Pfeiffer Sydrome: Treatment

Treating Sleep Apnea:
There are various degrees of sleep apnea.  A small amount of sleep apnea can be considered normal, and the amount of sleep apnea that is normal varies with age.  There are also two kinds of sleep apnea, central and obstructive.  Central apnea basically occurs when the brain “forgets” to breathe.  It may be caused by cerebellar tonsillar herniation (sometimes called a “Chiari Malformation”), or it just may occur on it’s own.  The treatment for central apnea may include enlarging the back of the skull, and Chiari decompression.  Obstructive apnea occurs with a narrowed or blocked airway, and is the most common cause in Pfeiffer syndrome.  If a child is found to have significant sleep apnea, there are different treatments that may be prescribed.

The first treatment for obstructive sleep apnea sometimes is medication.  There are a number of medicines that can open up a partially blocked airway in order to make breathing easier.  There is also a medicine that can help central apneas (as long as it isn’t caused by cerebellar tonsillar herniation).  The next step for treating obstructive apnea, if medications are not completely successful, is to consider removing the tonsils (if they are enlarged at all).  If the tonsils are to be removed, it may be a good idea to leave in the adenoids to help with speech; however, this varies with a number of factors.  Frequently, if the sleep apnea is not too severe, a tonsillectomy will be all that is needed.  The last option, short of major surgery, is a CPAP or BiPAP mask.  This is a facemask that is worn at night, which delivers a rush of air, each time the child breaths in.  It usually works very well when worn, however, most parents find it difficult to keep on a child all night.  Often children will pull the mask off sometime in the middle of the night, making this treatment not very effective.  I recommend that if children cannot fall asleep with the mask on, that parents let them first fall asleep, and then put it on. If none of the above treatments work, then either a tracheostomy is recommended, or surgery to bring the mid face forward is required (depending on the patient’s age).