Bilateral Congenital Choanal Atresia in a Female Neonate: Importance of Bedside Diagnosis

Abstract

Congenital bilateral choanal atresia is a neonatal emergency in which there is narrowing or imperforation of the posterior nares. The obstruction is usually at the junction of the posterior nasal cavity and the anterior aspect of nasopharynx. The aetiology has been attributed to a persistence of the embryological bucconasal membrane. This membrane separates the nasal cavity from the stomatodeum until it breaks down at about the seventh week of intrauterine development, allowing communication through the primitive posterior nares. It has a prevalence rate of about 1 in 7000 – 10000 live birth 2 and affecting frequently the females about twice more than the males. It can be bilateral or unilateral, complete or incomplete and purely membranous(10%), purely bony(30%) and mixed bony and membranous(70%). Unilateral atresia is commoner than bilateral atresia in the proportion of 3:2 When it is unilateral, the right nostril is affected more than the left nostril at a ratio of 2: 1. Unlike the unilateral atresia which may remain undiagnosed until persistent unilateral rhinorrhoea and nasal obstruction attracts attention, bilateral atresia presents immediately after birth with respiratory distress especially during feeding and requiring immediate intervention. Congenital atresia is present in 10% to 30% of patients with “CHARGE” syndrome (coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and/or development, genital and/or urinary defects, ear anomalies). Other anomalies such as Crouzons, Di George, Amniontic band, Fetal alcohol and Treacher Collins Syndromes may be associated with choanal atresia.