Laryngeal Cleft

Is a rare abnormality of the separation between the larynx and the esophagus.  When the larynx develops normally it is completely separate from the esophagus, so swallowed foods and liquids go directly into the stomach.  A laryngeal cleft creates an opening between the larynx and the esophagus so food and liquid are in danger of passing through the larynx into the lungs.  They 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 crinoid cartilage, type III extends into the cervical section oft he trachea and type IV extends the furthest- into the thoracic section of the trachea. Occurs in less than 0.1 percent of the population. 

Symptoms:  Swallowing problems, coughing, gagging, frequent respiratory infections and chronic lung disease, cyanosis, failure to gain weight overtime, pulmonary infections.

Tests:  Diagnosed through a comprehensive aero-digestive evaluation.  A barium swallow study or FEES swallow study will determine if aspiration on liquids/foods is happening.  It is diagnosed through a endoscopic examination (microlaryngoscopy and bronchoscopy). 

Treatment:  Depends on the length and resulting severity of symptoms.  A type I cleft may not require surgical intervention.  Symptoms can be managed by thickening liquids and foods.  A slightly longer cleft is repaired endoscopically (long type I and short type II).  A cleft that is longer (type II or type III) is repaired directly through the neck with a tracheotomy. 

Resources:  Dr. Cotton (513) 636-4355 at Children's Hospital in Cincinnati
Dr. Rahbar (617) 355-6460 at Children's Hospital of Boston
Online Support Group- http://laryngealcleftkids.ning.com/

Photo: Marcelle and her little miracle, Aiden

Marcelle's Story:Our son, Aiden, is 2 years old and has a laryngeal cleft.  He was diagnosed with a type II laryngeal cleft when he was 2 months old.  We first saw symptoms such as choking and having apnea spells with these feeds.  He was admitted to Children's Hospital for a week of testing.  While there Aiden was spiking fevers after his feeds.  After numerous tests and procedures, he had a swallow study that showed aspiration on thin liquids.  He then had a bronchoscopy which showed a type II laryngeal cleft.  His laryngeal cleft was surgically (endoscopically) repaired when he was 6 months old.  Aiden has been in swallow therapy since he was 5 months old.  Despite the repair and swallow therapy he continues to aspirate on thin liquids.  We will continue swallow therapy and repeated swallow studies to ensure he is making improvements in his ability to swallow liquids.