A laryngeal cleft or laryngotracheoesophageal cleft is a rare congenital abnormality in the posterior laryngo

Larynx

The larynx , commonly called the voice box, is an organ in the neck of amphibians, reptiles and mammals involved in breathing, sound production, and protecting the trachea against food aspiration. It manipulates pitch and volume...

-trachea

Vertebrate trachea

In tetrapod anatomy the trachea, or windpipe, is a tube that connects the pharynx or larynx to the lungs, allowing the passage of air. It is lined with pseudostratified ciliated columnar epithelium cells with goblet cells that produce mucus...

l wall. It occurs in approximately 1 in 10,000 to 20,000 births. It means there is a gap between the oesophagus and trachea, which allows food or fluid to pass into the airway.

Symptoms and diagnosis

Diagnosis of a laryngeal cleft in an infant often comes after problems with feeding which include coughing, cyanosis

Cyanosis

Cyanosis is the appearance of a blue or purple coloration of the skin or mucous membranes due to the tissues near the skin surface being low on oxygen. The onset of cyanosis is 2.5 g/dL of deoxyhemoglobin. The bluish color is more readily apparent in those with high hemoglobin counts than it is...

 (blue skin) and failing to gain weight over time. Pulmonary

Lung

The lung is the essential respiration organ in many air-breathing animals, including most tetrapods, a few fish and a few snails. In mammals and the more complex life forms, the two lungs are located near the backbone on either side of the heart...

 infections are also common. The longer the cleft is, the more severe the symptoms. A cleft is diagnosed through endoscopic

Endoscopy

Endoscopy means looking inside and typically refers to looking inside the body for medical reasons using an endoscope , an instrument used to examine the interior of a hollow organ or cavity of the body. Unlike most other medical imaging devices, endoscopes are inserted directly into the organ...

 examination, specifically microlaryngoscopy

Laryngoscope

Laryngoscopy is a medical procedure that is used to obtain a view of the vocal folds and the glottis. Laryngoscopy may be performed to facilitate tracheal intubation during general anesthesia or cardiopulmonary resuscitation or for procedures on the larynx or other parts of the upper...

 and bronchoscopy

Bronchoscopy

Bronchoscopy is a technique of visualizing the inside of the airways for diagnostic and therapeutic purposes. An instrument is inserted into the airways, usually through the nose or mouth, or occasionally through a tracheostomy. This allows the practitioner to examine the patient's airways for...

. Laryngeal clefts are classified into four types according to length. Type I extends no further down than the vocal cords

Vocal folds

The vocal folds, also known commonly as vocal cords, are composed of twin infoldings of mucous membrane stretched horizontally across the larynx...

, type II extends below the vocal cords and into the cricoid cartilage, type III extends into the cervical

Neck

The neck is the part of the body, on many terrestrial or secondarily aquatic vertebrates, that distinguishes the head from the torso or trunk. The adjective signifying "of the neck" is cervical .-Boner anatomy: The cervical spine:The cervical portion of the human spine comprises seven boney...

 section of the trachea and type IV extends the furthest—into the thoracic

Chest

The chest is a part of the anatomy of humans and various other animals. It is sometimes referred to as the thorax or the bosom.-Chest anatomy - Humans and other hominids:...

 section of the trachea.

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. Symptoms can be managed by thickening the infant's feeds. 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

Tracheotomy

Among the oldest described surgical procedures, tracheotomy consists of making an incision on the anterior aspect of the neck and opening a direct airway through an incision in the trachea...

.

Associated conditions

20–27% of individuals with a laryngeal cleft also have a tracheoesophageal fistula

Tracheoesophageal fistula

A tracheoesophageal fistula is an abnormal connection between the esophagus and the trachea...

 and approximately 6% of individuals with the fistula also have the cleft. Other congenital anomalies commonly associated with laryngeal cleft are gastro-oesophageal reflux

Gastroesophageal reflux disease

Gastroesophageal reflux disease , gastro-oesophageal reflux disease , gastric reflux disease, or acid reflux disease is chronic symptoms or mucosal damage caused by stomach acid coming up from the stomach into the esophagus...

, tracheobronchomalacia

Tracheobronchomalacia

Tracheobronchomalacia or TBM is a condition characterized by flaccidity of the tracheal support cartilage which leads to tracheal collapse with condition extends further to the bronchi....

, congenital heart defect

Congenital heart defect

A congenital heart defect is a defect in the structure of the heart and great vessels which is present at birth. Many types of heart defects exist, most of which either obstruct blood flow in the heart or vessels near it, or cause blood to flow through the heart in an abnormal pattern. Other...

, dextrocardia

Dextrocardia

Dextrocardia is a congenital defect in which the heart is situated on the right side of the body. There are two main types of dextrocardia: dextrocardia of embryonic arrest and dextrocardia situs inversus...

 and situs inversus

Situs inversus

Situs inversus is a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. The normal arrangement is known as situs solitus...

. Laryngeal cleft can be a component of other genetic syndromes, including Pallister-Hall syndrome

Pallister-Hall syndrome

Pallister-Hall syndrome is a disorder that affects the development of many parts of the body.It is named for Judith Hall and Philip Pallister.-Presentation:...

 and G syndrome (Opitz-Friaz syndrome).