Atenolol oral comparado con propranolol oral para hemangioma infantil / Oral atenolol compared to oral propranolol for infantile hemangioma

INTRODUCCIÓN: El hemangioma infantil corresponde al tumor vascular benigno más frecuente de la infancia, con una incidencia de 3 a 10%. Entre los pacientes que requieren tratamiento el uso oral de propranolol, un betabloqueador no selectivo de tipo lipofílico, es usualmente considerado como la terapia de elección. Sin embargo, su uso se ha asociado a diversos efectos adversos, relacionados con su acción ß-2, y a su capacidad de cruzar la barrera hematoencefálica. Debido a esto, el uso oral de atenolol, un betabloqueador selectivo de receptores ß-1, de tipo hidrofílico, podría representar una alternativa válida de tratamiento. Sin embargo, aún existe controversia en relación con la eficacia y seguridad del tratamiento con atenolol como monoterapia, en comparación con el uso de propranolol como monoterapia para esta condición. MÉTODOS: Se realizó una búsqueda en Epistemonikos, la mayor base de datos de revisiones sistemáticas en salud, la cual es mantenida mediante el tamizaje de múltiples fuentes de información, incluyendo MEDLINE/PubMed, EMBASE, Cochrane, entre otras. Se extrajeron los datos desde las revisiones identificadas, se analizaron los datos de los estudios primarios, se realizó un metanálisis y se preparó una tabla de resumen de los resultados utilizando el método , GRADE. RESULTADOS: Se identificaron nueve revisiones sistemáticas, que en conjunto incluyeron 10 estudios primarios y tres ensayos aleatorizados. Se incluyeron los tres ensayos aleatorizados en el análisis del presente trabajo. CONCLUSIONES: El uso de atenolol oral como monoterapia, comparado con el uso de propranolol oral como monoterapia, podría resultar en poca o nula diferencia en cuanto a la probabilidad de remisión completa, la disminución del , la probabilidad de recaída posterior al tratamiento y el riesgo de presentar efectos adversos y efectos adversos severos, en el hemangioma infantil (certeza de la evidencia baja).

INTRODUCTION: Infantile hemangioma is the most frequent benign vascular tumor in childhood, with an incidence of 3 to 10%. When patients require treatment, oral propranolol, a non-selective lipophilic beta-blocker, is usually considered the therapy of choice. However, its use has been associated with several adverse events related to its ß-2 action and its ability to cross the blood-brain barrier. Because of this, oral atenolol, a hydrophilic ß-1 receptor-selective beta-blocker, may represent a valid treatment alternative. Nonetheless, there is still controversy regarding the efficacy and safety of atenolol when compared with propranolol as monotherapy for this condition. METHODS: We searched Epistemonikos, the largest database of systematic reviews in health science, which is maintained by screening multiple sources of information, including MEDLINE/PubMed, EMBASE, and Cochrane, among others. Data were extracted from the identified reviews, data from the primary studies were analyzed, a meta-analysis was performed, and a summary table of the results was prepared using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) method. RESULTS: Nine systematic reviews were identified, including 10 primary studies and three randomized trials. The three randomized trials were included in the analysis of this investigation. CONCLUSION: The use of oral atenolol compared with oral propranolol as monotherapies may result in little or no difference in terms of likelihood of complete remission, decrease in Hemangioma Activity Score, likelihood of post-treatment relapse, and risk of adverse events and severe adverse events, in infantile hemangioma (low certainty of evidence).

Hemangioma segmentario de la barba / Segmental Beard Hemangioma

Los hemangiomas infantiles son el resultado de la proliferación de células del endotelio vascular y representan los tumores benignos más frecuentes en la infancia, con una incidencia estimada del 4-10% en bebés caucásicos. Se clasifican según el número, la profundidad y la distribución. Dentro de esta última clasificación se encuentran aquellos denominados segmentarios, que se caracterizan por su distribución extensa en áreas de prolongaciones mesodérmicas embrionarias. Se comunica el caso de una paciente evaluada al mes y medio de vida, con un hemangioma extenso del área mandibular y cuello anterior (hemangioma segmentario de la barba). Se describe la importancia de los estudios complementarios para evaluar el compromiso de órganos subyacentes, para detectar síndromes asociados y definir el tratamiento sobre la base de estos resultados. (AU)

Infantile hemangiomas arise from the proliferation of vascular endothelial cells and represent the most common benign tumors in infancy, with an estimated incidence of 4-10% in Caucasian infants. They vary according to their number, depth, and distribution. Within the latter classification are the so-called segmental ones, which feature an extensive distribution in areas of embryonic mesodermal extensions. We report the case of a patient evaluated at one and a half months of life with an extensive hemangioma of the mandibular area and anterior neck (segmental hemangioma of the beard). We describe the importance of complementary studies for evaluating the involvement of underlying organs, detecting associated syndromes, and defining the treatment based on these findings. (AU)

Manejo de hemangioma ulcerado segmentario: combinación de tratamiento sistémico y tópico en dos casos pediátricos / Management of ulcers on a segmental hemangioma: A combination of systemic and topical therapies in two pediatric cases

Los hemangiomas infantiles (HI) son los tumores benignos más frecuentes de la infancia; la variante segmentaria es rara y se asocia con un mal pronóstico. Una de sus principales complicaciones es la ulceración durante la fase de crecimiento del tumor, a pesar de no presentar características macroscópicas compatibles con una lesión agresiva. El manejo en estos casos es dificultoso e impone la necesidad de asociar múltiples estrategias, algunas orientadas específicamente a impedir la proliferación del hemangioma y otras orientadas a la curación de la herida, el manejo del dolor y la prevención de la infección agregada. Presentamos dos casos a fin de comunicar nuestra experiencia respecto del manejo de dicha patología y su evolución final.

Infantile hemangiomas (IHs) are the most common benign tumors of childhood, and segmental ones are rare and associated with a poor prognosis. While these tumors look harmless, one of their main related complications is ulceration during tumor growth. The management in these cases is extremely challenging, requiring a combination of multiple approaches, some specifically aimed at preventing the proliferation of the hemangioma and others aimed at wound care, pain management, and prevention of further infection. Here we discuss two cases to narrate our experience on the management of this condition and its outcome.

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.

Success in the use of oral propranolol in the treatment of infantile hemangioma in nasal tip - Report of two cases

Abstract Infantile hemangioma is the most common pediatric vascular tumor, with the following risk factors: low birth weight, prematurity, white skin, female gender, multiparity and advanced maternal age. The use of oral and topical beta-blockers, although recent, has emerged as the first line of treatment, with superior safety and efficacy to previously used therapies, such as corticosteroids and surgeries. This report describes two cases of nasal tip infantile hemangioma, treated with oral propranolol. Both presented excellent therapeutic responses.

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.

Tratamiento de hemangioma lingual con polidocanol al 0.5 %. Relato de caso clínico. / Treatment of lingual hemangioma with 0.5% polidocanol. Clinical case report

El hemangioma, según la Organización Mundial de la Salud (OMS), es una neoplasia vascular benigna, caracterizada por la proliferación anormal de vasos sanguíneos, se presenta como una lesión única, pudiéndose observar en cualquier parte del organismo, sin embargo es más incidente en el área de cabeza y cuello, con predominio en los labios, en la lengua y en la mucosa yugal. Técnicamente, su exrpación quirúrgica es dicil y desafiante, al estar cargada con complicaciones como hemorragia, escisión incompleta, desfiguración facial y recurrencia. Objevo: relatar el caso clínico de un paciente con hemangioma lingual tratado con Polidocanol al 0,5%. Se necesitaron de 4 sesiones de aplicación del agente esclerosaste para que retrocediera completamente la lesión donde en cada infiltración se ulizó 1ml y en la úlma sesión 0.7 ml, logrando en esta úlma obtener la desaparición de la lesión por lo que el pronósco fue bueno. Conclusión: De acuerdo a los resultados de este trabajo el uso del Polidocanol al 0.5% en tratamientos de hemangioma linguales resulta ser una técnica bastante efecva, ya que con su prácca nos da una disminución del volumen de dicha lesión

The hemangioma, according to theWorldHealthOrganizaon (WHO), is a benign vascular neoplasm, characterized by the abnormal proliferaon of blood vessels, it presents as a single lesion, it can be found anywhere in the body, however it is more incident in thearea of the head and neck, predominantlyonthelips, onthetongue and onthejugal mucosa. Technically, its surgical removal is difficult and challenging, incomplete disease, facial disfigurement and recurrence. Objecve: to report the clinical case of a paentwith lingual hemangioma treatedwith 0.5% Polidocanol. iook 4 sessions of applicaon of thesclerosingagent to completelyretrachelesionwhere 1 ml wasused in eachinfiltraon and 0.7 ml in thelastsession, obtainingthedisappear of thelesion in thelaer, so the prognosis wasgood. Conclusion: According to the results of this study, the use of Polidocanol at 0.5% in lingual hemangioma treatmentsis a very effecve technique, and with its pracce we have a decrease in the volume of this lesion

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.

Effects of oral propranolol for circumscribed choroidal hemangioma / Efeitos do propranolol oral para de hemangioma circunscrito de coroide

ABSTRACT Purpose: We aimed to evaluate the effects of oral propranolol for circumscribed choroidal hemangioma. Methods: In this prospective, longitudinal interventional study, we administered oral propranolol at a dosage of 1.5 mg/kg/day to five patients with circumscribed choroidal hemangioma. We then evaluated visual acuity, binocular indirect ophthalmoscopy, optical coherence tomography, optical coherence tomography angiography, fluorescein and indocyanine green angiography, and ocular ultrasonography at regular intervals and compared changes from the baseline assessments. Results: No clinical or diagnostic changes were observed in the sizes of the circumscribed choroidal hemangiomas during treatment. Complications due to the hemangioma were reduced in the first four months of treatment, followed by maintenance, before worsening in the subsequent three months. Conclusions: The study showed that oral propranolol at a dose of 1.5 mg/kg/day did not offer effective monotherapy in the treatment of circumscribed choroidal hemangioma.

RESUMO Objetivo: Avaliar o efeito do propranolol oral para hemangioma circunscrito da coroide. Métodos: O estudo é do tipo prospectivo, quantitativo e descritivo. Propranolol oral na dose de 1.5 mg/kg/dia foi administrada em cinco pacientes com hemangioma circunscrito da coroide. Todos os pacientes foram avaliados com acuidade visual, oftalmoscopia binocular indireta, tomografia de coerência óptica, angiografia com tomografia de coerência óptica, angiografia com fluoresceína e indocianina verde e ultrassonografia ocular. Resultados: Nenhuma mudança clínica ou no tamanho do hemangioma circunscrito da coroide foi vista através de métodos diagnósticos em qualquer momento do tratamento. Uma atenuação das complicações foi observada nos primeiros quatro meses de tratamento, com manutenção da condição e piora nos meses seguintes. Conclusão: O estudo mostrou que o propranolol oral na dose de 1.5 mg/kg/dia não se mostrou efetivo como monoterapia no tratamento do hemangioma circunscrito da coroide.

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 mamario en una lactante con aparente mastitis: Caso clínico / Mammary hemangioma in an infant with apparent mastitis: Case report

La patología mamaria en pediatría es infrecuente. El hallazgo de una masa a nivel mamario en un lactante es una situación poco común. Los posibles diagnósticos a esta edad son absceso mamario, mastitis, ingurgitación mamaria por estimulación hormonal materna y hemangioma. Es importante llegar al diagnóstico adecuado para emplear un tratamiento acorde y evitar la aparición de complicaciones de dichas patologías. Se presenta un caso de una lactante con una masa en la mama derecha detectada desde el nacimiento. Inicialmente, se trató como una mastitis, pero dada la mala evolución, se plantearon diagnósticos diferenciales y se concluyó que se trataba de un hemangioma. Debido a la ulceración de la lesión, junto con el riesgo existente de desarrollar hipoplasia mamaria, se decidió iniciar tratamiento con propanolol, con resolución casi completa de la tumoración.

Mammary pathology is infrequent during childhood. It is rare the probability of finding a breast mass in an infant. The most frequent possible diagnoses at this age are breast abscess, mastitis, breast engorgement due to maternal hormonal stimulation and hemangioma. Reaching the proper diagnosis is essential in order to apply a suitable treatment and avoid the potential disease complications. We present the case of a female infant having a mass in the right breast from birth. Initially the entity was treated as mastitis. Nevertheless, the bad evolution made necessary considering the differential diagnosis. It was concluded to be a hemangioma. Due to the lesion ulceration and the potential risk of developing breast hypoplasia, treatment with propranolol was started. The tumor was almost completely resolved.

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.

Propranolol inhibits the proliferation, migration and tube formation of hemangioma cells through HIF-1α dependent mechanisms

The aim of this study was to investigate the mechanism of propranolol on the regression of hemangiomas. Propranolol-treated hemangioma tissues were collected and the expression of hypoxia inducible factor-1α (HIF-1α) was examined. We also established HIF-1α overexpression and knockdown hemangioma cells, and determined the effects of HIF-1α on the hemangioma cells proliferation, apoptosis, migration and tube formation. Significantly increased HIF-1α level was found in the hemangioma tissues compared to that in normal vascular tissues, whereas propranolol treatment decreased the HIF-1α level in hemangioma tissues in a time- and dose-dependent manner. Moreover, propranolol treatment significantly decreased cell proliferation, migration and tube formation as well as promoted cell apoptosis in HIF-1α overexpression and knockdown hemangioma cells. Propranolol suppressed the cells proliferation, migration and tube formation of hemangioma cells through HIF-1α dependent mechanisms. HIF-1α could serve as a novel target in the treatment of hemangiomas.

Hemangioma coroideo / Choroidal hemangioma

El hemangioma coroideo circunscrito es un tumor vascular hamartomatoso benigno de baja incidencia. Se presenta este caso debido a su evolución lenta y la pobre respuesta a tratamiento con terapia fotodinámica. El paciente, de sexo masculino, 49 años de edad, con disminución de la visión del ojo derecho de ocho meses de evolución y sin otra sintomatología asociada. Las exploraciones complementarias realizadas (retinografia, OCT, Ecografía y Angiografía flurosceínica) evidencian un tumor yuxtapapilar nasal que es diagnosticado como hemangioma circunscrito de coroides. Se decide tratarlo con terapia fotodinámica. En este caso, aunque el resultado en la ganancia visual no es el esperado, en los exámenes complementarios realizados se observa una discreta mejoría. La lenta evolución de este paciente responde, a criterio de los autores, a varios factores como la edad, el deterioro de la visión en el momento del diagnóstico, el número de aplicaciones de tratamiento con la TFD realizadas, el tiempo de evolución del tumor y el tamaño de la lesión(AU)

Circumscribed choroidal hemangioma is a benign hamartomatous vascular tumor of low incidence. This case is presented for its slow natural history and the poor response to treatment with photodynamic therapy. The 49-year-old male patient with right-eye visual reduction of eight month of evolution and without any other associated symptomatology. Complementary explorations carried out (retinography, OCT, sonogram, fluorescein angiography) evinces a juxtapapillary nasal tumor diagnosed as circumscribed choroidal hemangioma. The decision is to treat it with photodynamic therapy. In this case, despite the fact the visual gain outcome is not the desired, the complementary tests performed revealed a fair improvement. According to the authors, the slow evolution of this patient is due to several factors such as age, visual decay at the time of the diagnosis, the TFD number of applications, the tumor´s time of evolution and the lesion seize(AU)

Tratamento de hemangioma da infância com maleato de timolol tópico / Treatment of infantile hemangioma with topical timolol maleate

Introdução: o hemangioma da infância é uma proliferação neoplásica benigna de células endoteliais, decorrente de um desequilíbrio na angiogênese. Atinge 10 a 12% das crianças com menos de ano de vida, sendo, portanto, o tumor mais comum da infância. O tratamento, geralmente, é expectante, mas são utilizados, em alguns casos, propranolol oral, corticosteroides, interferon alfa-2a, laserterapia, embolização, imunomoduladores e cirurgia. Descrição do caso: criança do sexo masculino com hemangioma em couro cabeludo de 3 cm de diâmetro. Utilizou-se maleato de timolol 0,5%, solução oftálmica, três gotas duas vezes ao dia na superfície da lesão, durante dois anos. Após um ano de tratamento, a lesão apresentava-se praticamente plana, com involução quase completa e alopecia residual. Nenhum efeito colateral foi observado durante o período de tratamento. Discussão: o hemangioma da infância possui amplo espectro clínico e graus variados de gravidade, o que torna o seu manejo difícil e controverso. Diante do alto número de efeitos colaterais descritos para os tratamentos convencionais, o timolol tópico tem se tornado excelente alternativa para os casos não complicados. Conclusão: o timolol tópico vem sendo uma nova opção terapêutica eficaz e desprovida de efeitos colaterais para o tratamento do hemangioma da infância, sendo capaz de acelerar sua involução e prevenir complicações. Entretanto, é necessário que sejam realizados estudos que padronizem a dosagem terapêutica mais segura e avaliem o real risco x benefício do uso do fármaco no tratamento desse tipo de tumor.(AU)

Introduction: Infantile hemangioma is a benign neoplastic proliferation of endothelial cells, resulting from an imbalance in angiogenesis. Reaches 10-12% of children under one year old, therefore it is the most commom tumor of childhood. Treatment is usually expectant, but in some cases can be used oral propranolol, corticosteroids, interferon alpha-2a, laser therapy, embolization, immunomodulators and surgery. Case report: Male child with scalp hemangioma of 3 cm diameter. It was used eye drop solution of timolol maleate 0.5%, three drops, twice daily, on the surface of the lesion, during two years. After one year, it was almost plane and completely involuted with residual alopecia. No side effects were observed during the treatment. Discussion: Infantile hemangioma has a wide spectrum of clinical presentation and varying degrees of severity, which make it difficult and controversial management. Due to the high number of side effects reported for conventional treatments, topical timolol has become an excellent alternative for uncomplicated cases. Conclusion: Topical timolol has been an effective therapeutic option devoid of side effects for treatment of infantile hemangioma, being able to accelerate tumors involution and to prevent complications. Still, it is necessary studies to standardize the safest therapeutic dose and evaluate the actual risk-benefit ratio of timolol use in treatment of this tumor.(AU)

Hemangioma infantil / Infantile haemangioma

Introducción: los hemangiomas infantiles suponen uno de los principales motivos de consulta pediátrica. La nomenclatura para designarlo ha sufrido constantes cambios, lo que ha derivado cierta confusión. Es fundamental el conocimiento de la clasificación actual de las lesiones vasculares que pueden tener características clínicas similares, pero distinta evolución, pronóstico y tratamiento. Objetivo: profundizar y ampliar los conocimientos sobre el diagnóstico y el tratamiento actualizado del hemangioma infantil. Métodos: se realizó una revisión bibliográfica, la localización de artículos fue en las bases de datos informatizadas on-line Medline, Cochrane Library, Lilacs, así como el metabuscador Google. La búsquede se realizó solo entre 2009-2014. Síntesis de la información: los hemangiomas infantiles son los tumores benignos más frecuentes en lactantes. Se observan entre el 1,1 y 2,6 por ciento. Son más frecuentes en la raza blanca, en el sexo femenino (3:1 o 5:1). Se presentan de forma esporádica y pueden localizarse en cualquier región anatómica, con predilección en la cabeza y el cuello. Su diagnóstico es clínico, la mayoría son pequeños y no requieren de tratamiento. Conclusiones: se considera necesario la difusión del conocimiento sobre el hemangioma infantil, ya que somos los receptores iniciales de estos pacientes y su diagnóstico oportuno es vital para iniciar un tratamiento precoz en caso necesario(AU)

Introduction: infantile hemangioma is one of the main reasons for pediatric consultation. The nomenclature used to define this type of lesion has constantly undergone changes throughout history, which has resulted in some confusion. It is essential to have knowledge on the current classification of the different vascular lesions that may have similar clinical characteristics but different progression, prognosis and treatment. Objective: to delve into and broaden knowledge on the current diagnosis and treatment of infantile hemangioma. Methods: a literature review was made to locate articles in the most important on-line databases, including Medline, Cochrane Library, Lilacs, and the meta-searcher Google. The years of publications comprised 2009 through 2014. Data synthesis: infantile hemangiomas are the most frequent benign tumors in breastfed children. They account for 1.1 to 2.6 percent. They are more common in Caucasians, in females (3:1 or 5:1). They occur occasionally and t can be found in any anatomical region, mainly in the head and the neck. Their diagnosis is clinical, most of them are small and do not require treatment. Conclusions: it is necessary to spread the knowledge on infantile hemangioma as we are the first ones to receive these patients, so timely diagnosis is vital to start an early treatment if required(AU)

Propranolol en el tratamiento de los hemangiomas: una alternativa terapéutica / Propranolol in the treatment of hemangiomas: a therapeutic alternative

Introducción: los hemangiomas son los tumores benignos más frecuentes de la infancia. Generalmente involucionan espontáneamente después del año. Un porcentaje pequeño requiere tratamiento debido a complicaciones. Recientemente se ha comunicado beneficio del tratamiento con propranolol. Objetivo: describir la experiencia de tratamiento con propranolol en 6 niños con hemangiomas infantiles (HI) en dos centros asistenciales de Montevideo en el período 1/9/2010 – 31/12/2013. Metodología: se incluyeron los menores de 15 años portadores de hemangiomas asistidos en el Centro Hospitalario Pereira Rossell y en la Asociación Española tratados con propranolol. En todos los casos se realizó evaluación cardiológica previa. Se excluyeron aquellos con contraindicaciones absolutas al fármaco. En todos los casos se solicitó, previo al tratamiento, consentimiento informado. Se describe las características clínicas de los niños y la evolución. Resultados: se incluyeron 6 niños (5 niñas y un varón); rango 1 mes y 21 días a 20 meses. Las topografías fueron: labio superior con compromiso de narina 2, mejilla izquierda 1, raíz nasal e intraorbitario 1, intraocular 1, múltiple 1. En todos los casos la respuesta fue favorable con disminución progresiva de las lesiones en los primeros meses de tratamiento. Ninguno presentó complicaciones. Discusión: ésta es la primera comunicación de una serie de niños con hemangiomas tratados con propranolol en Uruguay. A pesar del escaso número de pacientes, se observó beneficio terapéutico sin complicaciones. El propranolol debe considerarse como una alternativa terapéutica al tratamiento con glucocorticoides sistémicos, cuya administración prolongada se asocia con serios efectos adversos.

Introduction: hemangiomas are the most common benign tumors of childhood. They usually regress spontaneously after the year of life. A small percentage require treatment due to complications. Recently it has been reported the benefit of treatment with propranolol. Objective: describe the experience of treatment with propranolol in 6 children with infantile hemangiomas (HI) in two centers of Montevideo in the period 1/9/2010-31/12/2013.Methodology: included infants under de age of 15 carriers of hemangiomas assisted in the Pereira Rossell hospital and the Spanish Association treated with propranolol. Cardiological assessment was previously performed in all cases. Those with absolute contraindications to the drug were excluded. Informed consent was requested prior to the treatment in all cases. The clinical features of children and evolution are described. Results: 6 children were included (5 girls and a male); range 1 month and 21 days to 20 months. The surveys were: upper lip with commitment of nostril 2, left cheek 1, nasal root and intraorbitary 1, intraocular 1, multiple 1. In all cases the response was favorable with progressive decrease in injuries in the first month of treatment. None of the patients presented complications. Discussion: this is the first communication of a series of children with hemangioma treated with propranolol in Uruguay. Despite the small number of patients therapeutic benefit without complications was observed. Propranolol should be considered as a therapeutic alternative to treatment with systemic glucocorticoids whose prolonged administration is associated with serious adverse effects.