PFAPA, syndrome

(Marshall syndrome)

Acronym for Periodic Fever, Aphtous stomatitis, Pharyngitis, and cervical Adenitis.

Disease starting before the age of 5 years and characterized by cyclic febrile attacks (3-4 days every 3 to 8 weeks) accompanied by stomatitis, pharyngitis and cervical lymphadenopathy.
Other rarer manifestations are: headache, abdominal pain, arthralgias. The cause is unknown: it seems to be an autoinflammatory disease such as the mediterranean fever.

Treatment is symptomatic: antipyretics. Some use colchicine or steroids.
Tonsillectomy often leads to a spectacular remission of the febrile episodes but it is used only for cases resistant to medical treatment.


Anesthetic implications:

continue the usual treatment. In case of colchicine treatment: hematologic control. In case of steroids treatment: perioperative supplementation. Problem: differential diagnosis between attacks of periodic fever and a surgical complication.


References : 

-         Garavello W, Pignataro L, Gaini L, Torretta S, Somigliana E, Gaini R. 
Tonsillectomy in children with periodic fever with aphtous stomatitis, pharyngitis, and adenitis syndrome. 
J Pediatr 159; 159: 138-42.


Updated: October 2018