Laryngeal Atresia

Laryngeal atresia is an extremely rare condition.  During the early part of the baby’s development, the voicebox (larynx) does not form correctly.  It stays closed and does not open up once the baby is born.  This causes severe breathing problems for the baby at birth.

Sometimes a baby with laryngeal atresia may have other serious problems with their airway. 

Diagnosis

Laryngeal atresia is often found during a routine prenatal ultrasound. However, it may go unnoticed during pregnancy. If discovered at birth, the baby will have severe breathing problems. 

Initial Lifesaving Measures

If the condition was not found during pregnancy, once the baby is born an incision is immediately made into the lower part of the windpipe (trachea) to allow them to breathe. A tube is placed through this opening and breathing is done through this tube rather than through the nose and mouth.

If the laryngeal atresia is found before birth, the baby is delivered by the ex-utero intrapartum treatment procedure (referred to as EXIT). The EXIT procedure is performed in an operating room equipped to treat mother and baby in one location.

Treatment

Once the infant is stable, a team of specialists performs a thorough exam. These doctors, members of the Cincinnati Children’s Aerodigestive Center, will discuss their findings and treatment plan with you. Early treatment focuses on getting the infant ready for surgery to repair the airway. This may include treating any other conditions that may affect the success of the surgery.

In most cases, reconstructive surgery is not performed until the child is 4 years of age. At that time, a cricotracheal resection with a posterior graft is performed. A plastic tube that holds the airway open during healing, called a stent, along with a tracheotomy, or a T-tube (T-shaped breathing tube inserted through the skin), is left in place for six months.

Surgery for these children is challenging, and many children need more than one operation before removing the tracheotomy.

Long-Term Outlook

Many children with laryngeal atresia require multiple operations during their lifetime. They will have voice problems as they grow. Their voice will sound different because it will not come from the vocal cords. Such post-surgical voice problems are managed by a team of voice experts at Cincinnati Children’s Center for Pediatric Voice Disorders.

Last Updated 03/2016