriovenous fistula and subarachnoid hemorrhage, and to report 1 patients with this disease who were successfully treated with interventional treatment. Methods One female children patient with clinically confirmed Klippel-Trenaunay syndrome complicated by spinal arteriovenous fistula and subarachnoid hemorrhage were enrolled in this study, who was underwent the embolization of arteriovenous fistula in the spinal. Results The interventional procedure was successfully accomplished in this case. The patient's clinical symptoms was disappeared gradually. No complications occurred. Conclusion For the treatment of Klippel-Trenaunay syndrome complicated by spinal arteriovenous fistula and subarachnoid hemorrhage, interventional management is minimally-invasive, safe and Reliable.

Phacomatosis Pigmentovascularis Type II: Case Report

Phacomatosis pigmentovascularis is a rare syndrome first described by Ota et al, in 1947. It ischaracterized by a combination of capillary malformation and other pigmented naevi. It had originallybeen classified into four major types. A fifth type, in which the vascular lesion is cutis marmoratatelangiectatica congenita (CMTC), was subsequently added. Each type was further categorizedaccording to the absence or presence of associated extra-cutaneous signs as types (a) and (b),respectively. We reported this case due to its rare clinical presentation, with probable overlap Klippel-Trenaunay syndrome.

Klippel-Trenaunay and Parkes-Weber syndromes: two case reports / Síndromes de Klippel-Trenaunay e Parkes-Weber: dois relatos de caso

Abstract Parkes-Weber syndrome is a congenital vascular disease that comprises capillary, venous, lymphatic, and arteriovenous malformations. Although Parkes-Weber syndrome is a clinically distinct entity with serious complications, it is still frequently misdiagnosed as Klippel-Trenaunay syndrome, which consists of a triad of malformations involving the capillary, venous, and lymphatic vessels, without arteriovenous fistulas. Both syndromes are generally diagnosed with Doppler ultrasound and confirmed by magnetic resonance angiography. The aim of this study is to describe one case of Klippel-Trenaunay syndrome, in a 36-year-old patient, and one case of Parkes-Weber syndrome, in a 21-year-old patient. We review the literature in order to discuss the possible causes and consequences of these diseases related to venous hypertension and angiodysplasia, taking a clearer approach to their differences, and discussing their treatment.

Resumo A síndrome de Parkes-Weber é uma doença vascular congênita que consiste em malformações capilares, venosas, linfáticas e arteriovenosas. Embora seja uma entidade clinicamente distinta com complicações graves, essa síndrome ainda é frequentemente diagnosticada erroneamente como síndrome de Klippel-Trenaunay, que consiste em uma tríade de má formação nos vasos capilares, venosos e linfáticos, sem fístula arteriovenosa. Ambas as síndromes são geralmente diagnosticadas através de ultrassom Doppler e confirmadas pela angiografia por ressonância magnética. O objetivo deste estudo é descrever um caso de síndrome de Klippel-Trenaunay em um paciente de 36 anos de idade e um caso de síndrome de Parkes-Weber em uma paciente de 21 anos. A literatura foi revisada com o objetivo de discutir as possíveis causas e consequências dessa doença e sua associação à hipertensão venosa e angiodisplasia. O presente trabalho também levanta discussão a respeito das diferenças sintomatológicas de ambas as síndromes e seus respectivos tratamentos.

MR TRICKS technique application in lower extremity venous diseases of Klippel-Trenaunay syndrome / 实用放射学杂志

Objective To explore the clinical value of magnetic resonance time-resolved imaging of contrast kinetics (MR-TRICKS)in the assessment of lower extremity venous lesions in Klippel-Trenaunay syndrome (KTS).Methods Image data of 20 patients (total 21 affected limbs)diagnosed with KTS in our hospital were analyzed retrospectively.All patients underwent MR-TRICKS angiography and ascending phlebography of the lower extremities.The images acquired by these two methods were graded and compared.Kappa test was used to examine the consistency between the results of two inspection methods.Results The image quality of MR-TRICKS angiography and ascending phlebography graded on excellent proportion were 85.71％(18/21)and 80.95％(17/21)for the deep veins,95.24％(20/21)and 90.48％(19/21)for the superficial veins,and 90.48％(19/21)and 85.71％(18/21) for venous malformation,respectively.MR-TRICKS demonstrated that the deep veins were normal in 61.90％ (13/21),hypogenetic in 25.57％(7/21),atretic in 4.86％(1/21).Superficial vein diseases including varicosis of the great saphenous vein accounted for 25.57％(6/21), varicosis of the small saphenous vein accounted for 14.29％ (3/21 ),while marginal veins in venous malformation accounted for 100％ (21/21).Ascending phlebography of the lower limbs showed that the deep veins were normal in 57.14％(12/21)and dysplasia in 39.10％(8/21),while the other results were consistent with those of TRICKS.Consistency between results from the two methods were excellent (Kappa >0.75).Conclusion MR-TRICKS can accurately describe the angioarchitecture of the lower extremity vena in patients with KTS,and has an important clinical value in providing support for the assessment and treatment of KTS.

A Case of Klippel-Trenaunay Syndrome with Microcystic Lymphatic Malformation on Anus / 대한피부과학회지

No abstract available.

MR TRICKS technique application in lower extremity venous diseases of Klippel-Trenaunay syndrome / 实用放射学杂志

Objective To explore the clinical value of magnetic resonance time-resolved imaging of contrast kinetics (MR-TRICKS)in the assessment of lower extremity venous lesions in Klippel-Trenaunay syndrome (KTS).Methods Image data of 20 patients (total 21 affected limbs)diagnosed with KTS in our hospital were analyzed retrospectively.All patients underwent MR-TRICKS angiography and ascending phlebography of the lower extremities.The images acquired by these two methods were graded and compared.Kappa test was used to examine the consistency between the results of two inspection methods.Results The image quality of MR-TRICKS angiography and ascending phlebography graded on excellent proportion were 85.71％(18/21)and 80.95％(17/21)for the deep veins,95.24％(20/21)and 90.48％(19/21)for the superficial veins,and 90.48％(19/21)and 85.71％(18/21) for venous malformation,respectively.MR-TRICKS demonstrated that the deep veins were normal in 61.90％ (13/21),hypogenetic in 25.57％(7/21),atretic in 4.86％(1/21).Superficial vein diseases including varicosis of the great saphenous vein accounted for 25.57％(6/21), varicosis of the small saphenous vein accounted for 14.29％ (3/21 ),while marginal veins in venous malformation accounted for 100％ (21/21).Ascending phlebography of the lower limbs showed that the deep veins were normal in 57.14％(12/21)and dysplasia in 39.10％(8/21),while the other results were consistent with those of TRICKS.Consistency between results from the two methods were excellent (Kappa >0.75).Conclusion MR-TRICKS can accurately describe the angioarchitecture of the lower extremity vena in patients with KTS,and has an important clinical value in providing support for the assessment and treatment of KTS.

Síndrome de Parkes Weber en una infante / Parkes Weber syndrome in a child

Se describe el caso clínico de una infante de 4 años de edad atendida inicialmente en el Servicio de Pediatría del Hospital el Instituto Ecuatoriano de Seguridad Social de Ibarra, de la Repúblicade Ecuador y luego en el de Cirugía Vascular, donde se le diagnosticó síndrome de Parkes Weber.

The case report of a 4 years child is described assisted initially in the Pediatrics Service of the Social Security Ecuadorian Institute Hospital of Ibarra in Ecuador Republic and then in the Vascular Surgery Service, where she was diagnosed Parkes Weber syndrome.

Síndrome de Klippel Trenaunay / Klippel-Trenaunay Syndrome

El Síndrome de Klippel Trenaunay es una malformación vascular congénita caracterizada por una triada de manifestaciones clínicas que comprende una malformación vascular venular, linfática y venosa, junto con hipertrofia esquelética y aumento de tejidos blandos de uno o más miembros. De etiología desconocida, patogénicamente se cree que se produce una alteración en el desarrollo del mesodermo en el feto, que afecta a las líneas angioblástica, linfoblástica y osteoblástica. Se presenta el caso de un paciente femenino de 5 años de edad quien presenta una malformación vascular, diagnosticada inicialmente como hemangioma vascular y posteriormente se asoció a Síndrome de Klippel Trenaunay. El objetivo de la presentación de este caso es generar conocimiento sobre las características clínicas del síndrome de Klippel Trenaunay para un diagnóstico y tratamiento oportuno(AU)

Klippel - Trenaunay -Weber congenital vascular malformation characterized by a triad of clinical manifestations comprising a venular, venous and lymphatic vascular malformation, with hypertrophy increased skeletal and soft tissue of one or more members. Of unknown etiology, it is believed to pathogenically an alteration occurs in mesoderm development in the fetus, which affects angioblastic , and osteoblastic lymphoblastoid lines. Is presentedthe case of a 5 year old that has a vascular malformation, initially diagnosed as vascular hemangioma and later was associated with Klippel- Trenaunay -Weber.Objective: To generate knowledge about the clinical features of KlippelTrenaunay Syndrome for diagnosis and treatment(AU)

Síndrome de Klippel-Trenaunay-Weber en una gestante / Klippel-Trenaunay-Weber syndrome in a pregnant woma

El síndrome de Klippel-Trenaunay-Weber o síndrome de angiosteohipertrofia es una enfermedad congénita rara, caracterizada por la presencia de un nevo en el miembro derecho y grandes venas varicosas. Se presenta el caso clínico de una gestante con dicho síndrome, a quien se le realizó la operación cesárea a las 37,4 semanas, sin complicaciones peroperatorias, en el Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba. Luego de un examen clínico exhaustivo al recién nacido, se determinó que no presentaba las manifestaciones clínicas de esta afección. Se obtuvo el consentimiento de la paciente para la presentación del caso y la toma de imágenes.

The syndrome Klippel-Trenaunay-Weber syndrome or angiosteo hypertrophy syndrome is a strange congenital disease characterized by the presence of a nevus in the right member and big varicose veins. The case report of a pregnant woman with this syndrome is presented, who underwent the cesarean section at 37.4 weeks, without perioperative complications, in Dr. Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba. After an exhaustive clinical exam to the newborn, it was determined that he didn't present the clinical manifestations of this disorder. The patient's consent was obtained for the case presentation and images taking.

Recurrent Myelopathy in a Patient with Klippel-Trenaunay Syndrome / 대한임상신경생리학회지

Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation syndrome involving blood and lymph vessels, which is characterized by triad of cutaneous hemangioma, venous varicosities, and overgrowth of the affected limbs. Because vascular malformation in KTS can be located anywhere except the face and brain, the clinical presentation could be extremely variable. But there are only rare case reports that KTS is associated with spinal cord lesion. We report a case of recurrent myelopathy in a patient with KTS.

A Case of Klippel-Trenaunay Syndrome with Acute Submassive Pulmonary Thromboembolism Treated with Thrombolytic Therapy

Klippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by varicose veins, cutaneous hemangiomas, soft tissue and bony hypertrophy of limb. Potential complications such as deep venous thrombosis and pulmonary thromboembolism have not been reported in Korea to date. We demonstrate the case of a 48-year-old woman with Klippel-Trenaunay syndrome with extensive varicose veins on right lower limb, hypertrophy of left big toe and basilar artery tip aneurysm, complicated with acute submassive pulmonary thromboembolism treated successfully with intravenous thrombolytic therapy.

Sturge-Weber syndrome in association with Klippel-Trenaunay syndrome and phakomatosis pigmentovascularis type IIb.

Phakomatosis pigmentovascularis (PPV) is a rare combination of pigmentary and vascular components with or without systemic involvement. We report here a rare association of Sturge-Weber syndrome, Klippel-Trenaunay syndrome, and PPV type IIb in a 15-year-old boy who had right upper limb monoparesis along with a history of recurrent convulsions.

Síndrome de Klippel-Trénaunay: a propósito de un caso en un lactante / Klippel-Trénaunay syndrome: a case report in an infant

Se presenta el caso de un lactante, con una extensa mancha en vino de oporto y deformación del miembro pélvico derecho, a expensas de tejido blando sin afección ósea compatible con el síndrome de Klippel-Trénaunay; no se observan las alteraciones venosas clásicas por su temprana edad.

We report the case of an infant with a large port-wine stain and right pelvic limb deformation, at the expense of soft tissue without bone involvement compatible with Klippel-Trénaunay syndrome, no venous abnormalities was observed, typical for his early age.