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Congenital High Airway Obstruction Syndrome

Home Congenital High Airway Obstruction Syndrome

Introduction

Congenital High Airway Obstruction Syndrome, also known as CHAOS, is a medical condition that affects babies in utero. It is a rare condition and affects about 1 in 50,000 babies. If left untreated, the condition can prove lethal and cause severe health complications.

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What is Congenital High Airway Obstruction Syndrome (CHAOS)?

Congenital High Airway Obstruction Syndrome refers to the blockage of the windpipe (trachea) or voice box (larynx) of foetuses.

When the baby is in the uterus, its lungs constantly produce fluid. This fluid is released out of the baby’s body through the mouth. The blockage of the windpipe and voice box makes it difficult for the fluid to come out of the mouth. This leads to the collection of fluid in the baby’s lungs. The excessive fluid collection puts pressure on the baby’s heart and affects its ability to function properly. It may even lead to congestive heart failure.

What Causes Congenital High Airway Obstruction Syndrome?

The exact causes are not known yet. However, the doctors believe the following to be the causes of congenital high airway obstruction syndrome:

 

  • ● Laryngeal atresia – occurs when the baby’s larynx gets blocked during its development in utero
  • ● Tracheal agenesis – a birth defect where the trachea is absent in the baby
  • ● Subglottic stenosis – a condition in which the airway is narrowed below the vocal cords and above the trachea in the baby
  • ● Laryngeal cysts – a birth defect in which cysts develop in the baby’s larynx and epiglottis.

What are the Symptoms of Congenital High Airway Obstruction Syndrome?

If your baby has Congenital High Airway Obstruction Syndrome, the following symptoms may appear on the ultrasound:

  • ● Enlarged lungs
  • ● Dilated bronchi or trachea
  • ● Diaphragmatic flattening or inversion
  • ● Presence of free fluid in the baby’s abdomen
  • ● The fetal heart may look displaced.

How is Congenital High Airway Obstruction Syndrome Diagnosed?

Depending on the symptoms that appear on the ultrasound, the doctor may do the following tests to confirm the diagnosis of Congenital High Airway Obstruction Syndrome:

  • Amniocentesis

This procedure involves taking a small sample of the amniotic fluid that surrounds the foetus in the uterus. It will be taken for laboratory analysis to check for chromosomal abnormalities and genetic syndromes.

  • Fetal echocardiogram

This test is like that of ultrasound. The doctor will place a small instrument known as a transducer on your abdomen. The transducer will emit sound waves that will move through your body and to your baby’s heart. Here, the sound waves will bounce off the structure of the heart and return to the transducer. It will create a detailed image of your baby’s heart and help the doctor look for abnormalities.

  • Fetal MRI

An MRI will create images of the baby’s airway and heart. It can help the doctor determine the extent of the obstruction in the airway.

How is Congenital High Airway Obstruction Syndrome Treated?

Once the doctor diagnoses your baby’s Congenital High Airway Obstruction Syndrome through proper evaluation, he/she will devise a treatment plan for you.

If the diagnosis shows the excess build-up of fluid in your baby’s lungs, abdomen, or scalp, the doctor may recommend fetal surgery to relieve the obstruction.

In most cases, babies with Congenital High Airway Obstruction Syndrome are born with breathing problems. If this is the case with your baby, the doctor may recommend an emergency C-section.

The C-section will be performed with a special procedure, known as the EXIT (Ex-Utero Intrapartum Treatment). It will help provide the baby with an airway to resolve the breathing problem before getting separated from the placenta. This is done in order to ensure the baby is not deprived of oxygen for too long after the delivery and minimise the chances of brain damage.

What is the The EXIT Procedure for Congenital High Airway Obstruction Syndrome?

For this procedure, the surgeon will open up your abdomen and uterus, just like a C-section. Then, the baby’s head and upper body will be delivered. Before cutting the umbilical cord, the surgeon will evaluate your baby’s breathing status and insert a tube to create an artificial airway. This will help your baby breathe after the delivery.

Once your baby and his/her airway are stable, the surgeon will cut the umbilical cord.

Depending on the extent of the airway obstruction, the surgeon may even repair it at the time of the EXIT procedure.

Result

The result of the treatment options for babies with Congenital High Airway Obstruction Syndrome depends on its severity and causes. In severe cases, the baby may have long-term complications such as speaking or drinking and eating through the mouth.

What are the risks associated with Congenital High Airway Obstruction Syndrome surgery?

As the surgeon performs the EXIT procedure, there is a risk of a few complications that may arise during the surgery. Since the baby is halfway out of the mother’s uterus, there is a risk of:

  • ● Umbilical cord compromise
  • ● Excessive uterine bleeding
  • ● Premature placental detachment
  • ● Malpositioned endotracheal tube
  • ● Failure to secure an airway.

In extremely rare cases, there is a risk of fetal death as well. However, in case of any complication, the medical team will work together to ensure you and your baby are both healthy and the surgery turns out to be successful.

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FAQ's

What is the Most Common Associated Genetic Disorder with Congenital High Airway Obstruction Syndrome?

Fraser’s syndrome is the most common genetic disorder associated with Congenital High Airway Obstruction Syndrome. It can be characterised by:

  • Renal Agenesis: A condition where the newborn is missing one or both kidneys
  • Tracheal or Laryngeal Atresia: A condition where the baby’s trachea or larynx is blocked
  • Microphthalmia: An abnormal condition where one or both eyes of the baby are small. Sometimes, it may look like the eyeball is completely missing

Is Congenital High Airway Obstruction Syndrome Fatal?

Yes, Congenital High Airway Obstruction Syndrome is fatal. However, it is extremely rare as well. Even though some researchers have reported chromosomal abnormalities and genetic syndromes to be associated with Congenital High Airway Obstruction Syndrome, the exact causes are still unknown, which proves it to be fatal.

Can a Baby be Completely Cured of Congenital High Airway Obstruction Syndrome?

There have been successful fetal surgeries that have cured babies of Congenital High Airway Obstruction Syndrome. But, to date, the EXIT procedure remains to be the only chance of survival of the foetus.

Can the Compression of the Oesophagus in Utero Result in Other Conditions?

Yes, when an excess fluid build-up develops in the baby’s airway, it can lead to other conditions such as polyhydramnios – an abnormal medical condition where an excess amount of fluid gets collected in the amniotic sac.

What are the Other Genetic Disorders Associated with Congenital High Airway Obstruction Syndrome?

The other genetic disorders associated with Congenital High Airway Obstruction Syndrome are:

  • ● Shprintzen–Goldberg Omphalocele Syndrome (SGOS)
  • ● Short-Rib Polydactyl Syndrome (SRPS)
  • ● VATER Syndrome

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