31 March 2008

Mystery Fevers Cured With Surgery

by Kate Melville

Many families go through a regular nightmare where their child suffers a high fever - sometimes causing seizures - and is rushed to ER, where despite a battery of tests no explanation is found. The fevers seem to vanish on their own after four to five days, only to return four to six weeks later with specialists drawing a blank as to the cause. Now, however, the Archives of Otolaryngology and Head and Neck Surgery reports that tonsillectomy (with or without removal of the adenoids) is almost always curative in such cases.

No one knows why the surgery works, as the tonsils and adenoids show no evidence of unusual infection or other abnormality when pathologists study them. But according to the report, desperate parents are opting for the surgery as a last-ditch measure and finding it to be life-changing. "Why taking out tonsils and adenoids works is unclear, but it works in almost every single kid," says Boston Children's Hospital otolaryngologist Greg Licameli, the paper's first author. "I tell parents, 'I don't know why this works, but it has a good chance of ridding your child of fevers.'"

Licameli has now treated 60 children with the condition (known as periodic fever, aphthous ulcers, pharyngitis and adenitis (PFAFA)) and the findings continue to hold up. Given how many children he's seen in just five years, he thinks the condition isn't all that uncommon, though it is underrecognized and unknown to most pediatricians and otolaryngologists.

The hallmark of PFAFA is high fevers of a cyclical nature. There are typically no other symptoms. Some children have a sore throat, swollen glands and small mouth ulcers, but these symptoms aren't very specific and can be very subtle. "On the face of it, it doesn't seem like a surgical problem," Licameli says. "The tonsils and adenoids appear normal, even when you study them pathologically, yet surgery is immediately curative."

What causes this condition, and why does surgery work? Licameli suspects the tonsils may harbor a chronic indolent infection to which the immune system is hypersensitive. He and his colleagues at Children's - including specialists in infectious disease, rheumatology and immunology - plan to pursue these questions further.

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Source: Children's Hospital Boston