Laryngeal cleft

A laryngeal cleft or laryngotracheoesophageal cleft is a rare congenital abnormality in the posterior laryngo-tracheal wall.[1] It occurs in approximately 1 in 10,000 to 20,000 births.[2] It means there is a gap between the oesophagus and trachea, which allows food or fluid to pass into the airway.[3]

Symptoms and diagnosis

Diagnosis of a laryngeal cleft in an infant often comes after problems with feeding which include coughing, cyanosis (blue skin) and failing to gain weight over time.[3] Pulmonary infections are also common.[4] The longer the cleft is, the more severe the symptoms. A cleft is diagnosed through endoscopic examination, specifically microlaryngoscopy and bronchoscopy.[4][5] Laryngeal clefts are classified into four types according to length. Type I extends no further down than the vocal cords, type II extends below the vocal cords and into the cricoid cartilage, type III extends into the cervical section of the trachea and type IV extends the furthest—into the thoracic section of the trachea.[5]

Treatment

Treatment of a laryngeal cleft depends on the length and resulting severity of symptoms. A shorter cleft (type I) may not require surgical intervention.[5] Symptoms can be managed by thickening the infant's feeds.[5] A slightly longer cleft (long type I or shorter type II) is repaired endoscopically. A cleft that is longer still (type II or type III) is repaired directly through the neck, with a tracheotomy.[5]

Associated conditions

20–27% of individuals with a laryngeal cleft also have a tracheoesophageal fistula and approximately 6% of individuals with the fistula also have the cleft.[4] Other congenital anomalies commonly associated with laryngeal cleft are gastro-oesophageal reflux, tracheobronchomalacia, congenital heart defect, dextrocardia and situs inversus.[5] Laryngeal cleft can be a component of other genetic syndromes, including Pallister-Hall syndrome and G syndrome (Opitz-Friaz syndrome).[4]

References

  1. ↑ Thornton, M.; H. Rowley; B. J. Conlon; J. D. Russell (2001). "Type I laryngeal cleft: late presentation". Journal of Laryngology & Otology. 115 (10): 821–822. doi:10.1258/0022215011909053. PMID 11667997.
  2. ↑ Pezzettigotta, S. M.; Leboulanger N.; Roger G.; Denoyelle F.; Garabédian E. N. (October 2008). "Laryngeal cleft". Otolaryngologic Clinics of North America. 41 (5): 913–33, ix. doi:10.1016/j.otc.2008.04.010. PMID 18775342.
  3. 1 2 Cherry, John R. (1997). Ear Nose & Throat for Lawyers. Routledge. p. 368. ISBN 1-85941-210-6.
  4. 1 2 3 4 Bluestone, Charles D. (2003). Pediatric otolaryngology, Volume 2. Elsevier Health Sciences. p. 1468. ISBN 0-7216-9197-8.
  5. 1 2 3 4 5 6 Graham, John M.; Glenis K. Scadding; Peter D. Bull (2008). Pediatric ENT. Springer. p. 193. ISBN 3-540-69930-9.
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