Congenital High Airway Obstruction Syndrome

Also known as: CHAOS, congenital upper airway obstruction

What is congenital high airway obstruction syndrome?

If a fetus’s upper airway (windpipe -”trachea” or voice box- “larynx”) becomes blocked during intrauterine development, the problem is called congenital high airway obstruction syndrome, or CHAOS.

What causes congenital high airway obstruction syndrome? 

CHAOS is extremely rare. The cause of this birth defect is not known.

What are the signs/ symptoms of congenital high airway obstruction syndrome? 

CHAOS is a life-threatening condition. In the fetus, it presents as very large lungs filled with fluid, a flattened diaphragm, fluid in the abdomen, and signs of heart failure .which may develop and cause the death of the baby in the womb or shortly after birth, depending on its severity.

What are congenital high airway obstruction syndrome care options? 

During pregnancy the baby will require close monitoring and at birth a multidisciplinary team of pediatric specialists (neonatologist, pediatric surgeon and maternal-fetal specialist) may be needed to ensure the baby's ability to breath and survival. While the baby usually survives to birth, he/she may suffer from extreme difficulties breathing and survival may depend on the partial delivery of the baby by cesarean section while still being attached to the mother by the placental cord, during which time an artificial airway is established for the baby to breath through (EXIT). Once this is completed the baby is then fully delivered by the completion of the cesarean section.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 6/11/2018 2:29:41 PM

From the Newsdesk

Pediatric Neurosurgeon and Chief of Surgery for Nicklaus Children’s Passes Away
05/24/2018 — The medical staff, employees and volunteers of Nicklaus Children’s Hospital mourn the passing of our esteemed Dr. Sanjiv Bhatia, a longstanding leader and dedicated champion for children with complex medical conditions and their families.
Meet Yamilet Tirado, MD - The Division of Otolaryngology (ENT) at Nicklaus Children's Hospital
04/11/2018 — Dr. Yamilet Tirado is employed by Pediatric Specialists of America (PSA), the physician-led multispecialty group practice of Nicklaus Children’s Health System. She is a pediatric otolaryngologist/ear, nose and throat (ENT) specialist within the Division of Otolaryngology and sees patients at Nicklaus Children's Hospital and the Nicklaus Children's Aventura Care Center.


Dr. David Drossner, a pediatric cardiologist with The Heart Program at Nicklaus Children's Hospital, explains the importance of identifying congenital hearth disease.