Mysterious fevers of unknown origin: Could surgery be a cure?

Searching the medical literature for answers, Licameli found two small case studies in Europe, where doctors diagnosed PFAPA, removed the tonsils and the children got better, and decided to try this approach. It worked, with an immediate cessation of his daughter’s fever cycles (she’s now almost 6), and other doctors began referring patients to him.

The hallmark of PFAFA is high fevers of a cyclical nature – parents can often look at their calendars and predict what day the fevers will arrive. There are typically no other symptoms. Some children have 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.”

Before undergoing surgery, patients are evaluated for any rheumatologic or infectious etiologies that can also cause cyclical fever.

So 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.

In the meantime, they hope that increased physician awareness will allow children struggling with this condition to be offered surgery sooner.

The study was supported by the Clinical Research Program, Committee of Clinical Investigation and Department of Otolaryngology at Children’s Hospital Boston.

Reference: Arch Otolaryngol Head Neck Surg 2008 Feb; 134:136-140.

Children's Hospital Boston is home to the world's largest research enterprise based at a pediatric medical center, where its discoveries have benefited both children and adults since 1869. More than 500 scientists, including eight members of the National Academy of Sciences, 11 members of the Institute of Medicine and 12 members of the Howard Hughes Medical Institute comprise Children's research community. Founded as a 20-bed hospital for children, Children's Hospital Boston today is a 397-bed comprehensive center for pediatric and adolescent health care grounded in the values of excellence in patient care and sensitivity to the complex needs and diversity of children and families. Children's also is the primary pediatric teaching affiliate of Harvard Medical School. For more information about the hospital and its research visit: www.childrenshospital.org/newsroom.