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A very rare developmental abnormality of the larynx.

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The exact incidence remains to be determined. Approximately 50 cases with no sexual predilection have been reported. Inheritance is most likely autosomal dominant with the responsible gene located on chromosome 5.

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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 from supraglottic and/or infraglottic stenoses leading to a laryngeal lumen with reduced size at birth. Clinical signs of complete atresia at birth may include stridor, laryngeal obstruction or absent cry, and require tracheostomy within minutes after birth. Partial atresia of the larynx may be revealed by direct laryngoscopy. 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 anomalies, some of which are potentially life-threatening. These anomalies may affect 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). Because complete atresia requires immediate therapy (tracheostomy/tracheotomy) after birth, most children born with this condition will die unless the condition is 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 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.

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These patients present immediately after birth. As mentioned earlier, immediate action in the form of a tracheo(s)tomy is required for complete laryngeal atresia. Preserve spontaneous ventilation in partial atresia until the airway has been secured. Anesthesia for this procedure must mainly rely on local anesthesia. Ex utero intrapartum treatment (so-called EXIT procedure) has been used successfully in a fetus at 35 weeks of gestation just prior to delivery via cesarean section.

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Fraser Syndrome: A polymalformative condition characterized by the association of cryptophthalmos with a wide range of abnormalities (orofacial defects, syndactyly, decreased number of digits, urogenital and renal malformations), easily recognized at birth by the evident absence of eyelids.

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Cat Cry Syndrome: A syndrome clinically characterized by microcephaly, round face, macrostomia and micrognathia, scoliosis, muscular hypotonia, severe mental retardation and often congenital heart defects. One of the most important signs in the newborn is a high-pitched cat-like cry that ...

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