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Key Points

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  1. Most type 1 and type II clefts can be repaired endoscopically.

  2. When open cleft repair is required, a transtracheal approach with a layered closure is recommended.

  3. When there is a higher risk of repair breakdown, an interposition graft is recommended.

  4. Before embarking on cleft repair, consideration should be given to: (a) placement of a gastrostomy tube; (b) fundoplication; and (c) performing a tracheotomy.

  5. Type IV clefts that involve the carina require a highly individualized approach.

  6. Even in the presence of optimal intervention, morbidity and mortality rates remain high.

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History

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Posterior laryngeal cleft is a rare congenital anomaly resulting from embryologic failure of the laryngotracheal groove to fuse. This condition was first described by Richter in 1792 in reference to a baby with multiple congenital anomalies. No further reports occurred until 1949, when Finlay reported a posterior laryngeal cleft progressing to the level of the midcricoid found on autopsy in a child with longstanding stridor. In 1955, Petterson performed the first successful repair of a posterior laryngeal cleft using a lateral pharyngotomy approach. However, it was not until 1982 that Donahoe performed the first successful repair of a posterior laryngeal cleft extending to the carina. At that time, the survival rate of children with posterior laryngeal clefts of any severity was less than 60%. Over the past 3 decades, transtracheal and translaryngeal repair of longer laryngeal clefts has replaced the laryngeal pharyngotomy approach, while over the past decade, endoscopic repair of shorter clefts has been enthusiastically adopted. Although overall survival rates have steadily increased, the mortality rate of children with long type IV clefts still exceeds 90%.

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Embryology

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The laryngotracheal groove appears at day 25 of normal embryonic development, with lateral indentations fusing to form a tracheoesophageal septum. This fusion commences distally and progresses proximally, reaching the first tracheal ring by day 34. The 2 lateral chondrification centers of the cricoid fuse ventrally at 40 to 42 days and dorsally at 46 to 48 days. Disruption of normal development often results in a variety of anomalies, including those associated with the spectrum of tracheoesophageal fistulae and esophageal atresia. Approximately 20% of patients with posterior laryngeal clefts also have a concurrent tracheoesophageal fistula (TEF).

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Classification System

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The most useful classification system is that proposed by Benjamin and Inglis. It is an anatomic classification that divides posterior laryngeal clefts into the following 4 subtypes:

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  • Type I: A supraglottic interarytenoid cleft present to, but not below, the level of the true vocal cords. This cleft could be considered a deep interarytenoid notch.

  • Type II: A partial cricoid cleft that extends into, but not through, the posterior cricoid cartilage.

  • Type III*: A total cricoid cleft with or without extension of the cleft into the cervical trachea.

  • Type IV*: A cleft extending to the thoracic trachea.

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