CHAOS: síndrome de obstrucción congénita de las vías aéreas superiores / CHAOS: congenital high airway obstruction syndrome

Resumen El síndrome de obstrucción congénita de vías áreas superiores (CHAOS) es una condición que se caracteriza por la existencia de una obstrucción en las vías áreas altas en el feto, la cual puede ser parcial o completa. Comúnmente es una situación incompatible con la vida, por lo que su diagnóstico prenatal es importante considerando el pronóstico y los diferentes manejos prenatales y posnatales que existen. Presentamos un caso de CHAOS diagnosticado en la semana 21, con una breve revisión de la literatura sobre su diagnóstico, pronóstico y alternativas terapéuticas.

Abstract Congenital high airway obstruction syndrome (CHAOS) is a condition characterized by the existence of an obstruction of the fetal upper airways, which may be partial or complete. It is commonly incompatible with life, so its prenatal diagnosis is important due to the prognosis and the recently described pre and postnatal management options. We present a case of CHAOS in a pregnancy of 21 weeks with a brief review of the current literature about its diagnosis, prognosis and therapeutic alternatives.

Primer caso de tratamiento ex utero intraparto realizado por vía vaginal en un feto con síndrome de obstrucción congénita de la vía aérea superior / The first case of exutero intrapartum treatment performed using the vaginal route in a fetus with congenital high airway obstruction syndrome

El síndrome de obstrucción congénita de la vía aérea superior (CHAOS), es una condición infrecuente que causa asfixia o muerte perinatal inmediata, de no mediar una estrategia terapéutica que permita permeabilizar la vía aérea del paciente durante el nacimiento. El diagnóstico prenatal, es fundamental para delinear estrategias de tratamiento perinatal con el fin de minimizar la morbimortalidad de niños con anomalías congénitas. El tratamiento ex-útero intraparto (EXIT) es el procedimiento de elección. Clásicamente se realiza mediante una cesárea programada, manteniendo el soporte fetal a través de la circulación útero-placentaria. Se requiere un equipo altamente calificado y un trabajo coordinado para concretar el procedimiento en estas condiciones. Objetivo: El objetivo es reportar un caso de Síndrome de CHAOS, en el que se realizó un procedimiento EXIT en un niño nacido por parto vaginal, con la participación de un equipo multidisciplinario de profesionales de dos Instituciones Públicas de la Ciudad de Buenos Aires, en el marco de un Programa Conjunto de Diagnóstico y Tratamiento Fetal (AU)

Congenital high airway obstruction syndrome (CHAOS) is a rare entity causing perinatal asphyxia or immediate death if no therapeutic strategy is undertaken to correct airway patency at birth. Prenatal diagnosis is essential to plan perinatal strategies to decrease morbidity and mortality in children with congenital anomalies. The exutero intrapartum treatment (EXIT) is the procedure of choice. Classically, a programmed cesarean section is performed while the fetus is maintained on uteroplacental circulation. A highly trained team is required in the coordinated effort to perform the procedure. Aim: The aim of this study was to report on a case of CHAOS managed with an EXIT procedure in a child born through vaginal delivery performed by a multidisciplinary team of professionals belonging to two public institutions of the city of Buenos Aires in the framework of the Joint Program of Fetal Diagnosis and Treatment (AU)

A Case of Congenital Laryngeal Atresia with Diaphragmatic Hernia / 대한주산의학회잡지

Congenital laryngeal atresia is a rare cause of airway obstruction that is almost always lethal within short period of time after birth unless diagnosed prenatally and emergency tracheostomy was performed. Other life-threatening anomalies such as tracheoesophageal fistula, gastrointestinal or urinary anomalies, and VATER syndrome are often associated with laryngeal atresia. Recently, we experienced a case of congenital laryngeal atresia with diaphragmatic hernia, ear and skull anomalies, not diagnosed prenatally, died of asphyxia due to intubation failure, and confirmed by autopsy. We report this case with a brief review of the literatures.

A Case of Congenital Laryngeal Atresia with Single Umbilical Artery Who Required a Tracheotomy / 소아과

Congenital laryngeal atresia is a rare cause of airway obstruction that is almost always lethal within a few minutes after birth. Therefore congenital laryngeal atresia should be diagnosed in the prenatal period. If not, it should be considered in newborn infant with life-threatening symptoms at birth such as cyanosis and dyspnea that need emergent procedures like a tracheotomy. We report a case of congenital laryngeal atresia with single umbilical artery diagnosed in a one-day-old neonate who required a tracheotomy and ventilator care after intubation failure.

Laryngeal Atresia with Tracheoesophageal Fistula: 1 case report

Laryngeal atresia is a very rare congenital anomaly requiring immediate tracheotomy. We present a case of laryngeal atresia with tracheoesophageal fistula who showed immediate respiratory difficulty after ligation of umbilical cord and died of aspiration pneumonia at 8 days of age. The atretic portion of larynx is composed of irregulary arranged cartilaginous tissue, bundles of intrinsic muscle and soft tissue without epithelium-lined lumen. The lungs show normal development and evidences of aspiration pneumonia.