Assuntos
Síndrome de Klippel-Trenaunay-Weber , Doenças Vasculares , Malformações Vasculares , Feminino , Humanos , Síndrome de Klippel-Trenaunay-Weber/complicações , Síndrome de Klippel-Trenaunay-Weber/diagnóstico por imagem , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Sirolimo/uso terapêutico , Malformações Vasculares/complicações , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/tratamento farmacológicoRESUMO
RATIONALE: Klippel-Trenaunay Syndrome (KTS) is a congenital vascular disease characterized by cutaneous hemangiomas, venous varicosities, and limb hypertrophy. Although extremely rare in pregnant women, the present vascular alterations may be aggravated, consequent to postural and hormonal changes inherent to the pregnancy. Pregnancy is not advised in KTS women due to increased obstetrical risk. PATIENT CONCERNS: A 31-year-old pregnancy woman presented with prominent vascularity in pelvis, right lower limb, spleen, and liver at 28 weeks of gestation. We started administration of anticoagulant therapy and obstetrics management. DIAGNOSIS: MRI and ultrasound revealed that multiple varicosities in her pelvis, right lower limb, spleen, and liver. She was diagnosed with KTS. INTERVENTIONS: At her first visit at 28 weeks of gestation, multidisciplinary evaluation had been done. Blood transfusion and iron supplement had been given for anemia correction. Anticoagulant therapy was performed to prevent potential thrombus risk. She had a vaginal delivery with a healthy newborn in her second visit without any complications at the gestation of 36 weeks due to rupture of preterm membranes. OUTCOMES: After successful management, the patient was discharged without any complications 2 days after vaginal delivery. No symptoms of hemorrhage or thrombus were observed. At 6 months follow-up, her right lower toes enlarged obviously, MRI revealed that no obvious changes of hemangiomas was found compared to those during the pregnancy and ultrasound revealed that there was no thrombus in her right lower limb. LESSONS: Patients with KTS can be pregnant and have healthy babies safely with regularly monitor and reasonable treatment during pregnancy. A careful follow-up and guidance are necessary.
Assuntos
Anticoagulantes/uso terapêutico , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Complicações Cardiovasculares na Gravidez/tratamento farmacológico , Adulto , Feminino , Humanos , Recém-Nascido , Nascido Vivo , GravidezRESUMO
CONTEXT: Klippel-Trénaunay syndrome (KTS) is a very rare congenital vascular anomaly. It is characterized by the presence of capillary malformation, venous malformation as well as limb overgrowth, generally affecting one extremity. Although clinical characteristics of KTS are well known, the epidemiology and pathophysiology still remain to be defined. Awareness of these disorders is important for rural physicians for managing potential complications. ISSUE: A 60-year-old male presented with symptomatic varicosities and chronic venous insufficiency of the left leg. The patient had a history of several episodes of ulceration and thrombophlebitis on the left side. Physical examination revealed extensive varicosities and a large port-wine stain on the lateral side of the left leg, despite a previous ligation of the saphenofemoral junction. The left leg was circumferentially larger than the right leg. Additional investigations were conducted to rule out arterial disorders and confirm the venous nature of the abnormalities. The patient was clinically diagnosed with KTS and his varicosities were treated surgically using phlebectomy with stab avulsion technique. LESSONS LEARNED: KTS is a distinct entity that belongs to the vascular malformations group. In a rural practice, identification and accurate diagnosis are challenging, as these patients often require a multidisciplinary approach and do not present until later in the course of the condition. An understanding of the correct nomenclature and associated complications is imperative for proper assessment and management in rural and remote settings.
Assuntos
Anticoagulantes/uso terapêutico , Aspirina/uso terapêutico , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Síndrome de Klippel-Trenaunay-Weber/fisiopatologia , Guias de Prática Clínica como Assunto , Serviços de Saúde Rural/normas , Humanos , Masculino , Pessoa de Meia-Idade , Terra Nova e Labrador , Doenças Raras/diagnóstico , Doenças Raras/tratamento farmacológico , Resultado do TratamentoAssuntos
Colo/patologia , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/patologia , Síndrome de Klippel-Trenaunay-Weber/complicações , Síndrome de Klippel-Trenaunay-Weber/patologia , Reto/patologia , Antifibrinolíticos/uso terapêutico , Criança , Colonoscopia , Feminino , Hemorragia Gastrointestinal/tratamento farmacológico , Humanos , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Ácido Tranexâmico/uso terapêuticoRESUMO
The purpose of this study was to describe the epidemio-clinical profile, and treatment of vascular cutaneous abnormalities in Togo. It was a retrospective study of patients recorded in dermatology for vascular cutaneous abnormality between 1998 and 2017. During the study period, 120 (0.1%) of 88,869 patients received in dermatology have consulted for vascular cutaneous abnormalities. Their mean age was 25.3 months and the sex-ratio (M/F) 0.4. The most recurring vascular cutaneous abnormalities were vascular tumors (97 cases; 80.8%), mainly infantile hemangioma (IH) (93 cases; 77.5%). The mean time of onset of IH after birth was 2.9 months. The IH (54 cases; 50.5%) and port wine stains (8 cases, 53.3%) were predominantly localized at the cephalic region and Klippel-Trenaunay syndrome cases on lower limbs. We opted for a therapeutic abstention in the majority of the patients (50 cases of IH, 16 cases of simple vascular abnormalities, all Klippel-Trenaunay syndrome cases and telegiectasia cases). Of the 43 IH cases treated, the main drugs used were corticosteroids (23 cases of which 17 between 1998 and 2011 and 6 from 2012) followed by propranolol (11 cases from 2012). We had a favorable response in 9 of the 15 patients seen again. Cutaneous vascular abnormalities are very rare in dermatology in Togo and are dominated by IH with female predominance. In IH treatments, propranolol use, started in 2012 in Togo, is increasing when corticotherapy has declined.
L'objectif de cette étude était de décrire le profil épidémioclinique et thérapeutique des anomalies vasculaires cutanées au Togo. Il s'agit d'une étude rétrospective portant sur les dossiers des patients reçus en dermatologie pour une anomalie vasculaire cutanée entre 1998 et 2017. Durant la période d'étude, 120 (0,1 %) des 88 869 patients reçus en dermatologie ont consulté pour une anomalie vasculaire cutanée. L'âge moyen des patients était de 25,3 mois et le sex-ratio (H/F) de 0,4. Les anomalies vasculaires les plus recensées étaient les tumeurs vasculaires (97 cas ; 80,8 %), principalement les hémangiomes infantiles (HI) [93 cas ; 77,5 %]. Le délai moyen d'apparition des HI après la naissance était de 2,9 mois. Les HI (54 cas ; 50,5 %) et les angiomes plans (8 cas, 53,3 %) étaient localisés de façon prépondérante à la région céphalique et les cas de syndrome de Klippel-Trenaunay aux membres inférieurs. Nous avons opté pour une abstention thérapeutique chez la plupart des patients (50 cas d'HI, 16 cas de malformations vasculaires simples, tous les cas de syndrome de Klippel-Trenaunay et le cas de télangiectasies). Sur les 43 cas d'HI traités, les principaux médicaments utilisés étaient les corticoïdes (23 cas dont 17 entre 19982011 et 6 à partir de 2012) suivis du propranolol (11 cas à partir de 2012). Nous avions noté une réponse favorable chez 9 des 15 patients revus. Les anomalies vasculaires cutanées sont très rares en dermatologie au Togo et sont dominées par les HI, avec une prédominance féminine. Dans le traitement des HI, l'usage du propranolol, débuté en 2012 au Togo, est en augmentation pendant que celui de la corticothérapie baisse.
Assuntos
Dermatopatias Vasculares , Corticosteroides/uso terapêutico , Pré-Escolar , Estudos de Coortes , Feminino , Hemangioma/diagnóstico , Hemangioma/tratamento farmacológico , Hemangioma/epidemiologia , Humanos , Lactente , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Síndrome de Klippel-Trenaunay-Weber/epidemiologia , Masculino , Propranolol/uso terapêutico , Estudos Retrospectivos , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/tratamento farmacológico , Dermatopatias Vasculares/epidemiologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/epidemiologia , Togo/epidemiologiaRESUMO
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)
Assuntos
Humanos , Feminino , Criança , Propranolol/uso terapêutico , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Síndrome de Klippel-Trenaunay-Weber/etiologia , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Angiografia por Ressonância Magnética/métodos , Meias de CompressãoRESUMO
Lymphatic malformations (LMs) are congenital lymphatic lesions that impose significant and costly morbidities on affected patients. Treatment options are limited due to incomplete understanding of LM pathobiology. Expression of an activated ß2-adrenergic receptor has been described in LM tissue, suggesting that this pathway may contribute to the clinical manifestations of LM. We hypothesized that propranolol, a ß-adrenergic receptor antagonist, might improve symptoms of patients with LMs and lymphatic anomalies. A retrospective chart review of patients treated with propranolol as an adjunct therapy was conducted; analyses included demographic characteristics, clinical features, and response to propranolol. Three patients with cystic and noncystic LMs displayed clinical improvement at a minimum dose of 0.7 mg/kg/d, whereas symptomatic relapses were observed when propranolol doses dropped below this threshold. Two patients with Klippel-Trenaunay syndrome demonstrated partial clinical responses with reduced edema. The fetus of a mother treated with propranolol from a gestational age of 35 weeks through delivery displayed arrested growth of a cervicofacial LM. Our retrospective review suggests that propranolol improved symptoms in a subset of patients with lymphatic anomalies. Propranolol treatment may also limit the growth of congenital LMs in utero.
Assuntos
Antagonistas Adrenérgicos beta/uso terapêutico , Anormalidades Linfáticas/tratamento farmacológico , Propranolol/uso terapêutico , Criança , Pré-Escolar , Esquema de Medicação , Feminino , Terapias Fetais , Humanos , Lactente , Recém-Nascido , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Anormalidades Linfáticas/diagnóstico , Masculino , Gravidez , Diagnóstico Pré-Natal , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemAssuntos
Anticoagulantes/administração & dosagem , Malformações Arteriovenosas/diagnóstico por imagem , Heparina/administração & dosagem , Síndrome de Klippel-Trenaunay-Weber/diagnóstico por imagem , Extremidade Inferior/irrigação sanguínea , Veia Poplítea/fisiopatologia , Adulto , Feminino , Humanos , Síndrome de Klippel-Trenaunay-Weber/complicações , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Veia Poplítea/diagnóstico por imagem , Mancha Vinho do Porto , Radiografia , Resultado do Tratamento , Ultrassonografia Doppler DuplaRESUMO
Abstract The ß-blocker propranolol has become a valuable and effective drug for the treatment of infantile hemangiomas. Its therapeutic action probably results from vasoconstriction, blocking of angiogenesis through effects on vascular endothelial growth factor and induction of apoptosis. It is reasonable to suggest that propranolol can also be used effectively in the treatment of other vascular abnormalities. This case report describes propranolol treatment of vascular malformations such as Klippel-Trénaunay syndrome or Parkes-Weber syndrome in adults.
Assuntos
Antagonistas Adrenérgicos beta/uso terapêutico , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Propranolol/uso terapêutico , Administração Oral , Antagonistas Adrenérgicos beta/administração & dosagem , Adulto , Feminino , Humanos , Síndrome de Klippel-Trenaunay-Weber/fisiopatologia , Propranolol/administração & dosagem , Resultado do Tratamento , Fator A de Crescimento do Endotélio Vascular/antagonistas & inibidores , Malformações Vasculares/tratamento farmacológico , Malformações Vasculares/fisiopatologiaRESUMO
Cerebrovascular manifestations in Klippel-Trenaunay Syndrome (KTS) have been reported but are extremely rare. Case reports describe brain embolism in KTS-associated coagulopathy as well as bleeding due to arteriovenous malformations. We describe a 45-year-old patient with KTS and both acute ischemic stroke and repeated cerebral haemorrhage. The underlying aetiology of both events remained undetermined despite extensive diagnostic work-up, including coagulation tests and dynamic MR angiography. It is most likely that both a pathological coagulation and increased vessel fragility comparable to amyloid angiopathy were responsible for the combined brain lesions in this patient. We conclude that KTS is a very rare but relevant aetiology of cerebral ischemia and that anticoagulation treatment in these cases should be carefully considered as the risk of cerebral haemorrhage is probably elevated.
Assuntos
Hemorragias Intracranianas/complicações , Síndrome de Klippel-Trenaunay-Weber/complicações , Acidente Vascular Cerebral/complicações , Anti-Hipertensivos/uso terapêutico , Humanos , Hemorragias Intracranianas/diagnóstico , Hemorragias Intracranianas/tratamento farmacológico , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Acidente Vascular Cerebral/diagnósticoAssuntos
Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Mancha Vinho do Porto , Terapia Combinada , Diagnóstico Diferencial , Embolização Terapêutica , Extremidades/irrigação sanguínea , Hemangioma Capilar/diagnóstico , Humanos , Recém-Nascido , Lasers de Corante , Desigualdade de Membros Inferiores/etiologia , Desigualdade de Membros Inferiores/cirurgia , Livedo Reticular , Terapia com Luz de Baixa Intensidade , Linfedema/etiologia , Mancha Vinho do Porto/complicações , Mancha Vinho do Porto/diagnóstico , Mancha Vinho do Porto/genética , Mancha Vinho do Porto/patologia , Mancha Vinho do Porto/psicologia , Mancha Vinho do Porto/terapia , Dermatopatias Vasculares , Neoplasias Cutâneas/diagnóstico , Síndrome de Sturge-Weber , Telangiectasia/congênitoRESUMO
Klippel - Trenaunay syndrome (KTS) is characterized by a cutaneous vascular nevus of the involved extremity, bone and soft tissue hypertrophy of the extremity and venous malformations. We present a case of KTS with splenic hemangiomas and rectal varices. A 29-year-old woman was referred for intermittent hematochezia for several years. She had history with a number of operations for cutaneous and soft tissue hamangiomas since the age of one year old and for increased circumference of her left thigh during the last few months. Abdominal CT revealed multiple hemangiomas in the spleen, fusiform aneurysmal dilatation of the deep veins and soft tissue hemangiomas. There was no evidence of hepatosplenomegaly or liver cirrhosis. Colonoscopy revealed hemangiomatous involvement in the rectum. There were rectal varices without evidence of active bleeding. Upon venography of the left leg, we also found infiltrative dilated superficial veins in the subcutaneous tissue and aneurysmal dilatation of the deep veins. The patient was finally diagnosed with KTS, and treated with oral iron supplementation only, which has been tolerable to date. Intervention or surgery is not required. When gastrointestinal varices or hemangiomatous mucosal changes are detected in a young patient without definite underlying cause, KTS should be considered.
Assuntos
Hemangioma/complicações , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Varizes , Adulto , Colonoscopia , Feminino , Humanos , Ferro da Dieta/uso terapêutico , Síndrome de Klippel-Trenaunay-Weber/complicações , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Reto/irrigação sanguínea , Baço/irrigação sanguínea , Tomografia Computadorizada por Raios XRESUMO
Klippel Trenauny Syndrome is a rare congenital syndrome characterized by port wine stain (capillary malformation), limb or hemihypertrophy and other vascular malformations. We present a case of this rare disorder in a young boy at an early stage of this disease.
Assuntos
Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Analgésicos/uso terapêutico , Pré-Escolar , Diagnóstico Diferencial , Humanos , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Masculino , Exame Físico/métodos , Prognóstico , Ultrassonografia DopplerRESUMO
A 36-year-old female patient was admitted to the emergency department of our hospital with symptoms and signs of pulmonary embolism. Further evaluation established the diagnosis and anticoagulant therapy was immediately started. Physical examination revealed left lower extremity edema, prominent varicose veins, greater length of the involved limb and a capillary malformation extending from the lower abdomen down to the left knee. The diagnosis of Klippel-Trenaunay syndrome (KTS) was suspected and a color duplex scan was next performed revealing derangements in the lower extremity venous system including deep venous thrombosis. KTS is a congenital anomaly characterized by capillary malformation, extensive varicosities and limb hypertrophy. Anomalies of the deep and perforator venous system coexist and predispose to thromboembolic events. Pulmonary embolism is infrequently encountered in the setting of this syndrome.
Assuntos
Síndrome de Klippel-Trenaunay-Weber/complicações , Embolia Pulmonar/etiologia , Adulto , Anticoagulantes/uso terapêutico , Feminino , Veia Femoral/diagnóstico por imagem , Veia Femoral/patologia , Humanos , Síndrome de Klippel-Trenaunay-Weber/diagnóstico por imagem , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Extremidade Inferior/irrigação sanguínea , Extremidade Inferior/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/tratamento farmacológico , Ultrassonografia Doppler Dupla , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/tratamento farmacológico , Trombose Venosa/etiologiaRESUMO
We present our experience of two cases with genitourinary manifestations of Klippel-Trenaunay syndrome. A MEDLINE search for the period 2001-2004 was done using the keywords "Klippel-Trenaunay syndrome", "vascular malformation" and "genitourinary". Only three cases with genitourinary manifestations of this syndrome were reported during this period. Genitourinary problems are rare in Klippel-Trenaunay syndrome; however, their management may be a challenge for the urologist. In one of our patients who failed to respond to endoscopic laser coagulation and partial cystectomy we used systemic alpha-interferon, with a good short-term response.
Assuntos
Inibidores da Angiogênese/uso terapêutico , Doenças Urogenitais Femininas/tratamento farmacológico , Interferon-alfa/uso terapêutico , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Doenças Urogenitais Masculinas , Pré-Escolar , Diagnóstico Diferencial , Feminino , Doenças Urogenitais Femininas/diagnóstico , Seguimentos , Humanos , Lactente , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Imageamento por Ressonância Magnética , Masculino , Tomografia Computadorizada por Raios XRESUMO
Venous angiomata, or venous malformations, are often present at birth, although they may not be evident until later. They consist of a spongy tangle of veins, and these lesions usually vary in size. Treatment of venous angiomata is often requested for cosmetic reasons, but painful ulcerations, nerve compression, functional disability can command care. This presentation describes management using sclerosant foam as the treating agent. During a 30-month period ending March 2004, 1,321 patients were investigated for venous disorders at the Vein Institute of La Jolla. Fourteen (incidence 1%) were found to have venous angiomata (: nine women). The age range was 15-76 years (mean 30.8 +/- 18.6). Lesions were classified by the Hamburg system and were primarily venous, extratruncular in 12 patients and combined extratruncular and truncular in two patients. Eight patients, three males, had manifestations of lower extremity Klippel-Trenaunay (syndrome; six had only venous angiomas. Only 10 of the 14 patients were treated. All patients were studied by Doppler duplex examination. Selected lesions were chosen for helical computed tomographic studies. Magnetic resonance venography was also used to image the lesions, define the deep circulation, note connections with normal circulation, identify vessels for therapeutic access, and determine infiltration of the lesion into adjacent soft tissue. Foam was produced by the Tessari two syringes one three-way stopcock teclinique, with the air to Polidocanol ratio being 4 or 5 to 1. This was used at 1% or 2% concentration, specific for each patient. The SonoSite 190 plus Duplex Doppler was used for ultrasound guidance, whenever deep access was required and to monitor progress and effects of treatment. A goal was set for each patient before treatment was begun. Ten patients were treated, and four await treatment. The mean number of treatments was 3.6 +/- 2.8 (range 1-10). A primary goal of pain-free healing was set in patients with nonhealing, painful ulceration or symptomatic varicose veins. This was achieved in all treated patients. Cosmetically, all of the patients were improved, and symptomatic patients were relieved of pain. The single complication was formation of a cutaneous ulcer following injection of telangiectasias. Sclerosant foam is a satisfactory tool to use in treating venous angiomata including the Klippel-Trenaunay syndrome. Use of foam sclerotherapy in this experience has proven the technique to be effective, essentially pain-free, and durable in the short term.
Assuntos
Angiomatose/tratamento farmacológico , Malformações Arteriovenosas/tratamento farmacológico , Polietilenoglicóis/uso terapêutico , Soluções Esclerosantes/uso terapêutico , Adolescente , Adulto , Idoso , Angiomatose/diagnóstico , Angiomatose/diagnóstico por imagem , Malformações Arteriovenosas/diagnóstico , Malformações Arteriovenosas/diagnóstico por imagem , Diagnóstico por Imagem , Feminino , Humanos , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Polidocanol , Ultrassonografia Doppler DuplaRESUMO
BACKGROUND: Klippel-Trenaunay syndrome is a rare congenital disease characterized by extensive cutaneous vascular malformations, venous varicosities, focal abnormalities of the deep venous system, and underlying soft tissue or bony hypertrophy. Given the rarity of the disease, there is little information available to counsel patients with Klippel-Trenaunay syndrome regarding obstetric outcome. CASES: We report our experience with 3 patients in whom Klippel-Trenaunay syndrome complicated 4 pregnancies. Successful delivery of a healthy infant at or beyond 36 weeks of gestation was achieved in all pregnancies. One of the 4 pregnancies was complicated by pulmonary embolism. CONCLUSION: Klippel-Trenaunay syndrome was once thought to be a contraindication to pregnancy. With careful management, successful pregnancies can be achieved.
Assuntos
Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Complicações Hematológicas na Gravidez/diagnóstico , Resultado da Gravidez , Adulto , Anticoagulantes/uso terapêutico , Feminino , Desenvolvimento Fetal/fisiologia , Seguimentos , Idade Gestacional , Humanos , Síndrome de Klippel-Trenaunay-Weber/tratamento farmacológico , Monitorização Fisiológica , Gravidez , Complicações Hematológicas na Gravidez/tratamento farmacológico , Diagnóstico Pré-Natal/métodos , Doenças Raras , Estudos de Amostragem , Índice de Gravidade de DoençaRESUMO
BACKGROUND: Treatment of congenital venous malformations poses a major clinical challenge. Surgery is difficult and frequently unsuccessful, radiological intervention with embolization has an ill-defined role, and conventional sclerotherapy has little to offer. OBJECTIVE: To evaluate the efficacy and safety of sclerosant in microfoam form in treating congenital venous malformations. DESIGN: A retrospective study of medical records, pretreatment and posttreatment color photographs, echo-Doppler ultrasonographic results, and/or magnetic resonance imaging in patients with congenital vascular malformations of venous predominance. Follow-up ranged from 6 to 103 months (mean, 30 months). SETTING: Private vascular surgery clinic in Granada, Spain, and dermatology department at a university hospital in Pamplona. Patients The study population comprised 50 patients, 19 with limited venous malformations, 16 with infiltrating venous malformations, and 15 with Klippel-Trénaunay syndrome.Intervention Percutaneous sclerotherapy by direct injection of 0.25% to 4% polidocanol microfoam under du-plex ultrasonographic guidance. The number of sessions varied between 1 and 46 (mean, 12 sessions). MAIN OUTCOME MEASURE: The primary efficacy end point was defined as a reduction in size after completion of the treatment. RESULTS: Sclerosis therapy with polidocanol microfoam was judged beneficial in 46 (92%) of the 50 patients. Among the 46 responders, 18 showed disappearance of treated malformations, 15 showed a reduction in malformation size of more than 50%, and 13 showed a reduction in malformation size of 50% or less. Of the 39 patients who presented with pain, the pain disappeared in 25 and was reduced in 14. No major adverse effects were reported by any patient. There were 4 cases of transient skin pigmentation and 3 cases of skin necrosis. CONCLUSIONS: Color echo-Doppler ultrasonography-guided sclerotherapy with polidocanol microfoam was effective in 46 (92%) of the patients. The technique is delivered on a strictly ambulatory basis and has proved simple and innocuous. The quality and durability of the outcome make this novel procedure the treatment of choice in the care of patients with congenital venous malformations.