Congenital Atresia of Larynx

At a glance

A very rare developmental abnormality of the larynx.

Incidence

The exact incidence remains to be determined. Approximately 50 cases with no sexual predilection have been reported.

Genetic inheritance

Most likely autosomal dominant with the responsible gene located on chromosome 5.

Diagnosis

Based on clinical findings, but typically found during ultrasound examination in pregnancy.

Clinical aspects

Laryngeal atresia is defined as a complete absence of the laryngeal lumen and results from an arrest in the embryonic development of the larynx at different stages. Depending on the localization of the atresia, three types of laryngeal atresia can be distinguished: atresia is both supraglottic and infraglottic in Type I, infraglottic in Type II, and glottic in Type III. Partial laryngeal atresia may result from congenital webs, which may be localized between the cords, or due to supraglottic and/or infraglottic stenoses leading to a laryngeal lumen with reduced size at birth. Clinical signs of near complete or complete laryngeal atresia (with tracheoesophageal fistula) at birth may include stridor, laryngeal obstruction, or absent cry. Partial atresia of the larynx may be revealed by direct laryngoscopy. Complete atresia requires tracheostomy within minutes after birth. A tracheoesophageal fistula, often associated with laryngeal atresia, may allow ventilation of the lungs through the fistula. Esophageal intubation with ventilation through the fistula until tracheotomy was performed has been described. A common finding in complete laryngeal atresia (but not in partial atresia) is pulmonary hyperplasia with dilatation of the tracheobronchial tree. Approximately half of the patients described in the literature also suffer from other, potentially life-threatening, anomalies. These anomalies may involve the central nervous system (hydrocephalus), the heart (single ventricle, cardiac failure secondary to venous obstruction), the respiratory and gastrointestinal tracts (tracheal atresia, tracheoesophageal fistula, esophageal atresia, duodenal atresia), the urogenital tract (aplastic/hypoplastic kidneys, hydroureter, urethral atresia, hypospadias), and the musculoskeletal system (craniofacial anomalies, cervical vertebral anomalies, pes varus). Complete atresia requires immediate therapy (tracheostomy/tracheotomy) after birth, thus most children born with this condition will die unless they are diagnosed prenatally (minority of cases), which is possible only in complete, but not in partial atresia (polyhydramnios, pulmonary hyperplasia, hydrops fetalis, ascites). However, survival also depends on the presence of other concomitant anomalies. Although survival in general is rare, the lifespan of a few patients with immediate and successful therapy of isolated laryngeal atresia reportedly is normal.

Precautions before anesthesia

If known antenatally, an EXIT procedure would be the safest way to treat these patients. In unanticipated cases, there is most likely no time for anesthetic evaluations.

Anesthetic considerations

These patients present with respiratory distress immediately after birth. As already mentioned, immediate action in the form of a tracheo(s)tomy is required for complete ...