Hamartoma fibrovascular facial grave en paciente con esclerosis tuberosa (ET). Resolución de caso problema. Nuestra experiencia / Severe facial fibrovascular hamartoma in a patient with tuberous sclerosis (TS). Problem case resolution. Our experience

La ET es un trastorno multisistémico autosómico dominante que se caracteriza por displasia celular y tisular en varios órganos (cerebro, corazón, piel, ojos, riñones, pulmones) que constituyen una fuente importante de morbilidad y mortalidad. Las manifestaciones comunes incluyen tubérculos corticales, nódulos subependimarios, astrocitomas subependimarios de células gigantes, convulsiones, rabdomiomas cardíacos, AML renales, hamartomas retinianos, linfangioleiomiomatosis pulmonar, angiofibromas faciales, manchas de hojas de ceniza, parches de Shagreen, discapacidad intelectual y trastorno del espectro autista. Se presenta a continuación la resolución de un caso problema grave de una paciente que llega a la consulta al hospital público, con severa incapacidad para mantener la permeabilidad de la válvula nasal externa, a expensas de formación harmartomatosa grave, de años de evolución, fétida y sangrante, decidiéndose tomar conducta quirúrgica urgente y agresiva dada las condiciones de la lesión, la poca colaboración de la paciente y el contexto familiar de la misma que presenta además trastornos conductuales asociados a manifestaciones neurológicas de la enfermedad (retraso madurativo)

ET is an autosomal dominant multisystem disorder characterized by cellular and tissue dysplasia in several organs (brain, heart, skin, eyes, kidneys, lungs) that constitute a major source of morbidity and mortality. Common manifestations include cortical tubercles, subependymal nodules, subependymal giant cell astrocytomas, seizures, cardiac rhabdomyomas, renal AML, retinal hamartomas, pulmonary lymphangioleiomyomatosis, facial angiofibromas, ash leaf spots, shagreen patches, intellectual disability, and autism spectrum disorder. This paper presents the resolution of a serious problem case of a patient who attends the consultation of a public hospital, with severe inability to maintain the patency of the external nasal valve, at the expense of severe harmartomatous formation, of many years of evolution, fetid and bleeding, deciding to undertake urgent and aggressive surgical conduct given the conditions of the lesion, the lack of collaboration of the patient and the family context of the same, which also presents behavioral disorders associated with neurological manifestations of the disease (maturational delay).

Extranasopharyngeal Angiofibroma Originating in the Inferior Turbinate: A Distinct Clinical Entity at an Unusual Site

Introduction: The extranasopharyngeal angiofibroma is histologically similar to juvenile nasopharyngeal angiofibroma, differing from the latterin clinical and epidemiologic characteristics. Objectives We present a case of extranasopharyngeal angiofibroma originating in the inferior turbinate. Resumed Report The patient was a girl, 8 years and 6 months of age, who had constant bilateral nasal obstruction and recurrent epistaxis for 6 months, worse on the right side, with hyposmia and snoring. Nasal endoscopy showed a reddish lesion, smooth, friable, and nonulcerated. Computed tomography showed a lesion with soft tissue density in the right nasal cavity. We used an endoscopic approach and found the lesion inserted in the right inferior turbinate. We did a subperiosteal dissection and excision with a partial turbinectomy with a resection margin of 0.5 cm. Histopathology reported it to be an extranasopharyngeal angiofibroma. Conclusion Although rare, extranasopharyngeal angiofibroma should be considered in the diagnosis of vascular tumors of the head and neck...

Multiple glass pieces in paranasal sinuses.

Here, a case has been reported of a road traffic accident with multiple glass pieces arranged in an unusual pattern in the left maxillary sinus, ethmoid sinus, nasopharynx and medial side of the orbit, as seen in the radiographs. Combined surgical approach through the existing wound and endoscopic surgery was successfully used to remove nearly all the glass pieces.

Angiofibroma juvenil: a propósito de un caso / Juvenile angiofibroma: a purpose of a case

El angiofibroma Juvenil es descrito como una rara y benigna enfermedad, que es principalmente diagnosticado en adolescentes masculinos. Es altamente vascularizado y un tumor de crecimiento lento, pero localmente invasivo y destructivo. Originándose en el margen del agujero esfenopalatino, este se extiende hacia la fosa pterigopalatina, senos paranasales y cavidad nasal, ocasionando síntomas como el dolor, obstrucción nasal unilateral y epistaxis. El objetivo principal es reportar un caso de un masculino de 14 años de edad con un angiofibroma. El presentó una evolución clínica de 4 meses con aumento de volumen de la media cara derecha, movimiento tónico clónico generalizado, anosmia y sangrado nasal. El fue operado realizando exéresis tumoral por una incisión transfrontal extendida y procedimientos de base de cráneo.

Lesão penetrante do crânio pela via transnasal: relato de caso / Transnasal penetrating brain injury: case report

Lesões penetrantes no crânio são incomuns pela via transnasal e geralmente ocorrem como resultado de violência,acidentes de trabalho,de trânsito,ou por um evento casual.Este relato trata de introdução acidental de barra de metal, por uma das narinas, atravessando os planos da base do crânio e se alojando no cérebro de uma criança do sexo masculino de 4 anos de idade.Esta apresentava cefaléia,epistaxe e alteração visual.A radiografia e a tomografia computadorizada demonstraram o corpo estranho atingindo a região da sela túrcica à esquerda.Na arteriografia digital cerebral observou-se que a barra atingia a artéria carótida, em sua bifurcação e região de artéria cerebral média. Foi feita craniotomia pterional esquerda para retirada da barra com visualização direta. Encontrou-se lesão superficial da artéria carótida e da artéria oftálmica.Realizou-se contenção do sangramento.O paciente evoluiu no pós-operatório imediato com edema cerebral frontal unilateral,hipertensão intracraniana e fístula liquórica.Com um ano de evolução apresentava-se apenas com déficit visual.Esse caso chama a atenção por sua raridade e por demonstrar a importância da craniotomia e da microcirurgia no controle da hemorragia.

Epistaxis masiva asociada a trauma: una indicación de angiografía y embolización superselectiva: caso clínico / Massive epistaxis associated to trauma, treated with angiography and selective embolization: clinical case

We report a 23 years old male admitted to the hospital after a fall from a great altitude with severe trauma. During the second week of hospitalization he presented a severe nosebleed that did not respond to conventional tamponade. A selective angiography was performed and the branches of the internal maxillary artery were embolized in two occasions, stopping the bleeding. The patient was discharged from the hospital after 71 days of hospitalization

Lupus tumidus involving facial skin nasal cavity throat and eye

A case of tuberculosis involving facial skin, nasal cavity throat and eyes was found in a yong korean girl. We report the case because of its unusual clinical picture and to recall the morbidity of cutaneos tuberculosis in the present dermatological field.Skin tuberculosis is one of the oldest diseases in dermatology. But the invasion of the skin by tubercle bacilli still seen and all the types of so called reinfection tuberculosis of the skin are being found sporadically all over the world.Generally patients with skin tuberculosis adapt themselves to their disorder during the long course of the disease actually, mostof the tuberculous skin lesions do not cause great troubles to the patient allowing him or her to lead a normal life. Recently we found a case which was quite unusual and seriosly treatened the patients normal activity because of naasal obstruction and facial disfiguration.