Estenosis congénita de la apertura piriforme: forma inusual de obstrucción nasal / Congenital nasal pyriform aperture stenosis: unusual form of nasal airway obstruction

La estenosis congénita de la apertura piriforme con megaincisivo central (ECAP) es una causa poco frecuente de obstrucción de la vía aérea en neonatos. Dada su baja frecuencia y por la similitud de la clínica se confunde inicialmente con la atresia coanal, trastorno más prevalente (1:5.000 nacidos vivos). Puede tratarse de una forma menor de holoprosencefalia, por lo que el diagnóstico incluye el screening de malformaciones cardíacas y cerebrales asociadas. Presentamos un caso de una recién nacida con ECAP (AU)

Congenital nasal pyriform aperture stenosis (CNPAE) with a single mega-incisor is an uncommon form of nasal airway obstruction in the neonate. Its low frequency and its similar clinical presentation may lead us to a wrong initial diagnosis of choanal atresia, which is more frequent (1:5000 live births). CNPAE may be a minor form of holoprosencephaly, so diagnosis should include screening for heart and central nervous system abnormalities. A case of a newborn with CNPAE is reported (AU)

[Inverted acoustic reflex: an analysis of its morphological characteristics in different physiological and pathological situations]. / Reflejo acústico invertido: análisis de sus características morfológicas en distintas situaciones fisiológicas y patológicas.

INTRODUCTION AND OBJECTIVES: Inverted acoustic reflex (IAR) or inverted acoustic effect is the name given to the positive deflexion observed during the carrying out of an admittancemetry when the ear is subjected to a high intensity sound, rather than the typical negative deflexion due to the contraction of the middle ear musculature. The objective of the paper is to analyze the morphology of the IAR by relating it to the situations (physiological or pathological) in which this effect appears with the aim of establishing a pathogenic hypothesis. METHODS: We study the admittance traces obtained in 50 patients on stimulating the ear with high sound intensities (between 100 and 110 dBs HL) in different situations (ageing, death, otosclerosis, facial palsy, perception hypoacusia and normoacusia). We analyse the morphologies of the resulting traces as well as the on-set and off-set latencies of the reflexes. RESULTS: The morphology and latency parameters are similar in all cases in which IAR appears, regardless of the physical conditions of the ear but always with the prior condition of the absence of any contraction of the stapes bone muscle. The inverted acoustic effect does not disappear after deep muscular relaxation induced by anaesthesia and is present in corpses at a time when neural activity can no longer take place. CONCLUSIONS: Although some of the characteristics of IAR cannot be explained by purely mechanical hypotheses, the appearance of this effect in a corpse and its non-disappearance with muscular relaxation means that we consider its origin to be a purely mechanical effect not mediated by any muscular reflex contraction in the human middle ear.

Reflejo acústico invertido: análisis de sus características morfológicas en distintas situaciones fisiológicas y patológicas / Inverted acoustic reflex: An analysis of its morphological characteristics in different physiological and pathological situations

Introducción y objetivos: Se denomina reflejo o efecto acústico invertido (RAI) a la deflexión positiva observada durante la realización de una admitanciometría al someter al oído a un sonido de intensidad elevada, en vez de la típica deflexión negativa debida a la contracción de la musculatura del oído medio. El objetivo del trabajo es analizar la morfología del RAI relacionándola con las situaciones (fisiológicas o patológicas), en las que este efecto aparece con el fin de establecer una hipótesis patogénica. Métodos: Estudiamos los trazados admitanciométricos obtenidos en 50 pacientes al estimular el oído con elevadas intensidades sonoras (entre 100 y 110dB HL) en distintas situaciones (envejecimiento, muerte, otosclerosis, parálisis facial, hipoacusia de percepción y normoacusia). Analizamos las morfologías de los trazados resultantes así como las latencias de aparición y de cese (on-set y off-set) de los reflejos. Resultados: La morfología y los parámetros de latencia son similares en todos los casos en los que el RAI aparece, independientemente de las condiciones físicas del oído, pero siempre con el condicionante previo de ausencia de contracción del músculo del estribo. El RAI no desaparece tras la relajación muscular profunda inducida por la anestesia y está presente en el cadáver cuando ya no puede aparecer actividad neural alguna. Conclusiones: Aunque algunas de las características del RAI no se pueden explicar sirviéndonos de forma exclusiva de hipótesis mecánicas, la aparición de ese efecto en el cadáver y la no desaparición con la relajación muscular hace que consideremos su origen como un efecto puramente mecánico no mediado por contracción refleja muscular alguna en el oído medio humano (AU)

Introduction and objectives: Inverted acoustic reflex (IAR) or inverted acoustic effect is the name given to the positive deflexion observed during the carrying out of an admittancemetry when the ear is subjected to a high intensity sound, rather than the typical negative deflexion due to the contraction of the middle ear musculature. The objective of the paper is to analyze the morphology of the IAR by relating it to the situations (physiological or pathological) in which this effect appears with the aim of establishing a pathogenic hypothesis. Methods: We study the admittance traces obtained in 50 patients on stimulating the ear with high sound intensities (between 100 and 110dBs HL) in different situations (ageing, death, otosclerosis, facial palsy, perception hypoacusia and normoacusia). We analyse the morphologies of the resulting traces as well as the on-set and off-set latencies of the reflexes. Results: The morphology and latency parameters are similar in all cases in which IAR appears, regardless of the physical conditions of the ear but always with the prior condition of the absence of any contraction of the stapes bone muscle. The inverted acoustic effect does not disappear after deep muscular relaxation induced by anaesthesia and is present in corpses at a time when neural activity can no longer take place. Conclusions: Although some of the characteristics of IAR cannot be explained by purely mechanical hypotheses, the appearance of this effect in a corpse and its non-disappearance with muscular relaxation means that we consider its origin to be a purely mechanical effect not mediated by any muscular reflex contraction in the human middle ear (AU)

[Total idiopathic laryngotracheal stenosis. Treatment with mitomycin C]. / Obstrucción laringotraqueal completa idiopática. Tratamiento con mitomicina C.

This case report involves a 69-year-old woman who presented idiopathic laryngotracheal stenosis with total obstruction of the airway. Seven surgical procedures involving open field and endoscopy techniques had to be performed due to repeated re-obstructions. The novelty in this case is that success was finally obtained with the use of mitomycin C. The drug action is well known, but is interesting to report a case in which success was only obtained when we used mitomycin C.

Obstrucción laringotraqueal completa idiopática. Tratamiento con mitomicina C / Total idiopathic laryngotracheal stenosis. Treatment with mitomycin C

Exponemos el caso de una paciente de 69 años en la que se desarrolló una estenosis laringotraqueal idiopática con obstrucción completa. Se tuvo que intervenir a la paciente 7 veces, con distintas técnicas endoscópicas o abiertas, por reobstrucciones postoperatorias. Lo novedoso del caso es que el éxito final se obtuvo tras la aplicación de mitomicina C. Es conocida la acción de este fármaco, pero tiene interés mostrar un caso complejo en el que sólo se obtiene éxito cuando se aplica mitomicina C

This case report involves a 69-year-old woman who presented idiopathic laryngotracheal stenosis with total obstruction of the airway. Seven surgical procedures involving open field and endoscopy techniques had to be performed due to repeated re-obstructions. The novelty in this case is that success was finally obtained with the use of mitomycin C. The drug action is well known, but is interesting to report a case in which success was only obtained when we used mitomycin C