Síndrome de CLOVES: tratamiento con rapamicina oral: reporte de dos casos / CLOVES syndrome: treatment with oral rapamycin: report of two cases

INTRODUCCIÓN: El síndrome de CLOVES se caracteriza por sobrecrecimiento lipomatoso asociado a malformaciones vasculares, representando un desafío diagnóstico y terapéutico. La rapamicina, un inhibidor de la vía mTOR, ha demostrado ser una buena alternativa terapéutica en un grupo de anomalías vasculares. Reportamos dos casos de síndrome de CLOVES con buena respuesta al tratamiento con rapamicina oral. OBJETIVO: Reportar la experiencia del uso de rapamicina oral en el tratamiento de dos pacientes con síndrome de CLOVES. CASOS CLÍNICOS: Caso 1: preescolar femenino de tres años de edad con sín drome de CLOVES e historia de hospitalizaciones reiteradas por infección severa de malformaciones linfáticas macroquísticas y episodios trombóticos. Evoluciona con mala calidad de vida, múltiples hospitalizaciones, riesgo quirúrgico y progresión de las lesiones, por lo que se indicó rapamicina oral. A los 6 meses de tratamiento se evidenció reducción clínica y radiológica del tamaño de las masas lipomatosas y linfáticas, ausencia de linforrea cutánea y mejoría significativa de la calidad de vida, sin requerir nuevas hospitalizaciones. Caso 2: escolar femenino de diez años de edad, portadora de síndrome de CLOVES, que desarrolló escoliosis y deterioro de su capacidad motora, haciéndose dependiente del uso de silla de ruedas. Se indicó rapamicina oral, evidenciándose a los cuatro meses de tratamiento mejoría en su capacidad física, independencia y autovalencia, con desaparición de la linforrea. CONCLUSIÓN: Proponemos la rapamicina oral para el tratamiento de pacientes con sín drome de CLOVES que presenten complicaciones y deterioro de la calidad de vida producto de su enfermedad.

INTRODUCTION: CLOVES syndrome is characterized by lipomatous overgrowth associated with vascular malforma tions, representing a diagnostic and a therapeutic challenge. Rapamycin, an mTOR inhibitor, has proved to be a good therapeutic option in some vascular anomalies. In this article, we report two ca ses of CLOVES syndrome with good response to oral rapamycin treatment. OBJECTIVE: To report the outcome of two patients with CLOVES syndrome treated with oral rapamycin. CLINICAL CASES: Case 1: A three-year-old female preschooler with CLOVES syndrome and history of repeated hospita lizations due to severe infections resulting from macrocystic lymphatic malformations and due to thrombotic episodes. The patient evolved with poor quality of life, multiple hospitalizations, surgical risk and progression of the lesions, therefore, oral rapamycin was indicated. After six months of treatment, clinical and radiological reduction in the size of the lipomatous and lymphatic masses, cutaneous lymphorrhea absence and a significant improvement of her quality of life were observed, without requiring new hospitalizations. Case 2: a ten-year-old female schooler with CLOVES syndro me, who developed scoliosis and deterioration of her motor skills, becoming wheelchair-dependent. Oral rapamycin was indicated, showing improvement in her physical capacity, independence and au tonomy, and absence of lymphorrhea after four months of treatment. CONCLUSION: We propose oral rapamycin for the treatment of patients with CLOVES syndrome who present with complications and deterioration in the quality of life as a result of the disease.

Tratamento de hemangioma labial por meio da escleroterapia / Labial hemangioma treatment through sclerotherapy: case report

O hemangioma é considerado como uma neoplasia vascular benigna, caracterizado por uma fase de crescimento rápido, com proliferação de células endoteliais, seguida por uma estabilização gradual. A principal queixa dos pacientes portadores dessa neoplasia é referente à estética e, por esse motivo, a escleroterapia vem sendo uma boa opção de tratamento para obter resultados estéticos satisfatórios. Para esse tipo de tratamento, são utilizadas aplicações com oleato de monoetanolamina a 0,05 g/ml (Ethamolin), que promovem a regressão da lesão por fibrose dos espaços endoteliais. No presente artigo, é descrito um relato de caso clínico de uma paciente do sexo feminino, portadora de um hemangioma no lábio superior esquerdo. Foram realizadas três aplicações do agente esclerosante Ethamolin no interior do tumor, até que ele regredisse o suficiente para ser removido cirurgicamente com maior segurança e eficiência. As aplicações com oleato de monoetanolamina proporcionaram a involução do hemangioma de forma rápida e segura, de modo a promover uma esclerose dos vasos tumorais, favorecendo a remoção cirúrgica.

The hemangioma is considered as a benign vascular neoplasm, characterized by a phase of rapid growth, with proliferation of endothelial cells, followed by gradual estabilization. The main complaint of patients with this neoplasm is related to aesthetics and, for this reason, sclerotherapy has been a good treatment option to obtain satisfactory aesthetic results. For this type of treatment, applications with 0.05 g/ml Monoethanolamine Oleate (Ethamolin), which causes regression of the lesion by fibrosis of the endothelial spaces, are used. In the present article a clinical case report of a female patient with a hemangioma in the upper left lip will be described. As a treatment, three applications of the Ethamolin sclerosing agent were performed inside the tumor until it regress to be excised with greater safety and efficiency. The applications with Monoethanolamine Oleate provided a quick hemangioma involution, in order to promote sclerosis of tumor vessels, favoring it surgical removal.