Papilomatosis laringea recurrente con compromiso pulmonar: reporte de caso / Recurrent laryngeal papillomatosis with pulmonary involvement: case report

La papilomatosis respiratoria recurrente es una enfermedad neoplásica causada por el virus del papiloma humano y caracterizada por el crecimiento de lesiones proliferativas exofíticas que afectan la mucosa de las vías respiratorias. En su epidemiología se presenta una distribución bimodal, con una forma juvenil en menores de 20 años, más agresiva, con múltiples lesiones papilomatosas y alta frecuencia de recurrencia, en comparación con la forma adulta. El compromiso pulmonar es poco frecuente y su manejo es un desafío. Se presenta el caso de un varón de 13 años con antecedente de papilomatosis laríngea desde los dos años. El paciente mostró dificultad respiratoria y múltiples nódulos estenosantes en laringe y tráquea, y varios quistes pulmonares visualizados en la tomografía de tórax. Se le realizó exéresis de las lesiones papilomatosas y traqueostomía. Recibió dosis única de bevacizumab 400 mg intravenoso y terapias respiratorias con evolución favorable, sin recurrencias en el seguimiento.

Recurrent respiratory papillomatosis is a neoplastic disease caused by the human papillomavirus and characterized by the growth of exophytic proliferative lesions affecting the mucosa of the respiratory tract. This condition has a bimodal age distribution; the juvenile form affects those under 20 years of age, is more aggressive and presents multiple papillomatous lesions and high frequency of recurrence, compared to the adult form. Pulmonary involvement is rare and challenging to treat. We present the case of a 13-year-old male with a history of laryngeal papillomatosis since the age of two years. The patient showed respiratory distress and multiple stenosing nodules in the larynx and trachea, as well as several pulmonary cysts identified on chest CT. The patient underwent excision of the papillomatous lesions and tracheostomy. Then, the patient received a single dose of intravenous bevacizumab 400 mg and respiratory therapies with favorable evolution, without recurrences during follow-up.

Efficacy of cidofovir versus bevacizumab in recurrent respiratory papillomatosis: A randomized, double-blind, placebo-controlled pilot study.

OBJECTIVES: After surgical resection of papilloma, adjuvant therapy may be recommended for the control of recurrent respiratory papillomatosis (RRP). As the efficacy of adjuvant therapy remains unproven, the aim of this study was to compare the efficacy of cidofovir versus bevacizumab used as adjuvant therapies for the control of RRP. METHODS: This randomized, double-blind, placebo-controlled pilot study was performed in a national respiratory disease referral centre. Patients with RRP were recruited prospectively and were divided into juvenile or adult RRP. Participants were randomly assigned to receive adjuvant therapy with cidofovir, bevacizumab or placebo. The study drug or placebo was administered after direct microlaryngoscopy with papilloma resection using cold instruments. The Derkay severity score and the Voice Handicap Index (VHI) were assessed at 3-6-week intervals, for a total of 3 visits. Follow-up included VHI and Derkay score assessments at 2-month intervals over the course of one year. Annual rates before and after surgical treatment were compared. RESULTS: Five children and 11 adults were enrolled in the study. After one year, the group treated with cidofovir had a significant decrease in Derkay score (p=.027). No difference between treatment arms was observed in the annual surgery rate. There was a significant decrease in the VHI score in all treatment groups (p<.001), and no significant difference was observed between groups (p=.32). CONCLUSION: While we observed a significant decrease in RRP severity with intralesional cidofovir, we were unable to provide proof of efficacy of intralesional bevacizumab. CLINICALTRIALS: gov Identifier: NCT02555800.

Eficacia de cidofovir frente a bevacizumab en la papilomatosis respiratoria recurrente: estudio piloto aleatorizado, doble ciego y controlado por placebo / Efficacy of cidofovir versus bevacizumab in recurrent respiratory papillomatosis: A randomized, double-blind, placebo-controlled pilot study

Objectives: After surgical resection of papilloma, adjuvant therapy may be recommended for the control of recurrent respiratory papillomatosis (RRP). As the efficacy of adjuvant therapy remains unproven, the aim of this study was to compare the efficacy of cidofovir versus bevacizumab used as adjuvant therapies for the control of RRP. Methods: This randomized, double-blind, placebo-controlled pilot study was performed in a national respiratory disease referral centre. Patients with RRP were recruited prospectively and were divided into juvenile or adult RRP. Participants were randomly assigned to receive adjuvant therapy with cidofovir, bevacizumab or placebo. The study drug or placebo was administered after direct microlaryngoscopy with papilloma resection using cold instruments. The Derkay severity score and the Voice Handicap Index (VHI) were assessed at 3–6-week intervals, for a total of 3 visits. Follow-up included VHI and Derkay score assessments at 2-month intervals over the course of one year. Annual rates before and after surgical treatment were compared. Results: Five children and 11 adults were enrolled in the study. After one year, the group treated with cidofovir had a significant decrease in Derkay score (p=.027). No difference between treatment arms was observed in the annual surgery rate. There was a significant decrease in the VHI score in all treatment groups (p<.001), and no significant difference was observed between groups (p=.32). Conclusion: While we observed a significant decrease in RRP severity with intralesional cidofovir, we were unable to provide proof of efficacy of intralesional bevacizumab. (AU)

Objetivos: Tras la extirpación quirúrgica de los papilomas, puede recomendarse terapia adyuvante para el control de la papilomatosis respiratoria recurrente (PRR). Como la eficacia de la terapia adyuvante no está demostrada, el objetivo de este estudio fue comparar la eficacia de cidofovir frente a bevacizumab, utilizados como terapias adyuvantes para el control de PRR. Métodos: Estudio piloto aleatorizado, doble ciego y controlado por placebo, realizado en un centro nacional de referencia de enfermedades respiratorias. Se reclutó prospectivamente a los pacientes con PRR, y se dividieron en 2 grupos: PRR de jóvenes y PRR de adultos. A los participantes se les asignó aleatoriamente la administración de terapia adyuvante de cidofovir, bevacizumab o placebo. La administración de los fármacos de estudio o placebo se realizó tras practicar microlaringoscopia directa con extirpación de papilomas, utilizando instrumental frío. Se llevaron a cabo evaluaciones utilizando la puntuación de gravedad de Derkay y la escala VHI (Voice Handicap Index) a intervalos de 3 a 6 semanas, con un total de 3 visitas. El seguimiento incluyó evaluación de las escalas VHI y Derkay a intervalos de 2 meses, durante el curso de un año. Se compararon las tasas anuales pre- y postratamiento quirúrgico. Resultados: El estudio incluyó 5 niños y 11 adultos. Transcurrido un año, el grupo tratado con cidofovir reflejó una reducción significativa en la escala Derkay (p=0,027). No se observó diferencia alguna entre las ramas terapéuticas en la tasa quirúrgica anual. Se vio una reducción significativa en la puntuación VHI en todos los grupos terapéuticos (p<0,001), y no se observó diferencia significativa entre los grupos (p=0,32). Conclusión: A pesar de verse una reducción significativa de la gravedad de PRR con la administración de cidofovir intralesional, no pudimos probar la eficacia de bevacizumab intralesional. (AU)

Papilomatosis confluente y reticulada de Gougerot-Carteaud / CONFLUENT AND RETICULATED PAPILLOMATOSIS OF GOUGEROT-CARTAUD

La papilomatosis confluente y reticulada de Gougerot-Cartaud es una dermatosis poco frecuente, de etiología incierta. Afecta a adolescentes y adultos jóvenes, con leve predilección por el sexo masculino. Se presenta como pápulas parduzcas que confluyen formando placas centrales con patrón reticulado en la periferia, asintomáticas o levemente pruriginosas. La localización característica es tronco anterior y posterior, a nivel de la línea media. Los antibióticos orales, principalmente la minociclina, constituyen el tratamiento de elección. Debe diferenciarse de otras entidades, entre ellas pitiriasis versicolor, acantosis nigricans y dermatosis terra firma-forme. Se presenta una paciente de 17 años con papilomatosis confluente y reticulada de GougerotCarteaud que respondió satisfactoriamente al tratamiento con minociclina vía oral y tretinoína 0,025% tópica.

Confluent and reticulated papillomatosis of Gougerot-Cartaud is a rare dermatosis, of still uncertain etiology. It affects adolescents and young adults, with a slight predilection for males. It presents as asymptomatic or slightly pruritic brownish papules that converge to form central plaques with a reticulated pattern on the periphery. The characteristic location is midline anterior and posterior trunk. Oral antibiotics, mainly minocycline, are the treatment of choice. It must be differentiated from other entities, including pityriasis versicolor, acanthosis nigricans, and terra firme-forme dermatosis. We present a 17-year-old patient with confluent Gougerot-Carteaud papillomatosis who responded satisfactorily to treatment with oral minocycline and topical tretinoin 0.025%.

Papilomatosis confluente y reticulada de Gougerot-Carteaud / Confluent and reticulated papillomatosis of Gougerot-Carteaud

La papilomatosis confluente y reticulada es una dermatosis infrecuente, benigna, de curso crónico y etiopatogenia desconocida. Actualmente, se acepta que se debe a un trastorno de la queratinización. Se caracteriza por máculas y pápulas hiperpigmentadas, que coalescen en el centro y adoptan un patrón reticular en la periferia. Se la puede confundir con otras patologías, como la pitiriasis versicolor y la acantosis nigricans. Con la administración de minociclina por vía oral suelen obtenerse excelentes resultados. Se comunica el caso de un paciente con diagnóstico de papilomatosis confluente y reticulada. Se describen sus características clínicas, criterios diagnósticos y trata-miento instaurado.

Confluent and reticulated papillomatosis is an infrequent, benign dermatosis of chronic course and unknown etiopathogenesis. Currently it is accepted that this dermatosis is due to a keratinization disorder. It is characterized by hyperpigmented macules and papules that coalesce in the center and adopt a reticular pattern in the periphery. It can be confused with other pathologies such as pityriasis versicolor and acanthosis nigricans. Oral minocycline usually shows excellent results. The present work reports a case of a patient with a diagnosis of confluent and retic-ulated papillomatosis, describing its clinical characteristics, diagnostic criteria and established treatment.

Successful cholangioscopic electrocoagulation for biliary papillomatosis: Report covering six cases (with video) / Electrocoagulación colangioscópica exitosa para la papilomatosis biliar: Informe de 6 casos (con vídeo)

Background: Biliary papillomatosis is a rare type of papillomatosis. Depending on the location of the disease, radical surgical resection or liver transplants are the treatment of choice. These radical surgical options may be unrealistic in patients who are not surgical candidates or who are unwilling to consider surgery. Aims: To evaluate the effectiveness of endoscopic electrocoagulation for the biliary papillomatosis. Methods: In this case series, we report six patients with unresectable biliary papillomatosis who underwent cholangioscopic electrocoagulation using needle knife and their clinic follow up information. Results: After patients received cholangioscopy with electrocoagulation of the residual biliary papilloma, the daily T-tube drainage volume increased to 200–400ml with improvement in the drainage content and significant relief of clinical symptoms, such as jaundice and abdominal pain. Conclusion: This method of using electrocoagulation to directly target and destroy tumor tissue is a safe and effective alternative for those with unresectable disease, and this method has shown to enhance T-tube drainage volume and improve patients’ overall clinical symptoms.(AU)

Antecedentes: La papilomatosis biliar es un tipo raro de papilomatosis. Dependiendo de la localización de la enfermedad, la resección quirúrgica radical o los trasplantes de hígado son el tratamiento de elección. Estas opciones quirúrgicas radicales pueden ser poco realistas en pacientes que no son candidatos a la cirugía o que no están dispuestos a considerarla. Objetivos: Evaluar la eficacia de la electrocoagulación endoscópica para la papilomatosis biliar. Métodos: En esta serie de casos, informamos de 6 pacientes con papilomatosis biliar irresecable que fueron sometidos a electrocoagulación colangioscópica mediante bisturí de aguja, y de su información de seguimiento clínico. Resultados: Después de que los pacientes recibieran la colangioscopia con electrocoagulación del papiloma biliar residual, el volumen de drenaje diario del tubo en T aumentó a 200-400ml con mejora del contenido del drenaje y alivio significativo de los síntomas clínicos, como la ictericia y el dolor abdominal. Conclusión: Este método de uso de la electrocoagulación para apuntar directamente y destruir el tejido tumoral es una alternativa segura y eficaz para aquellos con enfermedad no resecable, y este método ha demostrado mejorar el volumen de drenaje del tubo T y mejorar los síntomas clínicos generales de los pacientes.(AU)

Rol del virus papiloma humano en cabeza y cuello

Resumen El virus papiloma humano (VPH) es un virus de ADN circular doble hebra con alrededor de 200 genotipos. Este virus se relaciona a distintas lesiones tumorales que afectan cabeza y cuello, incluyendo lesiones malignas escamosas causadas por genotipos de alto riesgo como VPH-16 y VPH-18. Esta revisión tiene por objetivo determinar el rol del VPH en distintas patologías, haciendo distinción entre lesiones benignas y malignas, y con particular énfasis en aquellas en que se ha demostrado una asociación causal con el virus, tales como papilomatosis laríngea y carcinoma escamoso orofaríngeo. Además, se analizarán los mecanismos moleculares de daño, métodos de detección y de prevención como la vacunación contra el virus. Es necesario conocer la relevancia del VPH en nuestra especialidad, ya que su determinación puede tener implicancias en cuanto al manejo y pronóstico de los pacientes.

Abstract Human papillomavirus (HPV) is a double stranded circular DNA virus with around 200 genotypes. This virus is related to different tumor lesions that affect the head and neck, including malignant squamous lesions caused by high-risk genotypes such as HPV-16 and HPV-18. The objective of this review is to determine the role of HPV in different pathologies, distinguishing between benign and malignant lesions, and with particular emphasis on those in which a causal association with the virus has been demonstrated, such as laryngeal papillomatosis and oropharyngeal squamous carcinoma. In addition, molecular damage mechanisms, detection and prevention methods such as vaccination against the virus will be analyzed. It is necessary to know the relevance of HPV in our specialty, since its determination may have implications in terms of patient management and prognosis.

Efficacy of cidofovir versus bevacizumab in recurrent respiratory papillomatosis: A randomized, double-blind, placebo-controlled pilot study.

OBJECTIVES: After surgical resection of papilloma, adjuvant therapy may be recommended for the control of recurrent respiratory papillomatosis (RRP). As the efficacy of adjuvant therapy remains unproven, the aim of this study was to compare the efficacy of cidofovir versus bevacizumab used as adjuvant therapies for the control of RRP. METHODS: This randomized, double-blind, placebo-controlled pilot study was performed in a national respiratory disease referral centre. Patients with RRP were recruited prospectively and were divided into juvenile or adult RRP. Participants were randomly assigned to receive adjuvant therapy with cidofovir, bevacizumab or placebo. The study drug or placebo was administered after direct microlaryngoscopy with papilloma resection using cold instruments. The Derkay severity score and the Voice Handicap Index (VHI) were assessed at 3-6-week intervals, for a total of 3 visits. Follow-up included VHI and Derkay score assessments at 2-month intervals over the course of one year. Annual rates before and after surgical treatment were compared. RESULTS: Five children and 11 adults were enrolled in the study. After one year, the group treated with cidofovir had a significant decrease in Derkay score (p=.027). No difference between treatment arms was observed in the annual surgery rate. There was a significant decrease in the VHI score in all treatment groups (p<.001), and no significant difference was observed between groups (p=.32). CONCLUSION: While we observed a significant decrease in RRP severity with intralesional cidofovir, we were unable to provide proof of efficacy of intralesional bevacizumab. Clinicaltrials.gov Identifier: NCT02555800.

Successful cholangioscopic electrocoagulation for biliary papillomatosis: Report covering six cases (with video).

BACKGROUND: Biliary papillomatosis is a rare type of papillomatosis. Depending on the location of the disease, radical surgical resection or liver transplants are the treatment of choice. These radical surgical options may be unrealistic in patients who are not surgical candidates or who are unwilling to consider surgery. AIMS: To evaluate the effectiveness of endoscopic electrocoagulation for the biliary papillomatosis. METHODS: In this case series, we report six patients with unresectable biliary papillomatosis who underwent cholangioscopic electrocoagulation using needle knife and their clinic follow up information. RESULTS: After patients received cholangioscopy with electrocoagulation of the residual biliary papilloma, the daily T-tube drainage volume increased to 200-400ml with improvement in the drainage content and significant relief of clinical symptoms, such as jaundice and abdominal pain. CONCLUSION: This method of using electrocoagulation to directly target and destroy tumor tissue is a safe and effective alternative for those with unresectable disease, and this method has shown to enhance T-tube drainage volume and improve patients' overall clinical symptoms.

Trends in recurrent respiratory papillomatosis treatment. / Tendencias en el tratamiento de la papilomatosis respiratoria recurrente.

Recurrent respiratory papillomatosis (RRP) consist of benign tumours along the airway caused by human papillomavirus infection. Papillomas may cause changes in phonation and obstruct the airway. The purpose of this study was to evaluate the different surgical and adjuvant therapies available for the treatment of this condition reported between 2014-2018. A PubMed search was performed for RRP treatment articles published between 2014 -2018. Forty articles that encompassed 1425 patients with RRP met the criteria. Of these, 24 articles evaluate the use of adjuvant therapies such as bevacizumab, human papillomavirus vaccine and cidofovir. There has been an increase in adjuvant therapy options for RRP in the literature in recent years. An awareness of these options highlights gaps and opportunities in the care of these patients, opens the door to new protocols to control disease and increase intersurgical intervals, and guides us towards new management paradigms in the future.

Caracterización de pacientes con papiloma laríngeo / Characteristics of patients with lagyngeal papillomatosis

La papilomatosis laríngea es la neoplasia laríngea benigna más común y está causada por el virus de papiloma humano. Se considera que la papilomatosis laríngea tiene un papel independiente como factor de riesgo de cáncer; se clasifica dentro de tres grupos de acuerdo con sus características clínicas: juvenil, adulto y recurrente. Su incidencia asociada a neoplasia maligna es del 4%. Objetivos: Determinar si existen factores de riesgo asociados al tipo de papilomatosis laríngea; y si existe relación con el tipo de papilomatosis y neoplasias asociadas. Metodología: Estudio observacional descriptivo de pacientes que acuden al servicio de Otorrinolaringología del Hospital General San juan de Dios y su caracterización. Resultados: De los 33 pacientes evaluados con papilomatosis laríngea, 51 % fueron del género masculino, la edad promedio sin incluir pacientes pediátricos fue de 23 años. La mediana de reintervenciones es de 4 veces, y la mayoría tiene pérdida del seguimiento. La papilomatosis tipo infantil es la más común con pacientes de 7 a 14 años de edad. Los pacientes con papilomatosis adulta presentaron más factores de riesgo que los pacientes con papilomatosis infantil y recurrente, p=0.01. Los pacientes presentan ronquera, disfonía y dificultad respiratoria. El diagnóstico se realiza por clínica con laringoscopía sin biopsia en la mitad de los pacientes. El 9% de los pacientes estudiados presentaron neoplasia asociada. El número de reintervenciones está asociado a un mayor riesgo de progresión a neoplasia. Conclusión: Los pacientes con papilomatosis adulta presentaron más factores de riesgo que los pacientes con papilomatosis infantil, y el 9% de los pacientes presentan una neoplasia asociada, más alta de la reportada en la literatura.(AU)

Laryngeal papillomatosis is the most common benign neoplasm and is caused by the human papilloma virus. Laryngeal papillomatosis is considered to have an independent role as risk factor. Laryngeal papillomas are classified into three groups, according to their clinical characteristics: juvenile, adult and recurrent. 4% is associated with neoplasia. Objectives: Determine if there are risk factors associated with the type of laryngeal papillomatosis and if there is a relationship with the type of papillomatosis and associated neoplasms. Methodology: Descriptive observational study of patients attending the Otolaryngology service of the General Hospital San Juan de Dios de Guatemala and their characterization. Results: Of the 33 patients evaluated with laryngeal papillomatosis, 51% were male, with an average age of 23 years. The median number of reoperations is 4, and most do not return to follow-up. Infantile type papillomatosis is the most common, with patients 7 to 14 years of age. Patients with adult papillomatosis had more risk factors than patients with infantile and recurrent papillomatosis, p= 0.01. Patients present hoarseness, dysphonia and respiratory distress upon admission. Diagnosis is made clinically with laryngoscopy without biopsy in half of the patients. 9% of the patients studied had associated neoplasia. The number of reoperations is associated with an increased risk of developing a neoplasm. Conclusion: Patients with adult papillomatosis presented more risk factors than patients with infantile papillomatosis. And 9% of the patients present associated neoplasia, higher than reported in the literature

Caracterización clinicoepidemiológica y terapéutica de niños con papilomatosis respiratoria recurrente / Clinical, epidemiological and therapeutical characterization of children with recurrent respiratory papillomatosis

Introducción: La papilomatosis respiratoria recurrente es una enfermedad infrecuente de la vía aérea que afecta a niños y adultos, causada por el papilomavirus. Objetivo: Caracterizar niños con papilomatosis respiratoria recurrente desde los puntos de vista clínico, epidemiológico y terapéutico, así como evaluar su evolución a corto y mediano plazos. Métodos: Se realizó una investigación observacional, longitudinal y prospectiva de 40 niños con papilomatosis respiratoria recurrente, atendidos en el Servicio de Otorrinolaringología del Hospital Infantil Docente Sur Dr. Antonio María Béguez César de Santiago de Cuba, desde enero del 2010 hasta diciembre del 2016. Resultados: Los pacientes incluidos en el estudio representaron 3,2 % del total de los ingresos. Predominó el sexo masculino (60,0 %); el promedio de edad fue de 8,6 ± 4 años. Existió asociación estadística entre la localización anatómica de las lesiones y el sexo masculino; sin embargo, no hubo dicha asociación con la edad. Las localizaciones orofaríngea (50,0 %) y laríngea (42,5 %) fueron las de mayor observancia. El parto transvaginal se asoció con el mayor número de pacientes que presentaron la citada afección (85,0 %) y el tratamiento con exéresis e interferón se asoció a la evolución satisfactoria en 95,8 % de los afectados. Conclusiones: Las características clínicas y epidemiologias de los pacientes con papilomatosis respiratoria recurrente de esta casuística no difieren del contexto epidemiológico mundial, siendo una entidad de observación poco frecuente, pero no rara en los servicios de pediatría.

Introduction n The recurrent respiratory papillomatosis is an uncommon disease of the airways that affects children and adults, caused by the papillomavirus. Objective: To characterize children with recurrent respiratory papillomatosis from the clinical, epidemiologic and therapeutic points of view, as well as to evaluate their clinical course at short and mid terms. Methods: An observational, longitudinal and prospective investigation of 40 children with recurrent respiratory papillomatosis was carried out, they were assisted in the Otolaryngology Service of Dr. Antonio María Béguez Cesar Teaching Southern Children Hospital in Santiago de Cuba, from January, 2010 to December, 2016. Results: The patients included in the study represented 3.2 % of the total of admissions. The male sex prevailed (60.0 %); the average age was 8.6 ± 4 years. Statistical association existed between the anatomical localization of the lesions and the male sex; however, there was no such association with the age. The oropharyngeal (50.0 %) and laryngeal (42.5 %) localizations were those of more observance. The transvaginal childbirth was associated with the higher number of patients that presented the mentioned disorder (85.0 %) and the treatment with exeresis and Interferon was associated with the satisfactory clinical course in 95.8 % of those affected. Conclusions: The clinical and epidemiological characteristics of patients with recurrent respiratory papillomatosis of this case material don't differ from the world epidemiological context, being an unusual observation entity, but not strange in the pediatrics services.

Características clínicas y epidemiológicas de la papilomatosis respiratoria recurrente en pacientes pediátricos / Clinical and epidemiological characteristics of recurrent respiratory papillomatosis in pediatric patients

La papilomatosis respiratoria recurrente (PRR) es la segunda causa más frecuente de disfonía durante la infancia, cuyo agente causal más frecuente es el virus del papiloma humano serotipos 6 y 11. Con el objetivo de analizar las características clínicas y epidemiológicas de la PPR en pacientes pediátricos admitidos en el Servicio Desconcentrado Hospital Pediátrico Dr. Agustín Zubillaga, se realizó un estudio descriptivo transversal de recolección retrospectiva de datos durante el lapso 2011-2016. Entre las características sociodemográficas se encontró una edad promedio para el momento del diagnóstico de 4,4 ± 2,41 años, siendo los más afectados los preescolares (70%) sin predilección por sexo. Como antecedente prenatal, 90% fue producto de parto vaginal y 30% reportaron asma e infección respiratoria baja. Las características clínicas presentes fueron disfonía (90%), disnea (70%) y estridor (60%) y la localización de las lesiones fueron comisura anterior (100%), repliegues vocales (80% derecho y 90% izquierdo) y vestíbulo (40%). Se reportaron 40% de casos de VPH-6 y 10% de VPH-11. El tratamiento fue quirúrgico en el 100% de los casos, de los cuales 50% requirió una segunda poda y 30% tres podas; 10% de los pacientes ameritaron traqueotomía. Este estudio aporta información sobre la importancia de reconocer tempranamente la disfonía persistente, lo que permite disminuir la demora en el diagnóstico de PRR y facilitar un manejo oportuno con menores tasas de recidiva a largo plazo(AU)

Recurrent respiratory papillomatosis (RRP) is the second most frequent cause of dysphonia during childhood; the most frequent causative agent is human papillomavirus serotypes 6 and 11. In order to describe the clinical and epidemiological characteristics of RRP In pediatric admitted to the Servicio Desconcentrado Hospital Pediátrico Dr. Agustín Zubillaga, a cross-sectional descriptive study was conducted during the 2011-2016 period. The average age of diagnosis was 4.4 ± 2.41 years, with the highest prevalence in preschool children (70%) and with no sex predilection. 90% of patients were product of vaginal delivery and 30% reported asthma and low respiratory infection. The clinical features present were dysphonia (90%), dyspnea (70%) and stridor (60%); location of the lesions were anterior commissure (100%), vocal folds (80% right and 90% left) and vestibule (40%). 40% of patients reported HPV-6 and 10% HPV-11. Treatment was surgical in 100% of cases of which 50% required a second pruning and 30% three pruning; 10% needed a tracheotomy. This study provides information on the importance of early recognition of persistent dysphonia allowing to reduce diagnosis delay of RRP and facilitate timely management with lower rates of long-term recurrence(AU)

Síndrome de Goltz o Hipoplasia dérmica focal / Goltz Syndrome or focal dermal hypoplasia

RESUMEN Introducción: El síndrome de Goltz o hipoplasia dérmica focal es una enfermedad genética rara del grupo de las displasias ectodérmicas con un mecanismo de herencia dominante ligado al cromosoma X. Objetivo: Describir las características clínicas del síndrome de Goltz, su diagnóstico y tratamiento. Presentación del caso: Paciente femenina de 4 años de edad diagnosticada con síndrome de Goltz. Se valora en equipo multidisciplinario con las especialidades de genética, cirugía maxilofacial, estomatología, dermatología, oftalmología, ortopedia y el servicio de otorrinolaringología. Conclusiones: El síndrome de Goltz se caracteriza principalmente por afectación cutánea; anomalías oculares, dentales, faciales y esqueléticas; afectación del aparato gastrointestinal, urinario, cardiovascular y sistema nervioso central con grado variable de severidad. Su diagnóstico es clínico. La atención interdisciplinaria es fundamental para el adecuado diagnóstico y tratamiento; su pronóstico depende del grado de afectación(AU)

ABSTRACT Introduction: Goltz syndrome also known as focal dermal hypoplasia is a rare genetic disease in the ectodermal dysplasia´s group and with a mechanism of dominant inheritance linked to the X chromosome. Objectives: To describe the clinical characteristics of the Goltz syndrome, its diagnosis and treatment. Case presentation: Case of a 4 year-old female patient diagnosed with Goltz syndrome. She was studied by a multidisciplinary team including Genetics, Maxillofacial Surgery, Stomatology, Dermatology, Ophthalmology, Orthopedics and ORL specialists. Conclusions: Goltz syndrome or focal dermal hypoplasia is mainly characterized by skin affectations; eyes, dental, skeletal, and face anomalies; gastrointestinal tract, urinary, cardiovascular and central nervous systems´ affections with varying degrees of severity. The diagnosis is clinical. A multidisciplinary approach is essential for a proper diagnosis and treatment; and prognosis depends on the grade of severity(AU)

Síndrome de Goltz o Hipoplasia dérmica focal / Goltz Syndrome or focal dermal hypoplasia

Introducción: El síndrome de Goltz o hipoplasia dérmica focal es una enfermedad genética rara del grupo de las displasias ectodérmicas con un mecanismo de herencia dominante ligado al cromosoma X. Objetivo: Describir las características clínicas del síndrome de Goltz, su diagnóstico y tratamiento. Presentación del caso: Paciente femenina de 4 años de edad diagnosticada con síndrome de Goltz. Se valora en equipo multidisciplinario con las especialidades de genética, cirugía maxilofacial, estomatología, dermatología, oftalmología, ortopedia y el servicio de otorrinolaringología. Conclusiones: El síndrome de Goltz se caracteriza principalmente por afectación cutánea; anomalías oculares, dentales, faciales y esqueléticas; afectación del aparato gastrointestinal, urinario, cardiovascular y sistema nervioso central con grado variable de severidad. Su diagnóstico es clínico. La atención interdisciplinaria es fundamental para el adecuado diagnóstico y tratamiento; su pronóstico depende del grado de afectación(AU)

Introduction: Goltz syndrome also known as focal dermal hypoplasia is a rare genetic disease in the ectodermal dysplasia´s group and with a mechanism of dominant inheritance linked to the X chromosome. Objectives: To describe the clinical characteristics of the Goltz syndrome, its diagnosis and treatment. Case presentation: Case of a 4 year-old female patient diagnosed with Goltz syndrome. She was studied by a multidisciplinary team including Genetics, Maxillofacial Surgery, Stomatology, Dermatology, Ophthalmology, Orthopedics and ORL specialists. Conclusions: Goltz syndrome or focal dermal hypoplasia is mainly characterized by skin affectations; eyes, dental, skeletal, and face anomalies; gastrointestinal tract, urinary, cardiovascular and central nervous systems´ affections with varying degrees of severity. The diagnosis is clinical. A multidisciplinary approach is essential for a proper diagnosis and treatment; and prognosis depends on the grade of severity(AU)

Papilomatosis traqueobronquial y pulmonar sin compromiso de la laringe tratada con bevacizumab endovenoso en un niño / Tracheobronchial and pulmonary papillomatosis without involvement of the larynx treated with intravenous Bevacizumab in a child

La papilomatosis respiratoria recurrente es una neoplasia benigna infrecuente que, comúnmente, afecta la vía aérea superior con predilección por la laringe. El compromiso traqueobronquial aislado es muy raro. El diagnóstico y el tratamiento de esta enfermedad constituyen un desafío, debido a su manifestación clínica inespecífica y su naturaleza recurrente. Se presenta a un varón de 6 años con diagnóstico de asma refractario al tratamiento, sin historia ni evidencia de papilomatosis laríngea. El examen endoscópico reveló papilomatosis traqueobronquial extensa, y la tomografia computada, compromiso pulmonar. Recibió terapia adyuvante con bevacizumab endovenoso, con muy buena respuesta. Se alerta a los pediatras para considerar esta rara neoplasia traqueobronquial en todo niño con síntomas de asma atípicos o que no mejoran con el tratamiento médico convencional.

Recurrent respiratory papillomatosis is an infrequent benign neoplasm that commonly affects the upper airway with a predilection for the larynx. Isolated tracheobronchial involvement is very rare. Diagnosis and treatment of this disease is a challenge due to its non-specific clinical manifestation and its recurrent nature. We present a 6-year-old male with a diagnosis of asthma refractory to treatment, without history or evidence of laryngeal papillomatosis. The endoscopic examination revealed extensive tracheobronchial papillomatosis and the computed tomography, pulmonary involvement. He received adjuvant therapy with intravenous Bevacizumab with very good response. We alert pediatricians to consider this rare tracheobronchial neoplasm in all children with atypical asthma symptoms or in those who do not improve with conventional medical treatment.

Vacuna contra el virus papiloma humano como tratamiento para la papilomatosis respiratoria recurrente / Human papilloma virus vaccination treatment for recurrent respiratory papillomatosis

RESUMEN Una de las manifestaciones clínicas del virus papiloma humano (VPH) es la papilomatosis respiratoria recurrente (PRR), que se caracteriza por la proliferación de lesiones epiteliales verrucosas recurrentes en la mucosa respiratoria, pudiendo progresar a obstrucción de vía aérea o presentar transformación maligna. El tratamiento de primera línea quirúrgico, pero dada su alta recurrencia ha tomado peso el tratamiento adyuvante, como la vacuna tetravalente contra VPH. Sin embargo, existe controversia respecto a su eficacia. El objetivo de esta revisión es analizar la efectividad de la vacuna contra VPH como tratamiento adyuvante de la PRR, para lo cual hicimos una revisión de la literatura sobre la efectividad de la vacuna tetravalente contra VPH para PRR, realizando una búsqueda en diversas fuentes: Pubmed, MEDLINE, EMBASE, Cochrane, Google Scholar y Epistemonikos. Se seleccionaron los estudios que responden a la pregunta y se analizaron los datos de los estudios primarios. Se encontraron cinco estudios primarios no aleatorizados, todos a favor de la vacuna como tratamiento. Concluimos con baja certeza de evidencia, que la vacuna es posiblemente efectiva para pacientes con PRR en disminuir el número de recurrencias, aumentar el intervalo entre cirugías, lograr remisión completa o parcial de la enfermedad y aumentar significativamente los títulos de anticuerpos anti-VPH.

ABSTRACT A clinical manifestation of human papillomavirus (HPV) is recurrent respiratory papillomatosis (RRP), characterized by the proliferation of recurrent verrucous epithelial lesions in the respiratory mucosa, which may progress to airway obstruction or malignant transformation. First-line treatment is surgery, but given its high recurrence the use of adjuvant therapy, such as the quadrivalent vaccine, has gained importance. However, there is controversy regarding its effectiveness. To analyze the effectiveness of the HPV vaccine as an adjuvant treatment for RRP a review of the literature on the effectiveness of HPV tetravalent vaccine for RRP was performed by searching databases such as Pubmed, MEDLINE, EMBASE, Cochrane, Google Scholar and Epistemonikos. We selected the studies that answered the question and analyzed the data from all of which supported the vaccine as treatment. None were randomized controlled trials. We conclude, with low certainty of evidence, that the vaccine is possibly effective for RRP in decreasing the number of recurrences, increasing the intersurgical interval, achieving complete or partial remission of disease and significantly increasing anti-HPV antibodies.

Paciente con papilomatosis laringo-traqueo-bronquial recurrente y empiema at necessitates Dificultades de diagnóstico y manejo / Patient with recurrent laryngeal-tracheo-bronchial papillomatosis and empyema at necessitates Difficulties in diagnosis and management

Resumen Paciente masculino de 17 años, con cuadro de PLR desde los 18 meses, al que se le han realizado 85 intervenciones a nivel laringo-traqueal, por lo que requirió traqueostomía permanente desde los cinco años, presentando cuadros de neumonías a repetición con invasión papilomatosa a pulmón de predominio derecho con aislamiento de actinomicetos; desarrolló empiema at neccesitatis del que se aislaron múltiples gérmenes multirresistentes con severo compromiso del parénquima pulmonar por lo que requirió neumonectomía radical derecha en intento de controlar la infección. Fue manejado con anfotericina B deoxicolato, meropenem, tigeciclina y polimixina B a pesar de lo cual presenta empeoramiento del cuadro clínico con posterior choque séptico refractario que lo llevó a la muerte.

Abstract A 17-year-old male patient, with a clinical picture of RRP since18 months of age, who underwent 85 interventions at the laryngo-tracheal level, requiring permanent tracheostomy from the age of 5, presenting recurrent pneumonia with lung papillomatous invasion of right predominance with actinomycete isolation. He developed empyema at neccesitatis from which multiple multiresistant organisms with severe compromise of the pulmonary parenchyma were isolated, which required a right radical pneumonectomy in an attempt to control the infection. He was managed with am-photericin B deoxycholate, meropenem, tigecycline and polymyxin B, despite which he presented worsening of the clinical picture with subsequent refractory septic shock that led to his death.