Airway compression

What breathing problems can be seen in children with PHACE syndrome?

Rarely, infants with PHACE syndrome may have difficulty breathing. Three conditions seen in PHACE syndrome can cause compression (narrowing) of the airway.

  • Subglottic hemangioma
    A subglottic hemangioma is a hemangioma that is in the airway. The main difference between a cutaneous (skin) hemangioma and a subglottic hemangioma is the location of this blood vessel tumor. A subglottic hemangioma is in the airway just below the vocal cords, near the trachea (main breathing tube to the lungs). They are much less common than hemangiomas on the skin. Like other hemangiomas, these lesions undergo rapid growth in the first few months after birth. Infants with subglottic hemangiomas are generally born without any noticeable problems or issues for the first few weeks of life. At roughly 1 to 4 months of age, the hemangioma will begin to grow quickly and could lead to narrowing of the airway and problems with breathing.
  • External compression (narrowing) of the airway from a hemangioma in the neck or chest:
    Compression of any part of the airway can cause breathing problems. A large hemangioma in the neck or chest over the airway can grow big enough to compress the airway. This can lead to problems getting oxygen in and out of the lungs.
  • Vascular ring
    A vascular ring is an abnormal blood vessel that pushes on, or wraps around the trachea. The arteries that deliver blood from the heart to the arms and the veins that bring blood back to the heart are very close to the trachea (see the Congenital Heart Abnormalities section). In PHACE syndrome, there can be an abnormal passage of these blood vessels that can wrap around the trachea and push on or squeeze the airway. This can cause problems with breathing, as the airway will not be as open as it should be.

What are the signs and symptoms of airway compression?

Symptoms of airway compression are stridor, respiratory distress, recurrent diagnosis of croup and feeding problems. Stridor is the "barking" cough that is generally associated with a child who has the viral infection croup. It is due to narrowing of the airway. The infant could have stridor with both breathing in and out, and it could eventually get bad enough for the infant to have problems breathing and maintaining normal oxygen levels (respiratory distress). Sometimes, an infant with airway compression is mistakenly diagnosed with croup. Repeated episodes of croup may signal airway compression. The infant may also have difficulty feeding, because he or she has a hard time feeding and breathing at the same time.

How is airway compression diagnosed and treated?

A subglottic hemangioma is diagnosed by an otolaryngologist (an ear-nose-throat or ENT physician) who can make the diagnosis with an airway examination called endoscopy. An endoscope is a tube with a video camera that is inserted into the infant's nose or throat, where it can look at the airway and look for any obstruction, such as a subglottic hemangioma. Sometimes a lateral neck X-ray can be used to see if the airway is narrow.

Imaging with MRI/MRA can be used to diagnose neck and chest hemangiomas or vascular rings.

Most infants with subglottic hemangiomas need treatments such as oral steroids, vincristine or propranolol.

How common are breathing issues in PHACE syndrome?

Although subglottic hemangiomas are relatively rare, studies have shown that they are seen in 24-52 percent of kids with PHACE syndrome. Features that would make a subglottic hemangioma more likely include a hemangioma on the skin in the "beard" area (front of the ear, jaw, chin, front of the neck, and lips). It is possible, however, to have a subglottic hemangioma, a vascular ring or another hemangioma in the neck or chest pushing on the airway without having any hemangiomas on the skin.

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