Anomalías Vasculares de la Cavidad Oral: Revisión de la Clasificación y Tratamiento Aplicado a dos Casos Clínicos / Vascular Anomalies of the Oral Cavity: Review of Classification and Treatment Applied to Two Clinical Cases

RESUMEN: Las anomalías vasculares de cabeza y cuello son un grupo de lesiones que afectan vasos sanguíneos y linfáticos donde el tratamiento sigue siendo un desafío. La clasificación actualizada de anomalías vasculares de cabeza y cuello es la clasificación de Mulliken modificada, que las subdivide en a) tumores vasculares y, b) malformaciones vasculares. En este reporte, presentamos dos casos clínicos de pacientes de sexo masculino, con diagnóstico de anomalías vasculares que afectan al labio y paladar duro, diagnosticados como malformación arteriovenosa y malformación venosa, respectivamente. Dichas lesiones remitieron completamente mediante tratamientos conservadores (agentes esclerosantes) y/o quirúrgicos (exéresis quirúrgica completa de la lesión) logrando una remisión completa. Consecutivamente, presentamos una revisión de la literatura enfocado a la clasificación actual, enfoques terapéuticos actuales y futuros.

ABSTRACT: Vascular anomalies of the head and neck are a group of lesions that affect blood and lymph vessels where treatment remains a challenge. The updated classification of head and neck vascular anomalies is the modified Mulliken classification, which subdivides them into a) vascular tumors and b) vascular malformations. In this report, we present two clinical cases of male patients, with diagnosis of vascular anomalies affecting the lip and hard palate, diagnosed as arteriovenous malformation and venous malformation, respectively. These lesions were completely treated with conservative (sclerosing agents) and/or surgical (complete surgical exeresis of the lesion) treatments, achieving a complete remission. Consequently, we present a review of the literature focused on the current classification, current and future therapeutic approaches.

Rare association between atrioventricular septal defect and partial anomalous pulmonary venous connection

Abstract Clinical data: Infant, 7 months, female, referred to our department at one month of age, suspecting of congenital heart disease for further investigation. Chest radiography: Demonstrates cardiomegaly and prominent pulmonary vascular markings. Electrocardiography: Shows right ventricular hypertrophy and left anterior fascicular block. Echocardiography: Evidenced common atrioventricular valve with two orifices and the left superior pulmonary vein draining on the brachiocephalic vein. Computed tomography angiography: This complementary imaging exam was performed to confirm the diagnosis. Diagnosis: The patient presented an association between AVSD and PAPVC, a rare combination. The clinical picture of heart failure was preponderant, characterized by need for diuretics and complementary exams findings, and early surgical treatment was indicated. Operation: The operation was performed through a median sternotomy with 123 minutes of cardiopulmonary bypass and 89 minutes of cross-clamping time. The patient had no postoperative complications, remaining 10 days hospitalized.

Sling de la arteria pulmonar en paciente preescolar con compromiso de la vía aérea / Pulmonary artery sling in preschool patient with airway compromise

Resumen Introducción: El sling de la arteria pulmonar izquierda es una rara malformación congénita del arco aórtico en la que la rama pulmonar izquierda se origina a partir de la arteria pulmonar derecha. Generalmente se sitúa entre la tráquea y el esófago, que frecuentemente la comprimen. Dependiendo del grado de compromiso de la vía aérea, el esófago y las anomalías cardíacas asociadas, las manifestaciones clínicas varían. El tratamiento de esta malformación es quirúrgico; con el paso de los años, este abordaje ha mejorado, y se ha logrado una disminución de la morbilidad y mortalidad en esta condición. Caso clínico: Se presenta el caso de una paciente preescolar con hallazgo de sling de la arteria pulmonar izquierda con compromiso de la vía aérea. Conclusiones: Después de la corrección quirúrgica, se logró una respuesta clínica favorable y una mejoría significativa en la vía aérea, evidenciada en los hallazgos en la fibrobroncoscopia, sin necesidad de intervenciones adicionales.

Abstract Background: Left pulmonary artery sling is a rare congenital malformation of the aortic arch in which this artery originates from the right pulmonary artery and courses over the trachea and the esophagus. Clinical manifestations vary depending on the degree of airway compromise, the esophagus and associated cardiac anomalies. The treatment for this malformation is surgical; over the years, this approach has improved, achieving a decrease in morbidity and mortality in this condition. Case report: A preschool patient with incidental finding of sling of the left pulmonary artery with compromise of the airway is presented. Conclusions: After the surgical procedure, a favorable clinical outcome was observed and a significant improvement in the airway was shown by a fibrobronchoscopy, without any additional intervention.

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.

Manejo quirúrgico de pacientes adultos con anomalías vasculares: serie de casos / Surgical management of adult patients with vascular anomalies: series of cases

Introducción: Las anomalías vasculares son un grupo heterogéneo de patologías, que considera tanto tumores como malformaciones vasculares. Son, habitualmente, de diagnostico y manejo durante la infancia, algunas lesiones persisten hasta la edad adulta. El tratamiento habitual considera terapias poco invasivas, siendo la alternativa resecctiva un opcion infrecuente. Objetivos: El objetivo de este estudio es presentar el manejo quirúrgico de pacientes portadores de anomalías vasculares realizado por el equipo de Cirugía Plástica y Reconstructiva del Hospital Clínico de la Universidad de Chile. Material y Métodos: Serie de casos retrospectiva de pacientes con diagnóstico de anomalias vasculares, sometidos a manejo quirúrgico por el equipo de Cirugía Plástica y Reconstructiva del Hospital Clínico de la Universidad de Chile. Se incluyó todos los pacientes con diagnóstico de anomalías vasculares intervenidos entre los años 2010 ­ 2016. Se realizó una revisión del historial clínico de cada paciente elaborando un análisis descriptivo. Resultados: Serie de 9 pacientes, 6 (66,6%) de sexo femenino, con edad promedio de 34,88 ±15,70 años. De los cuales 6 (66,67%) presentaron malformaciones vasculares y 2 (22,22%) tumores vasculares. Se observo compromiso de cabeza en 6 (66,67%) casos y 2 (22,22,%) genital. Se utilizo colgajos locales de avance en 6 (66,67%) y colgajos de transposición en 2 (22,22%) casos. Conclusiones: El manejo quirúrgico es una alternativa terapéutica segura que ofrece resultados buenos a las lesiones complejas. Y por tanto, creemos que es necesario un trabajo multidisciplinario entre pediatras, dermatólogos, radiólogos fisiatras, psicólogos y cirujanos plásticos para poder ofrecer mejores resultados a nuestros pacientes.

Introduction: Vascular anomalies are a heterogeneous group of pathologies, which considers tumors and vascular malformations. Usually diagnosed and treated during childhood, some lesions persist into adulthood. The usual treatment considers non-invasive therapies, being the respective alternative an uncommon option. Objectives: Present the surgical management of adults patients with vascular anomalies performed by the Plastic and Reconstructive Surgery team of the Clinical Hospital of the University of Chile. Material and methods: Retrospective case series of patients with diagnosis of vascular anomalies submitted to surgical management by the Plastic and Reconstructive Surgery team of the Clinical Hospital of the University of Chile. All patients with diagnosis of vascular anomalies operated between the years 2010 - 2016 were included. A review of the clinical history of each patient was carried out and a descriptive analysis was done. Results: Series of 9 patients, 6 (66.6%) female, with a mean age of 34.88 ± 15.70 years. Of these, 6 (66.67%) had vascular malformations and 2 (22.22%) had vascular tumors. Head involvement was observed in 6 (66.67%) cases and 2 (22.22%) genital. Local flaps were used in 6 (66.67%) and transposition flaps in 2 (22.22%) cases. Conclusions: Surgical management is a safe therapeutic alternative that offers beneficial results to complex lesions. Therefore, we believe a multidisciplinary work between pediatricians, dermatologists, radiologists.

Malformación vascular de bajo flujo sinovial de la rodilla (hemangiolinfangioma): Reporte de caso / Low-low synovial vascular malformation of the knee (hemangiolymphangioma): Case report

Introducción: La malformación vascular venosa de bajo lujo se desarrolla generalmente durante la primera infancia y puede ser una causa poco frecuente de derrame recurrente de larodilla. La clínica, las pruebas de laboratorio y las radiografías suelen ser inespecíficas. Objetivo: Describir una patología infrecuente en pediatría, para enfatizar en la correcta clasificación y sospecha. Caso Clínico: Paciente de 2 años con historia de episodios de derrame articular de la rodilla derecha que requirió múltiples hospitalizaciones y tratamientos antibióticos. Exámenes de laboratorio normales. La radiografía simple de la rodilla no mostró cambios óseos. La resonancia magnética reportó malformación vascular de bajo lujo. Se realizó resección quirúrgica que evidenció lesión vascular entre las fibras musculares del vasto lateral del cuádriceps con extensión hasta la cápsula de la rodilla y disección de las fibras hasta el vasto lateral de la pierna derecha. El estudio histológico fue compatible con malformación vascular de bajo lujo. Ante el hallazgo benigno y la evolución favorable, continuó con tratamiento ambulatorio. Conclusiones: Aunque la malformación vascular de bajo lujo sinovial es una patología poco frecuente en la población pediátrica, debería considerarse la realización de un diagnóstico diferencial en pacientes con aparición repetida de hemartrosis sin historia de coagulopatía ni hemofilia.

Introduction: Low-low vascular malformation, which usually develops during the first stage of infancy, is a rare cause of recurrent effusion of the knee. History, laboratory and X-rays are usually non-specific. Objective: To describe a rare disease in pediatrics, emphasizing the correct classification and suspicion. Case Report: A case is presented of a two-year-old patient with a history of effusion of the right knee who required multiple hospitalizations and antibiotic treatments. Laboratory work-up was normal. Plain X-rays of the knee revealed no bone changes. MR imaging reported low-low vascular malformation. Surgical resection was performed, evidencing vascular lesion among the muscle fibers of the vastus lateralis of quadriceps until the capsule of the knee, as well as dissection of the fibers until the vastus lateralis of the right leg. Histology was consistent with low-low vascular malformation. Due to the benign outcome and favorable evolution, an outpatient management was possible. Conclusions: Although low-low synovial vascular malformation is a rare disease among the pediatric population, it should be considered in the differential diagnosis of patients with repeated hemarthrosis and no history of either coagulopathy or hemophilia.

Malformações capilares: resultados preliminares do tratamento associando laser ND:Yag 1064 nm e luz intensa pulsada / Capillary malformations: preliminary results of treatment associating ND:Yag 1064 nm laser and intense pulsed light

Introdução: As malformações vasculares congênitas do tipo capilar têm seus melhores resultados até o momento com o laser de corante. Nós associamos, em um único equipamento, o laser ND:Yag 1064 nm de pulso longo e a luz intensa pulsada, para o tratamento de lesões superficiais e profundas. Método: Dezessete pacientes foram tratados com a associação descrita em regime ambulatorial, com anestesia tópica, apenas em intervalos mensais, pelo mesmo cirurgião. O equipamento utilizado foi a plataforma Synchro (DEKA), com a ponteira de 4 mm de diâmetro e fluência de 110 J/cm2 para o laser ND:Yag 1064nm. Para a luz intensa pulsada foi utilizado um filtro de corte de 550 nm, a ponteira de 46 x 10 mm e 21 J/cm2 de fluência, divididos em dois disparos. Os resultados foram avaliados clinicamente e por meio de fotografias, em conjunto com os pacientes. Resultados: Dentre os dezessete pacientes tratados, quatro resultados foram considerados excelentes, nove, bons, quatro, regulares e nenhum insuficiente. Conclusão: O tratamento pode ser realizado adequadamente com anestesia tópica em regime ambulatorial. A associação das duas tecnologias possibilitou a obtenção de resultados satisfatórios e, provavelmente, é mais uma técnica no tratamento destas lesões.

Introduction: Congenital vascular malformations up to know, have their best results with dye laser. We associate, in single equipment, long pulse Nd:Yag 1064 nm laser and intense pulsed light, for simultaneous treatment of the superficial and deep lesions. Methods: Seventeen patients were treated with the association described in an office basis with topical anesthesia only at monthly intervals by the same surgeon. The equipment utilized was the Synchro Platform (DEKA) with a 4 mm diameter spot size and fluency of 110 J/cm2 for the ND:Yag 1064 nm laser. For the intense pulsed light was utilized a 550 nm cutoff filter with a 46 x 10 mm spot size and 21 J/cm2 as fluency, divided in two shots. Results were evaluated through clinical and photographical analysis together with the patient. Results: Among the seventeen patients treated, four results were considered excellent, nine, good,four, regular and none insufficient. Conclusion: The treatment can adequately be done in an office basis with topical anesthesia. Association of the two different technologies got a satisfactory result and it is probably one more technique for treating such lesions.

Tratamento de hemangioma ulcerado: relato de caso / Treating an ulcerated hemangioma: a case report

O hemangioma é o tumor vascular mais presente em crianças até 1 ano de idade. Geralmente, sua involução é espontânea, sendo por isso recomendada uma conduta expectante. Ulceração e infecção secundária, seguidas de hemorragia, são as complicações mais frequentes dos hemangiomas. Estima-se que apenas 10 a 20 por cento dos hemangiomas precisem ser tratados. A cirurgia é indicada nos casos de emergência, naqueles em que não há resposta aos tratamentos sistêmicos ou por razões estéticas. O objetivo deste estudo é relatar um caso de hemangioma combinado ulcerado do pavilhão auricular, em paciente de 8 meses de idade submetida a exérese total.

Hemangioma is the most common vascular tumor in children younger than 12 months. Because its regression is usually spontaneous, an expectant management is recommended in most cases. Ulceration and secondary infection followed by bleeding are the most frequent complications of hemangiomas. It is estimated that only 10 to 20 percent of hemangiomas require treatment. Surgery is usually indicated in emergency cases, when there is no response to systemic treatments, or for cosmetic reasons. The objective of this study was to report a case of ulcerated compound hemangioma on the external ear of an 8-month-old patient who underwent a complete surgical excision.