PFAPA, syndrome
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(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