Laryngeal cleft

Is laryngeal cleft associated with any other abnormalities?

In about 50% of cases, laryngeal clefts are found in association with other abnormalities. Examples include tracheobronchomalacia, cleft lip and/or palate, heart and blood vessel anomalies (such as pulmonary valvular stenosis, patent ductus arteriosus, aortic valvular stenosis, or ventricular septal defect), pulmonary agenesis, bronchoesophageal fistula, and tracheoesophageal fistula. Laryngeal clefts may also occur as part of an underlying syndrome or condition such as Opitz-Frias, VACTERL, Pallister-Hall, or CHARGE.

Last updated on 05-01-20

What is a laryngeal cleft?

A laryngeal cleft is a rare malformation involving the larynx (known as the voice box) and the esophagus (known as the food pipe). Normally, when the larynx develops, it is completely separate from the esophagus so that swallowed foods travel directly through the esophagus into the stomach. A laryngeal cleft occurs when these structures do not develop normally in an embryo, and there is an opening connecting the larynx (and sometimes trachea) and the esophagus. This enables food and liquid that is swallowed to pass through the opening to the larynx, and into the lungs. Signs and symptoms may appear in the first few months of life and may include feeding problems, trouble swallowing, failure to thrive, reflux, coughing, wheezing, stridor, aspiration, respiratory distress, and recurrent lung infections. There are several different types of laryngeal clefts (types I through IV), which are classified based on the specific location and extent (severity) of the cleft.

A cleft can occur as an isolated abnormality, as part of an underlying syndrome or condition (such as Opitz- Frias, VACTERL, Pallister-Hall, CHARGE), or with other associated malformations. Treatment depends on the location and severity of the cleft and symptoms. For example, small clefts can sometimes be managed with medications to control symptoms such as reflux or aspiration, while some smaller clefts and larger clefts may need to be treated with endoscopic procedures or surgery.

Last updated on 05-01-20

What is the incidence of laryngeal cleft?

The incidence of laryngeal clefts is approximately 1 in 10,000 to 20,000 live births.They are more common in boys than in girls, with a male-to-female ratio of 5:3.

Last updated on 05-01-20

How might a laryngeal cleft be treated?

The best treatment for a laryngeal cleft depends on the type of cleft and the severity of the symptoms it causes. In some cases, minor clefts (particularly types I or II) can be managed with medications that treat or prevent symptoms such as reflux or aspiration. Type I clefts may correct themselves as a person grows. A smaller-sized cleft that requires additional treatment may be treated with endoscopic repair. More severe clefts (particularly types III and IV) often require endocopic procedures or surgery.

Last updated on 05-01-20

Selected Full-Text Journal Articles

Laryngeal cleft full text article

Rahbar R, Rouillon I, Roger G, Lin A, Nuss RC, Denoyelle F, McGill TJ, Healy GB, Garabedian EN. The Presentation and Management of Laryngeal Cleft: A 10-Year Experience. Archives of Ololaryngology-Head & Neck Surgery. 2006; 132(12):1335-1341.

Last updated on 04-27-20

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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