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Congenital anomalies of the larynx

Glenn C Isaacson, MD, FAAP
Section Editor
Anna H Messner, MD
Deputy Editor
Carrie Armsby, MD, MPH


Congenital anomalies are the product of errors in embryogenesis (malformations) or the result of intrauterine events that affect embryonic and fetal growth (deformations and disruptions) [1]. The more complex the formation of a structure, the more opportunities for malformation.

Defects in the formation and growth of the larynx lead to a variety of malformations. The embryology, clinical features, and management of congenital anomalies of the larynx are reviewed here. Congenital anomalies of the intrathoracic airways are discussed separately. (See "Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula".)


The formation of a median pharyngeal groove presages the appearance of the respiratory tract. At approximately 25 days of intrauterine life, the anlagen of the larynx, trachea, bronchi, and lungs arise from a ventromedial diverticulum of the foregut called the tracheobronchial groove. The cartilage of the trachea and connective tissue and muscle of the trachea and esophagus are derived from splanchnic mesenchyme. Lateral furrows develop on each side of the ventromedial diverticulum, deepen, and join to form the tracheoesophageal septum [2-4].

The distal esophagus can be distinguished from the stomach and the laryngeal primordia appear at approximately 33 days of intrauterine life. The T-shaped laryngeal slit (aditus) is formed anteriorly by the growth of the primordium of the epiglottis (from the hypobranchial eminence, arches III and IV) and laterally by the precursors of the arytenoid cartilages (ventral ends of arch VI) [2-4].

During the fifth and sixth weeks, the tracheoesophageal septum extends to the first tracheal ring. By the time the embryo is 13 to 17 mm in length, laryngeal cartilage and muscle development are clearly identifiable, and lateral cricoid condensation is underway. By the seventh week of development, the cricoid ring is complete, and the cartilaginous hyoid is visible below the epiglottis (picture 1). Definitive tracheal cartilage appears at this stage and the esophagus has four discrete layers. The larynx, trachea, and esophagus are well formed by the end of the embryologic period (image 1 and picture 2) [2-4].

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Literature review current through: Nov 2017. | This topic last updated: Jan 09, 2017.
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