Asociación de hemangioma en zona cérvico facial y hemangioma subglótico en un neonato / Association of cervical-facial and subglottic hemangioma in neonates / Associação de hemangioma cervicofacial e subglótico em neonatos

Se presenta un neonato con hemangioma cérvico facial y posterior diagnóstico de hemangioma subglótico. Los hemangiomas en el período neonatal y los primeros meses de vida requieren una atención cuidadosa. Debido a su patrón de crecimiento y la futura aparición de nuevas lesiones, son considerados imprevisibles en esta etapa. Se encontró una fuerte asociación entre los hemangiomas difusos de localización cérvico facial y los hemangiomas sintomáticos de la vía aérea alta. El riesgo está relacionado con el grado de extensión de la afectación cutánea en un área que incluye la piel de la región mandibular, el mentón, el labio inferior y la parte anterior del cuello. Los hemangiomas infantiles requieren tratamiento cuando presentan riesgo vital y alteraciones funcionales, como compromiso de la vía aérea.

This is the case of a newborn with cervical hemangioma and a subsequent diagnosis of subglottic hemangioma. Hemangiomas in neonates and infants require careful attention. Due to their growth pattern and the potential appearance of new lesions, they are considered unpredictable at this stage. A strong link was found between diffuse cervical-facial and symptomatic upper airway hemangiomas. The risk is related to the extent of skin involvement in a given area, which might include the jaw, chin, lower lip, and front of the neck skin. Infant hemangiomas require treatment when they present life-threatening and functional alterations, such as airway compromise.

Relatamos o caso de um recém-nascido com hemangioma cervical com diagnóstico posterior de hemangioma subglótico. Hemangiomas em recém-nascidos e lactentes requerem atenção cuidadosa. Devido ao seu padrão de crescimento e ao potencial aparecimento de novas lesões, são considerados imprevisíveis nessa fase. Uma forte associação foi encontrada entre hemangiomas cervicofaciais difusos e hemangiomas sintomáticos das vias aéreas superiores. O risco está relacionado à extensão do envolvimento da pele da mandíbula, o queixo, o lábio inferior e a pele da frente do pescoço. Os hemangiomas infantis necessitam de tratamento quando apresentam alterações funcionais ou risco de vida, como comprometimento das vias aéreas.

Infantile hemangiomas: risk factors for complications, recurrence and unaesthetic sequelae

Abstract Background: Infantile hemangiomas (IH) occur in approximately 4% to 10% of the pediatric population. The identification of clinical subtypes and conditions that indicate increased risk for complications is essential for therapeutic success. Objectives: To identify risk factors for complications, recurrence and unaesthetic sequelae. Methods: Retrospective cohort of patients with infantile hemangiomas undergoing follow-up at the Dermatology Service of Universidade Federal de Ciências da Saúde de Porto Alegre, between 2006 and 2018. Results: 190 patients were included; 24% had some type of complication, ulceration being the most frequent, and 86% required treatment. On correlation, ulceration was statistically related to mixed IH (p = 0.004), segmental IH (p < 0.01) and location in the gluteal region (p = 0.001). The mean time of treatment with propranolol was 12.7 months. Patients with PHACES syndrome and segmental infantile hemangioma required longer treatment (p < 0.001 and p = 0.0407, respectively), as well as those who started treatment after five months of life (p < 0.0001). Recurrence occurred in 16.6% of the treated patients, all-female; 94% were located on the head and neck (mainly on the upper eyelid, cyrano, S3 segment, and with parotid involvement); 61% and 38.8% were of the mixed and deep subtypes, respectively. Approximately 1/3 of the patients had some unaesthetic sequelae. Study limitations: As this is a retrospective study, data and photos of some patients were lost. Conclusions: Mixed and segmental hemangiomas are risk factors for ulceration and sequelae. Recurrence occurs more often in females and segmental hemangiomas. Segmental infantile hemangioma and PHACES syndrome require a longer time of treatment. Specific protocols are required for infantile hemangiomas with a high risk of recurrence.

Una década de atención al paciente con miocardiopatía hipertrófica / A decade of care to patients with hypertrophic cardiomyopathy

Introducción: La miocardiopatía hipertrófica es una enfermedad derivada de una alteración genética autosómica dominante que produce un aumento de la masa del ventrículo izquierdo, obstructiva o no. Es la principal causa de muerte súbita en adultos jóvenes. Objetivo: Mostrar la prevalencia de la miocardiopatía hipertrófica en la práctica del Cardiocentro Pediátrico William Soler y sus formas de presentación. Métodos: Estudio observacional longitudinal retrospectivo de serie de casos. Se resumieron variables demográficas y clínicas en los ingresos realizados durante 10 años de los pacientes con diagnóstico de miocardiopatía hipertrófica y se analizaron según correspondieran con pruebas para variables cualitativas y cuantitativas. Resultados: Se identificaron 21 pacientes; 12 con características obstructivas y 9 no obstructivas. No hubo predominio de sexo. La media de edad de diagnóstico y de debut clínico de los pacientes con enfermedad obstructiva fue significativamente menor que las edades de los pacientes sin obstrucción del tracto de salida izquierdo. El diagnóstico fue posible en más de 50 por ciento de los casos por sospecha por soplo o por pesquisa. El tratamiento quirúrgico permitió la reducción significativa del gradiente en el tracto de salida izquierdo. El propranolol fue el betabloqueador más usado acorde a consensos internacionales. No hubo fallecidos en la serie estudiada. Conclusiones: La miocardiopatía hipertrófica tiene una baja prevalencia en la práctica cardiopediátrica. Los síntomas tempranos se corresponden con la variedad obstructiva. Su diagnóstico temprano y el tratamiento específico, permite garantizar mejor calidad y expectativa de vida a los portadores de esta afección(AU)

Introduction: Hypertrophic cardiomyopathy is a disease derived from autosomal dominant genetic alteration that causes an increase in the mass of the left ventricle, and can be obstructive or not. It is the leading cause of sudden death in young adults. Objective: Show the prevalence of hypertrophic cardiomyopathy and its forms of presentation in the practice of the William Soler Pediatric Cardiocenter. Methods: Retrospective, observational, longitudinal study of a case series. Demographic and clinical variables were summarized in the admissions made during 10 years of patients diagnosed with hypertrophic cardiomyopathy whom were analyzed as appropriate with qualitative and quantitative variable testing. Results: 21 patients were identified; 12 with obstructive characteristics and 9 with non-obstructive ones. There was no predominance of sex. The average diagnostic age and clinical onset of patients with obstructive disease was significantly lower than the ages of patients without obstruction of the left outflow tract. Diagnosis was possible in more than 50 percent of cases by suspicion due to a murmur or by investigation. Surgical treatment allowed a significant reduction in the gradient of the left outflow tract. Propranolol was the most widely used beta-blocker according to international consensus. There were no deaths in the series studied. Conclusions: Hypertrophic cardiomyopathy has a low prevalence in cardiopediatric practice. Early symptoms correspond to the obstructive variety. Early diagnosis and specific treatment ensure better quality and life expectancy for carriers of this condition(AU)

Linfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico / Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente.Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5-4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalida

Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood.A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization.Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.

Hemangioma cavernoso / Cavernous Hemangioma

El hemangioma cavernoso es una neoplasia benigna de los vasos sanguíneos. Este trabajo tuvo como objetivo presentar el caso de un hemangioma cavernoso gigante que fue tratado con propranolol. El diagnóstico se realizó mediante examen físico, dúplex y otros complementarios de interés. Para la decisión de la conducta terapéutica, especialistas de diferentes especialidades evaluaron al paciente: Oncología, Dermatología, Ortopedia, Pediatría y Angiología. Una vez establecido el tratamiento, se realizó un seguimiento durante cuatro semanas y luego mensual. El resultado, después de 22 meses, fue la desaparición del hemangioma. El propranolol en el hemangioma cavernoso gigante debe ser el tratamiento de elección precoz para una evolución satisfactoria y evitar complicaciones(AU)

Cavernous hemangioma is a benign neoplasm of blood vessels. This work aimed at presenting the case of a giant cavernous hemangioma treated with propranolol. The diagnosis was made by physical examination, duplex Doppler sonography, and complementary tests of interest. For deciding the therapeutic approach, the patient was assessed by several specialists from different medical fields, such as oncology, dermatology, orthopedics, pediatrics, and angiology. Once the treatment was established, a follow-up was carried out for four weeks and then monthly. The outcome, after twenty-two months, was the hemangioma disappearance. Propranolol in giant cavernous hemangioma should be the treatment of early choice for a satisfactory evolution and to avoid complications(AU)

Tratamiento con propranolol en el síndrome de Kasabach-Merritt secundario a hemangioma hepático congénito. Caso clínico / Propranolol treatment in Kasabach-Merritt Syndrome secondary to congenital hepatic hemangioma. Clinical case

El hemangioma hepático es el tumor benigno de hígado más frecuente. Puede ser congénito o infantil, con diferentes evoluciones y complicaciones. La evolución clínica es muy variable, desde pacientes asintomáticos hasta cuadros de gravedad con insuficiencia cardíaca, síndrome de Kasabach-Merritt o síndrome compartimental. El diagnóstico se basa en la historia clínica y los estudios por imágenes, especialmente, la ecografía y el examen doppler en manos experimentadas. Resulta fundamental el diagnóstico diferencial con otras lesiones hepáticas, sobre todo, el hepatoblastoma. En los pacientes sintomáticos, el propranolol surge como primera línea terapéutica con buenos resultados y baja frecuencia de efectos adversos. Se presenta el caso de un recién nacido con hemangioma hepático asociado a síndrome de Kasabach-Merritt, con excelente respuesta y tolerancia al propranolol

Hepatic hemangioma is the most common benign liver tumor. It can be congenital or infantile with different outcomes and complications. The clinical manifestation varies from asymptomatic to severe conditions with heart failure, Kasabach-Merritt syndrome or compartment syndrome. Diagnosis depends on medical history and imaging studies, especially ultrasound and Doppler examination in experienced hands. Differential diagnosis is essential with other hepatic lesions, mainly hepatoblastoma. In symptomatic patients, propranolol emerges as the first line treatment with good results and low frequency of adverse effects. We present the case of a newborn with a hepatic hemangioma and Kasabach-Merritt syndrome with an excellent response and tolerance to propranolol.

Tirotoxicosis por enfermedad trofoblástica gestacional: revisión a partir de 3 casos / Thyrotoxicosis due to gestational trophoblastic disease: clinical cases

La enfermedad trofoblástica gestacional (ETG) es una complicación del embarazo poco común. Corresponde a un espectro de lesiones proliferativas del tejido trofoblástico: Mola Hidatiforme (MH) en sus formas parcial y completa, Coriocarcinoma, Tumor Trofoblástico y Tumor Trofoblástico Epiteloide. Los distintos tipos de ETG presentan en común la hipersecreción de gonadotrofina coriónica humana (hCG). La hCG es una hormona glicoproteica con una estructura muy similar a la TSH, por lo cual puede estimular la función tiroidea en condiciones fisiológicas y en algunas condiciones patológicas. La ETG puede cursar con hipertiroidismo, el cual puede variar en intensidad, desde una presentación asintomática con alteración leve de hormonas tiroideas a un cuadro de hipertiroidismo manifiesto. Se presentan 3 casos clínicos de pacientes con ETG, específicamente MH que evolucionaron con tirotoxicosis transitoria. Los casos presentaron un cuadro leve de hipertiroidismo con pocos síntomas asociados. La taquicardia fue el único síntoma en la mayoría de los casos. En todas las pacientes las hormonas tiroideas se normalizaron después del tratamiento de la ETG. Conclusión: Se debe tener presente la posibilidad de hipertiroidismo en toda paciente con ETG. Un alto nivel de sospecha permitirá identificar a aquellas pacientes que cursen con hipertiroidismo, permitiendo así un diagnóstico y tratamiento oportuno.

Gestational trophoblastic disease (GTD) is a rare complication of pregnancy. GTD includes a group of proliferative lesions of trophoblastic tissue: partial and complete hydatidiform mole, choriocarcinoma, epithelioid trophoblastic tumor, and placental site trophoblastic tumor. The different types of GTD have in common the hypersecretion of human chorionic gonadotropin (hCG). HCG is a glycoprotein hormone with a similar structure to TSH. In physiological and pathological conditions hCG can stimulate thyroid function. GTD can present with hyperthyroidism, which can vary in intensity, from an asymptomatic presentation with mild alteration of thyroid hormones to a manifest hyperthyroidism. We present 3 clinical cases of patients with GTD thyrotoxicosis. All cases presented mild hyperthyroidism. Tachycardia was the only symptom in most cases. In all patients thyroid hormones return to normal after treatment of GTD. Conclusion: In patients with GTD the possibility of hyperthyroidism should be kept in mind. A high level of suspicion will allow to identifying patients with hyperthyroidism.

Experiencia en el uso de propranolol para el tratamiento de la taquicardia supraventricular en pacientes menores de un año / Experience using propranolol for the management of supraventricular tachycardia in patients younger than 1 year

Objetivo. Evaluar los resultados y efectos adversos de la terapia con propranolol en menores de un año con taquicardia supraventricular. Población y métodos. Menores de 1 año con taquicardia supraventricular documentada, que recibieron tratamiento y prevención con propranolol por vía oral. Se analizaron sexo y edad, cardiopatía congénita asociada, pre excitación ventricular en el electrocardiograma basal, recurrencia intratratamiento y efectos adversos. Resultados. Se identificaron 107 pacientes. El primer episodio de taquicardia supraventricular ocurrió a una edad mediana de 190 días. En 10 pacientes, se observó cardiopatía congénita asociada. El 23,3 % presentó pre excitación ventricular en el electrocardiograma basal. El rango de la dosis de propranolol fue de 2 a 5 mg/kg/día. En el 30,8 %, se observó recurrencia intratratamiento. En 2 pacientes, se suspendió la medicación por efectos adversos graves. Conclusión. El propranolol evitó la recurrencia en el 70 % de los casos. En 2 pacientes, fue necesario suspenderlo por efectos adversos graves

Objective. To assess the results and adverse events of propranolol therapy in infants younger than 1 year with supraventricular tachycardia. Population and methods. Infants younger than 1 year with documented supraventricular tachycardia who received oral treatment and prophylaxis with propranolol. Sex and age, associated congenital heart disease, ventricular preexcitation in the base line electrocardiogram, on-treatment recurrence, and adverse events were analyzed. Results. A total of 107 patients were identified. The first supraventricular tachycardia event occurred at a median age of 190 days. Associated congenital heart disease was observed in 10 patients. Ventricular preexcitation in the baseline electrocardiogram was detected in 23.3 %. Propranolol dose ranged from 2 to 5 mg/kg/day. On-treatment recurrence was observed in 30.8 %. Medication was discontinued in 2 patients due to severe adverse events. Conclusion. Propranolol prevented recurrence in 70 % of cases. It was discontinued in 2 patients due to severe adverse events.

Retinopatía del prematuro: actualización en detección y tratamiento / Retinopathy of the premature: update in screening and treatment

Resumen: La Retinopatía del Prematuro (RDP) es una alteración proliferativa de los vasos sanguíneos de la retina inmadura, que afecta principalmente a los recién nacidos de muy bajo peso (RNMBP) y de menor edad gestacional. El objetivo de esta revisión es describir a qué niño se debe efectuar la detección de esta enfermedad y analizar los recientes avances en su tratamiento. La detección de RDP está dirigida principalmente a los RNMBP y a < de 32 semanas de edad gestacional, pero también se ha propuesto un criterio según edad postmenstrual. Además de la fotocoagulación con láser, tratamiento estándar en la actualidad, se han desarrollado nuevas terapias, como los agentes anti factor de crecimiento vas cular endotelial (VEGF), que se han utilizado exitosamente en la retinopatía umbral, especialmente localizada en zona I, con menos efectos adversos y mejores resultados oculares a futuro. que la fo tocoagulación con láser. En los últimos años, se han realizado ensayos clínicos con propranolol oral como tratamiento de la RDP, principalmente en la etapa pre-umbral (etapa 2 o 3 en zona II ó III). Este bloqueador beta-adrenérgico puede prevenir la progresión de la retinopatía en RNMBP de etapa pre- umbral a umbral y/o evitar la necesidad de terapias invasivas, como la fotocoagulación con láser o la administración intravítrea de agentes anti-VEGF. La fotocoagulación con láser continúa siendo el tra tamiento de elección en la RDP. Los agentes anti-VEGF y el propranolol oral, evitarían la progresión de esta patología de etapa pre-umbral a umbral, y podrían complementar el tratamiento de la RDP.

Abstract: Retinopathy of Prematurity (ROP) is a proliferative disorder of the blood vessels of the immature retina, which affects mainly very-low-birth-weight infants (VLBW). The objective of this review is to describe to which infant the screening examination of this disease should be performed and to analy ze the recent advances in the treatment of this disease, which have emerged in the last decade. The detection of this disease is mainly focused on VLBW infants and newborns < 32 weeks of gestational age. In addition to laser photocoagulation, standard treatment today, new therapies have appeared, such as the anti-VEGF agents, which have been successfully used in the threshold ROP, especially located in zone I. This therapy is less harmful than laser photocoagulation and with better ocular results in the future. In recent years, oral propranolol has been used as a treatment for ROP in clinical trials, mainly in the pre-threshold stage (stage 2 or 3 in zone II or III). This drug is a beta-adrenergic blocker that can prevent the progression of retinopathy in pre-threshold to threshold stage and/or avoid the need for invasive therapies, such as laser photocoagulation or intravitreal administration of anti-VEGF agents. Laser photocoagulation continues to be the standard treatment for ROP. New treatments have emerged for ROP, such as anti-VEGF agents and oral propranolol, which could pre vent the progression of this disease from the pre-threshold to the threshold stage.

Efectividad del propranolol en el tratamiento de malformaciones vasculares cutáneas / Effectiveness of propranolol in treatment of cutaneous vascular malformations

Se realizó un estudio cuasi-experimental de series temporales para evaluar la efectividad del propranolol en el tratamiento de malformaciones vasculares cutáneas en 48 pacientes que asistieron a la consulta del Servicio de Dermatología del Hospital Central Universitario Dr. Antonio María Pineda durante el período febrero-julio 2018. Los resultados muestran que existen diferencias estadísticamente significativas (p <0.05; p0,0001) antes y después del primer mes de tratamiento con propranolol, las cuales se mantiene hasta los seis meses, con respecto al tamaño, color, consistencia y temperatura. Se espera que los resultados sirvan para proponer el uso de propranolol como una opción terapéutica no invasiva en el tratamiento de las malformaciones vasculares cutáneas(AU)

A quasi-experimental study of time series was carried out to evaluate the effectiveness of propranolol in the treatment of cutaneous vascular malformations in 48 patients attending the Dermatology Service of the Hospital Central Universitario Dr. Antonio Maria Pineda during the period February - July 2018. The results show that there are statistically significant differences (p <0.05; p0,0001) before and after treatment with propranolol starting one month post-treatment which are kept until six months, related to size, color, consistency and temperature of lesions. We hope that these results will encourage the use of propranolol as a non-invasive therapeutic option in the treatment of cutaneous vascular malformations(AU)

Effectiveness of fixed doses of propranolol in the treatment of hemangiomas regardless of child's weight gain: a case report / Efetividade do tratamento de hemangiomas utilizando doses fixas de propranolol em comprimido independentemente do ganho de peso da criança: relato de caso

ABSTRACT Objective: To present the outcomes of fixed doses of propranolol tablets for the treatment of hemangiomas. Case description: Two illustrative cases of hemangioma in infant patients younger than six months old are described. Treatments were started in 2010 and 2011 and were monitored until August 2017. Patients were treated with fixed doses, initially calculated based on the upper limit of 3 mg/kg/day and administrated in two daily doses rounded down to the nearest multiple of five milligrams. Dosage was not adjusted to patients' weight gain. The tablets were crushed and then diluted in a maximum amount of 3 mL of water. This procedure was necessary because propranolol was not available in oral solution in 2009, when dosages available in the Brazilian market were 10, 40 and 80 mg. Both patients presented significative improvement in the first 60 days and were in complete remission by the end of the treatment. Comments: It is possible to treat patients with Propranolol 10 mg tablets, even though the dosage is not as precise as when calculated according to patients' weight. The maintenance of a fixed dose, ignoring the patient's progressive weight gains, helps avoiding the rebound effect and decreases complications.

RESUMO Objetivo: Apresentar a experiência com a utilização de propranolol em doses fixas, em forma de comprimido, para o tratamento de hemangiomas. Descrição do caso: Dois casos ilustrativos de portadores de hemangiomas com menos de seis meses de idade são descritos. O início de tratamento ocorreu nos anos de 2010 e 2011 com seguimento até agosto de 2017. Os pacientes foram tratados com doses fixas iniciais calculadas com limite máximo de 3 mg/kg/dia, divididas em duas doses diárias, sempre com quantidades múltiplas de 5 mg. Os comprimidos de 10 mg ou a sua metade eram macerados e diluídos em 3 mL de água. As doses não foram mais alteradas. Esse uso foi decorrente da ausência da forma líquida de propranolol em 2009, quando começamos a utilizar esse tratamento, sendo então apenas disponíveis comprimidos de 10, 40 e 80 mg. Os pacientes obtiveram melhora acentuada nos primeiros 60 dias e remissão completa posteriormente. Comentários: É possível o uso de comprimidos de 10 mg, apesar de resultar numa dose não exata, como a calculada por kg/peso. A manutenção da mesma dose, mesmo com aumento progressivo de peso, pode evitar o efeito rebote e diminuir o índice de complicações.

Tiempo de respuesta terapéutica al propranolol sistémico en el manejo de hemangiomas subglóticos pediátricos: serie de casos y revisión de la literatura / Therapeutic time of response of systemic propranolol in the management of pediatric subglottic hemangiomas: case series and review of the literature

Resumen: Introducción: Los hemangiomas de la vía aérea son neoplasias congénitas de baja incidencia. Su presentación clínica consiste en una obstrucción de la vía aérea superior con disnea progresiva y estridor, ocasionando una obstrucción grave, aguda y grave de la vía aérea. Los hemangiomas infantiles tienen una incidencia al nacimiento del 2%, incrementándose al 9% a los 12 meses de vida. Casi el 60% de los hemangiomas infantiles afectan la región de la cabeza y el cuello. Sin embargo, la localización en la vía aérea superior no es tan frecuente y solo se informa en el 1.8% de los pacientes con hemangiomas cutáneos. El tratamiento tradicional de los hemangiomas laríngeos ha sido la administración de corticoides sistémicos por periodos largos, la cirugía endolaríngea con láser de CO2, la cirugía abierta, la traqueostomía y, recientemente, el propranolol oral. Casos clínicos: Se presentan cinco casos de hemangiomas subglóticos tratados con propranolol sistémico y la evaluación del tiempo de respuesta terapéutica, definido como el control satisfactorio de los síntomas obstructivos y aseguramiento de la vía aérea. En estos casos, el propranolol mostró ser una opción efectiva y segura para el tratamiento de hemangiomas laríngeos, con un tiempo de respuesta terapéutica de 48 a 72 horas. Conclusiones: El tratamiento con propranolol oral se ha convertido en los últimos años en la primera opción terapéutica debido a su alta eficacia y su buen perfil de seguridad.

Abstract: Background: Airway hemangiomas are congenital neoplasms of low incidence. Its clinical presentation consists of obstruction of the upper airway with progressive dyspnea and stridor and the life of the patient at risk. Infantile hemangiomas have an incidence at birth of 2%, increasing to 9% at 12 months of life. Almost 60% of childhood hemangiomas affect the head and neck region. However, localization in the upper airway is not as frequent and it is only reported in 1.8% of patients with cutaneous hemangiomas. The traditional treatment of laryngeal hemangiomas has been the administration of systemic corticosteroids for long periods, endolaryngeal surgery with CO2 laser, open surgery, tracheostomy or, recently, oral propranolol. Case report: Five cases of subglottic hemangioma treated with systemic propranolol are presented. The therapeutic time of response with the satisfactory control of obstructive symptoms and assurance of the airway was evaluated. In these cases, propranolol was shown to be an effective and safe option for the treatment of laryngeal hemangiomas with a therapeutic response time of 48 to 72 hours. Conclusions: In recent years, the treatment with oral propranolol has become the first therapeutic option due to its high efficacy and safety profile.

Arritmias ventriculares sustentadas: a tempestade chegou / Sustained ventricular arrhythmias: the storm has arrived

Um dos grandes desafios no atendimento dos pacientes nas unidades de emergência é o tratamento das arritmias ventriculares, principalmente, quando sustentadas e recorrentes, pois são de difícil tratamento e estão associadas à alta mortalidade. O principal mecanismo envolvido na sustentação das taquicardias ventriculares é o mecanismo de reentrada, devido às cicatrizes miocárdicas secundárias a diversas cardiopatias estruturais. A tempestade elétrica pode ser séria quando ocorre em portadores de desfibriladores automáticos, provocando múltiplos choques correspondentes fora do ambiente hospitalar. Nesses casos é necessária a internação hospitalar, onde medidas específicas e escalonadas de tratamento são realizadas, indo desde o manejo clínico até intervenções específicas, como programação de dispositivos eletrônicos, intervenções eletrofisiológicas ou cirúrgicas

One of the biggest challenges in the care of patients in emergency units is the treatment of ventricular arrhythmias, particularly when sustained and relapsing, as they are difficult to treat and are associated with high mortality. The main mechanism involved in the maintenance of ventricular tachycardias is the mechanism of reentry, due to myocardial scars secondary to various structural heart diseases. The electrical storm may be serious when it occurs in patients with automatic defibrillators, causing multiple corresponding shocks outside the hospital setting. In these cases, admission to hospital is necessary, where specific and stepwise treatment measures are performed, ranging from clinical management to specific interventions, such as programming of electronic devices, and electrophysiological or surgical interventions

PHACE syndrome: clinical manifestations, diagnostic criteria, and management

Abstract: Infantile hemangioma can be linked to other organ malformations. In 1996, PHACE syndrome was first defined as the association of large and segmental infantile hemangioma, usually on the face, head, or cervical region, with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, coarctation of the aorta, cardiac defects, and ocular abnormalities. Over 300 cases of PHACE syndrome have been reported, and it is cconsidered one of the most common neurocutaneous vascular disorders in childhood. Knowledge of the features and locations of lesions that imply a greater risk of systemic involvement is crucial for the diagnosis and proper management of PHACE syndrome patients. This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients.

Algunas especificidades sobre los hemangiomas externos en niños / Some specificities on external hemangiomas in children

Los hemangiomas son tumores vasculares benignos con características biológicas muy específicas en la infancia; estos se pueden localizar en órganos, y reciben el nombre de internos, o en cabeza, cuello, tronco y extremidades, que son los denominados externos, los cuales crecen a una velocidad variable. Con este trabajo se buscó exponer algunas particulares clinicoepidemiológicas de los hemangiomas externos, su clasificación, diagnóstico y evolución. Asimismo, se refieren aspectos relacionados con la efectividad del propranolol como propuesta terapéutica de primera elección en niños y se presentan los resultados de algunos autores al respecto, que en su mayoría convergen en cuanto a la aplicación de este medicamento como de primera línea.

Hemangiomas are benign vascular tumors with very specific biological characteristics during childhood; these tumors can be located in organs, and receive the name of internal, or in head, neck, trunk and extremities that are denominated external, which grow to a variable speed. Some clinical epidemiological particularities of external hemangiomas were exposed with this work, such as their classification, diagnosis and clinical course. Also, there is a reference to some aspects related to the effectiveness of propranolol as therapeutic proposal of first election in children and the results of some authors in this respect are presented, most of which coincide as for its use as first line medication.

Hemangioma de crecimiento mínimo o detenido con lipoatrofia: presentación de un caso clínico / Hemangioma with minimal or arrested growth

Los hemangiomas de la infancia (HI) son tumo-res vasculares que presentan un patrón evolutivo característico. Existe un subgrupo de HI que pre-senta un tipo de crecimiento distinto denominados hemangiomas de la infancia con crecimiento míni-mo o detenido (HI-CMD). En estos, el componen-te proliferativo no supera el 25% de la superficie total; además, afecta a niños de al menos dos meses de edad en la consulta inicial o a niños menores de dicha edad en los que se realiza un seguimiento mayor a 2 meses. Los HI- CMD con lipoatrofia son una variante poco frecuente de HI de la cual exis-ten escasos reportes en la literatura. El objetivo de nuestro trabajo es presentar un caso de HI-CMD con lipoatrofia en una lactante. Los HI-CMD presentan un desafío diagnóstico ya que deben ser reconocidos precozmente y diferencia-dos de otras lesiones vasculares.

Infantile hemangiomas (IH) are vascular tumors that present a characteristic evolutive pattern. There is a subgroup of hemangiomas that pre-sents a different type of growth called infantile hemangiomas with minimal or arrested growth (IH-MAG). In these, the proliferative component does not exceed 25% of the total surface; it also affects children of at least two months of age in the initial consultation or children under that age in which a follow-up of more than 2 months is carried out. IH-MAG with lipoatrophy is a rare variant of IH of which there are few reports in the literature.The aim of our report is to present a case of IH-MAG with lipoatrophy in an infant. IH-MAGs present a diagnostic challenge since they must be recognized early, and differentiated from other vascular lesions.

Comparison of botulinum toxin and propranolol for essential and dystonic vocal tremors

OBJECTIVES: Vocal tremors, which cause social difficulties for patients, may be classified as resting or action tremors. Of the vocal action tremors, essential and dystonic tremors are the most common. Botulinum toxin and oral medications have been used to treat vocal tremors, but no comparative clinical trials have been performed. The aim of this study was to compare the effects of botulinum toxin injection and the oral administration of propranolol in the treatment of essential and dystonic vocal tremors. METHODS: This clinical trial recruited 15 patients, divided into essential and dystonic vocal tremor groups. Patients in both groups received successive treatment with botulinum toxin and propranolol. The treatments were administered at different times; the order of treatment was randomly selected. Patients were assessed with flexible nasofibrolaryngoscopy and with perceptual and acoustic voice evaluations. A statistical significance level of 0.05 (5%) was used. RESULTS: Botulinum toxin produced statistically significant improvements in perceptual measures of vocal instability in patients with dystonic vocal tremors compared with baseline values and treatment with propranolol. The acoustic measure of variability in the fundamental frequency was significantly lower in patients with dystonic vocal tremors after treatment with botulinum toxin. CONCLUSION: Essential and dystonic vocal tremors responded differently to treatment. Dystonic vocal tremors responded significantly to treatment with botulinum toxin but not oral propranolol. Essential vocal tremors did not respond significantly to either treatment, perhaps due to the small number of patients, which is a limitation of this research.