Tonsillectomy and Adenoidectomy Research

Wracked by seizures and a 105-degree fever, 19-month-old Claire is rushed to the emergency room. The hospital runs test after test and specialists are brought in, but no explanation is found. This isn't the first time: The fevers have struck like clockwork every three weeks for the past nine months. Antibiotics and fever reducers have offered no relief.

Claire's father, Greg Licameli, MD, of Boston Children's Hospital's Otolaryngology Department, decided to search the medical literature for answers. He found two reports of European children with unexplained cyclical fevers, some of whom got better after tonsillectomy. Seeing nothing to lose, he decided to try it. Colleague Dwight Jones, MD, removed Claire's tonsils and adenoids and the fevers immediately stopped.

Other doctors began referring patients to Licameli, who's now operated on 60 patients with this mysterious condition, first reported in 1987 and known as PFAPA (periodic fever, aphthous ulcers, pharyngitis and adenitis). In the March Archives of Otolaryngology and Head and Neck Surgery, he describes 27 of these children, the largest published experience to date; 26 had complete fever resolution.

The reason remains a mystery, since the removed tonsils and adenoids show no evidence of infection or any other abnormality. Licameli suspects the tonsils harbor a chronic, low-level infection to which the immune system overreacts. He and colleagues in Immunology plan to investigate further.

Meanwhile, the referrals keep coming, and desperate families are finding surgery to be life-changing. "I tell parents, 'I don't know why this works, but it has a good chance of ridding your child of fevers,'" Licameli says. "It works in almost every single kid."