Cirugía de Young para el tratamiento quirúrgico de la epistaxis en telangiectasia hemorrágica hereditaria: reporte de un caso y revisión de la literatura

La telangiectasia hemorrágica hereditaria (THH) es una displasia vascular multisistémica, de herencia autosómica dominante, caracterizada por el desarrollo de telangiectasias mucocutáneas y malformaciones arteriovenosas viscerales. El diagnóstico se realiza mediante los criterios de Curasao publicados en el año 2000 y su manejo requiere de un equipo multidisciplinario donde el rol del otorrinolaringólogo(a) es fundamental, puesto que la epistaxis se presenta en un 90%-95% de los pacientes siendo una de las primeras manifestaciones clínicas de la enfermedad y pudiendo amenazar la vida del paciente. En la literatura se describen múltiples alternativas de tratamiento médico y quirúrgico para la epistaxis, sin existir un tratamiento definitivo para la enfermedad. A continuación, presentaremos el caso de una paciente de 56 años con THH y epistaxis recurrentes severas que, tras no responder al tratamiento médico conservador y múltiples procedimientos quirúrgicos, se realizó el cierre nasal mediante el procedimiento de Young, constituyendo el primer caso reportado en nuestro país.

Hereditary hemorrhagic telangiectasia (HHT) is a multisystemic vascular dysplasia, of autosomal dominant inheritance, characterized by the development of mucocutaneous telangiectasias and visceral arteriovenous malformations. The diagnosis is made using the Curafao criteria published in 2000 and its management requires a multidisciplinary team where the role of the ENTs is fundamental, since epistaxis occurs in 90%-95% of patients, being one of the first clinical manifestations of the disease and may threaten the life of the patient. Multiple medical and surgical treatment alternatives for epistaxis are described in the literature without a definitive treatment for the disease. Here, we present the case of a 56-year-old patient with severe recurrent HHT and epistaxis who, after not responding to the initial conservative and surgical treatments, a nasal closure was performed, using the Young's procedure, constituting the first case reported in our country.

Angiofibroma nasofaríngeo juvenil: estudio de 6 casos en el Hospital Carlos van Buren de Chile / Juvenile nasopharyngeal angiofibroma: study of 6 cases in the Carlos van Buren Hospital in Chile

Introducción: El angiofibroma nasofaríngeo juvenil es una neoplasia vascular benigna y localmente agresiva, que se desarrolla casi exclusivamente en adolescentes de sexo masculino. Sus manifestaciones clínicas habituales son epistaxis y obstrucción nasal. Objetivo: Dar a conocer la experiencia en el Servicio de Otorrinolaringología del Hospital Carlos van Buren de pacientes con angiofibroma operados por vía endoscópica y abierta entre los años 2008 y 2015. Material y método: Estudio descriptivo retrospectivo de pacientes con diagnóstico de angiofibroma nasofaríngeo juvenil que ingresaron al Servicio de Otorrinolaringología del Hospital Carlos van Buren entre los años 2008 y 2015. Resultados: Hubo un total de 6 casos. La edad de los pacientes fluctuó entre los 12 y los 29 años, el 100% fueron pacientes masculinos. Los síntomas de presentación más frecuente fueron epistaxis recurrente y obstrucción nasal, presentes en 5/6 de los pacientes. La totalidad de los casos fueron estudiados con TC, RM y angiografía. El manejo en todos los casos fue con embolización endovascular 48 horas previo a la resección. Conclusión: Los resultados obtenidos se correlacionan con la literatura. El abordaje endoscópico sigue siendo de elección. Este tiene como ventajas menores pérdidas sanguíneas intraoperatorias, una disminución del número días de hospitalización y las tasas de recurrencia.

Introduction: Juvenile nasopharyngeal angiofibroma is a benign vascular neoplasm, locally aggressive that develops almost exclusively in adolescent males. Its usual clinical manifestations are epistaxis and nasal obstruction. Aim: To show the experience in the Department of Otolaryngology Hospital Carlos van Buren of angiofibromas operated by endoscopic and open surgery between the years 2008 and 2015, and review of the literature. Material and Method: Retrospective descriptive study of patients diagnosed with juvenile nasopharyngeal angiofibroma admitted in the Department of Otolaryngology Hospital Carlos van Buren, Valparaiso between 2008 and 2015. Results: A total of 6 cases were identified. The age of patients ranged from 12 to 29 years. The most common presenting symptoms were recurrent epistaxis and nasal obstruction, both present in 5/6 of patients. All the cases were studied with CT, MRI and angiography. All cases had pre-surgical endovascular embolisation48 hours prior to excision. Conclusions: The results correlate with those seen in the literature. The endoscopic approach is the better option, because of its lower intraoperative blood loss, days of hospitalization and recurrence.

Recurrent epistaxis from a post-traumatic infraorbital artery pseudoaneurysm

@#<p style="text-align: justify;"><strong>OBJECTIVE:</strong> To present a rare case of post-traumatic recurrent epistaxis in an elderly woman.<br /><strong>METHODS:</strong><br />  <strong>Design:</strong> Case Report<br /> <strong> Setting:</strong> Tertiary Private Hospital<br /><strong>  Patient:</strong> One<br /><strong>RESULT:</strong> A  93-year-old  woman  had  multiple  admissions  for  recurrent  life  threatening  nose-bleeding  that  was  not  controlled  until  a  post-traumatic pseudoaneurysm  of  the  infraorbital artery was diagnosed and embolized.<br />CONCLUSION: The diagnosis of pseudoaneurysm should be considered in such cases, and treatment involving surgeons and interventional radiologists should be initiated to minimize morbidity and mortality.</p>

Telangiectasia hemorrágica hereditaria: aspectos otorrinolaringologías / Hereditary hemorrhagic telangiectasia: ENT aspects

La telangiectasia hemorrágica hereditaria es una displasia vascular multisistémica caracterizada por el desarrollo de telangiectasias mucocutáneas y malformaciones arteriovenosas viscerales. Una de sus manifestaciones clínicas más frecuentes es la epistaxis recurrente, que se presenta en más del 90% de los pacientes, por lo que el otorrinolarin-gólogo debiera estar familiarizado con el diagnóstico y manejo de esta patología. Debido al carácter genético de la enfermedad, el manejo del sangrado nasal en estos pacientes es difícil. Existen diversas alternativas terapéuticas, farmacológicas y quirúrgicas, descritas para disminuir el número y gravedad de los episodios de epistaxis.

Hereditary hemorrhagic telangiectasia is a multisystemic vascular dysplasia, characterized by the development of mucocutaneoustelangiectasias and visceral arteriovenous malformations. One of its most frequent clinical manifestations is recurrent epistaxis, presenting in up to 90%% of these patients, so the otorhinolaryngologist should be familiarized with its diagnosis and management. Due to the genetic character of this disease, the management of nasal bleeding in these patients is difficult. There are several therapeutic alternatives, both pharmacological and surgical, described to decrease the number and severity of the episodes of epistaxis.

Recurrent and Massive Life Threatening Epistaxis due to Nasal Heroin Usage

Epistaxis, active bleeding from the nose, is a common ear nose and throat emergency, and can be severe or even fatal. We report a severe life threatening recurrent massive nasal bleeding caused by intranasal heroin use that has not hitherto been reported in the English literature. A 24-year-old male who took heroin several times nasally presented with massive nasal bleeding. A blood transfusion and an operation to halt nasal bleeding were required. The patient did not experience a bleeding attack 2 months following cessation of nasal heroin use.

Urinary vitamin C loading test and therapeutic effect of vitamin C in children with idiopathic recurrent epistaxis

Recurrent epistaxis is not one of rare symptoms in children, the well-known causes of which are anatomical abnormalities of nasal cavity and systemic bleeding tendency. but, in the majority of cases of recurrent epistaxis, it is usually very difficult to find out their underlying causes, so that the treatment is only symptomatic control of nasal bleeding whenever epistaxis occurs, but it usually is impossible to manage against their underlying causes as a specific therapy. The authors considered the breakdown or weakness of vascular integrity of nasal capillary vessel wall as an important factor of recurrent epistaxis, and vitamin C, which has an important role for the synthesis of collagen fiber, could influence to the vascular integrity of nasal capillary vessels. To elucidate the relation between recurrent epistaxis and the status of vitamin C in the tissue of the patients, the authors performed urinary vitamin C loading test measuring urinary excretion of vitamin C with high-performance liquid chromatography by Sirota et al in 19 cases of control group and 32 cases of patients with recurrent epistaxis, whose underlying diseases were not identified. And also the authors administered 1.0 gm/day of vitamin C orally for the treatment of recurrent epistaxis and follow-up was done over 1 year from the beginning of treatment to evaluate the treatment response. The results were as follows: 1) The hemoglobin level was significantly decreased in patient group compared with that of control group (12.4+/-0.8 vs 9.0+/-3.2 gm/dl). 2) The results of urine loading test of vitamin C, expressed as the percent excreted ratio, revealed significantly decreased in the patient group compared to that of the control group (9.1+/-6.1% vs 13.6+/-7.9%). 3) After treatment with 1.0 gm/day of vitamin C orally, follow-up was possible in 23 cases of total 32 patients. Of the 23 patients, "excellent" therapeutic response were in 16 cases (69.6%), "good" response in 5 cases (21.7%), and "no response" only in 2 cases (8.7%). The overall treatment response were in 21 cases (91.3%). In conclusion, it seems that majority of the patients with idiopathic recurrent epistaxis in the deficient state tissue vitamin C so that administration of vitamin C will be one of the effective therapy.