Periodic Fevers in a Child - PFAPA

Fever in a child is not uncommon. After all, they get a lot of infections whether it be ear infections, tonsillitis, viral syndromes, etc.

However, what can be particularly vexing and confusing for both physicians and families is a child who keeps getting recurring fevers every few weeks out of nowhere for seemingly no reason and no obvious infectious source.

The typical scenario is a child with abrupt onset of fevers to ~102 degrees F or higher that lasts 3-7 days and just as inexplicably disappears... only to come back again 3-6 weeks later. This cycle may be repeated numerous times. In between these fever episodes, the child is completely fine.

This fever cycle may be associated with one or more of the following:

• Aphthous stomatitis (inflammation of the mouth mucosa)
• Pharyngitis (inflammation of the throat mucosa)
• Cervical Adenopathy (large lymph nodes in the neck)

Though a specific finding of aphthous stomatitis or pharyngitis may not be made (child may be uncooperative with a full oral exam), the child will usually complain of a sore throat, swallowing difficulties, or may suffer from poor appetite.

Numerous tests can be obtained during a fever episode without any positive findings. Strep cultures are negative. Bloodwork is unrevealing.

Trial courses of antibiotics make no difference.

What can this be???

It is most likely due to a syndrome called PFAPA (Periodic Fevers, Aphthous stomatitis, Pharyngitis, cervical Adenitis). The hallmark feature of this syndrome is the cyclical almost clockwork regularity of acute-onset high fevers.

What is the treatment?

Tonsillectomy and adenoidectomy (T&A) is almost always curative.

Why?

Given we have no idea what even really causes this syndrome, we also don't really know why T&A works either,  but just know from studies that it does work.

Reference:
Long-term Surgical Outcomes of Adenotonsillectomy for PFAPA Syndrome. Arch Otolaryngol Head Neck Surg. 2012;138(10):902-906. doi:10.1001/2013.jamaoto.313