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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Laryngeal cleft


Other Names for this Disease
  • Anterior submucous laryngeal cleft (subtype)
  • Cleft, larynx posterior
  • Congenital laryngeal clefts (subtype)
  • Posterior laryngeal cleft (PLC)
  • Type 1A (minor laryngeal cleft)
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Your Question

My son was diagnosed with a laryngeal cleft 4 months ago and I can't find much information about this condition. Everything I read is from a medical journal. I'm just looking for more information about laryngeal cleft.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is laryngeal cleft?

laryngeal cleft is a rare abnormality of the separation between the larynx, or voice box, and the esophagus.[1][2] Normally, when the larynx develops, it is completely separate from the esophagus so swallowed foods go directly into the stomach. When a laryngeal cleft occurs, there is an opening between the larynx and the esophagus so food and liquid can pass through the larynx into the lungs.[1] There are several different types of laryngeal clefts (Types I through IV), classified based on the extent of the clefting.[3][4]
Last updated: 9/1/2011

Is laryngeal cleft associated with any other abnormalities?

In about 50% of cases, laryngeal clefts are found in association with other abnormalities. These may include tracheobronchomalacia, heart and blood vessel anomalies (pulmonary valvular stenosis, aberrant innominate artery, patent ductus arteriosus, aortic valvular stenosis, ventricular septal defect), pulmonary agenesis, bronchoesophageal fistula, tracheoesophageal fistula.  Some females have an underdeveloped uterus.  Other affected individuals have had blindness since birth.[3][4]  Laryngeal clefts may also occur as part of a genetic syndrome, such as Opitz GBBB syndrome or Pallister-Hall syndrome.[5]
Last updated: 9/2/2011

What is the incidence of laryngeal cleft?

The incidence of laryngeal clefts is approximately 1 in 10,000 to 20,000 live births.[2][4]
Last updated: 9/2/2011

What are the signs and symptoms of laryngeal cleft?

The symptoms of laryngeal clefts range from mild stridor to significant difficulties with breathing and swallowing.  Severity of symptoms depends on the severity of the cleft.[4]  Swallowing problems, a husky cry and feeding difficulties are common.[1]  Feeding often causes stridor, coughing, choking, gagging, cyanosis, regurgitation, and frequent respiratory infections.[1][3][4]  Many individuals with laryngeal clefts develop chronic lung disease.[3]
Last updated: 9/2/2011

What causes laryngeal cleft?

During fetal development, the trachea and esophagus begin as one tube.  They later separate when a wall of tissue known as the tracheoesophageal septum forms, dividing the original tube into the trachea and esophagus.  If the tracheoesophageal septum fails to form, the trachea and esophagus may remain open to each other or abnormally shaped, causing abnormalities such as a laryngeal cleft, tracheoesophageal fistula, or esophageal atresia.[2][4]  Exactly why these abnormalities occur is unknown.
Last updated: 9/2/2011

How might laryngeal cleft be treated?

Medical and feeding therapies are often the first treatments for patients with laryngeal cleft (particularly type I and type II).[4126]  Prevention of gastroesophageal reflux is also important in all types of clefts. Type I clefts often correct themselves over time with growth.  During infancy, nursing in the upright position or thickening of formula may be necessary.  If these treatments are not enough, surgery may be recommended.[3] Different surgical approaches have been proposed for the management of laryngeal cleft. The timing and approach of surgery may differ depending upon the severity of symptoms, associated abnormalities, and type of cleft.[4]
Last updated: 9/2/2011

References