1

Introduction

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome was first described by Marshall et al and is characterized by periodic episodes of fever (>39°C) lasting 3 to 6 days and recurring every 3 to 8 weeks, accompanied by aphthous stomatitis, pharyngitis, cervical adenitis, and normal growth and development . Because specific laboratory abnormalities have not been shown, PFAPA syndrome is defined clinically and the diagnosis is one of the exclusion . The purposes of the present study are ( a ) to evaluate specific histologic findings in children with PFAPA syndrome who had tonsillectomy and ( b ) to assess any improvement of symptoms after tonsillectomy.

2

Materials and Methods

This is a retrospective study of children with PFAPA syndrome who underwent tonsillectomy with or without adenoidectomy at the Department of Otorhinolaryngology Head and Neck Surgery of “Aghia Sophia” Children’s Hospital, a tertiary care pediatric hospital in Athens (Greece), between May 2007 and July 2008. Nine children with PFAPA syndrome were recruited into this study. Patients with defined diagnostic criteria for PFAPA syndrome till 14 years of age undergoing tonsillectomy with or without adenoidectomy were included in the study. These criteria included the following signs and symptoms: regularly recurring fevers of unknown origin with a typical, regular, and completely asymptomatic intervals of 2 to 5 weeks between episodes, and at least one of the following clinical signs: aphthous stomatitis, cervical lymphadenitis, and pharyngitis. Patients with cyclic neutropenia and significant medical disorders were excluded. A full blood count and erythrocyte sedimentation rate was performed to all of the children included.

The method of tonsillectomy used was the classical cold knife with emphasis on blunt dissection. Hemostasis especially in upper and lower tonsillar poles was obtained by clipping the site of hemorrhage and using silk sutures. Adenoidectomy was performed with the use of mirrors and curettes and suction cautery used for hemostasis. Tonsils were sent to the pathology department for histologic examination. Hematoxylin-eosin stains were used for histology examination. The immunohistochemical Bond Refine detection, a biotin-free polymeric horseradish peroxidase–linker antibody conjugate system (Bond Polymer Refine kit: Vision Biosystems Ltd, Newcastle Upon Tyne, UK), was used on paraffin sections for the detection of B (CD20/L-26: Novocastra Ltd, Newcastle Upon Tyne, UK) and T (CD3/PS1; Novocastra Ltd, Newcastle Upon Tyne, UK) lymphocytes on the Bond-automated system .

The research protocol was conducted after its approval by the departmental scientific and ethical review board. Informed parental consent was obtained from each eligible child before enrollment of the study.

2

Materials and Methods

This is a retrospective study of children with PFAPA syndrome who underwent tonsillectomy with or without adenoidectomy at the Department of Otorhinolaryngology Head and Neck Surgery of “Aghia Sophia” Children’s Hospital, a tertiary care pediatric hospital in Athens (Greece), between May 2007 and July 2008. Nine children with PFAPA syndrome were recruited into this study. Patients with defined diagnostic criteria for PFAPA syndrome till 14 years of age undergoing tonsillectomy with or without adenoidectomy were included in the study. These criteria included the following signs and symptoms: regularly recurring fevers of unknown origin with a typical, regular, and completely asymptomatic intervals of 2 to 5 weeks between episodes, and at least one of the following clinical signs: aphthous stomatitis, cervical lymphadenitis, and pharyngitis. Patients with cyclic neutropenia and significant medical disorders were excluded. A full blood count and erythrocyte sedimentation rate was performed to all of the children included.

The method of tonsillectomy used was the classical cold knife with emphasis on blunt dissection. Hemostasis especially in upper and lower tonsillar poles was obtained by clipping the site of hemorrhage and using silk sutures. Adenoidectomy was performed with the use of mirrors and curettes and suction cautery used for hemostasis. Tonsils were sent to the pathology department for histologic examination. Hematoxylin-eosin stains were used for histology examination. The immunohistochemical Bond Refine detection, a biotin-free polymeric horseradish peroxidase–linker antibody conjugate system (Bond Polymer Refine kit: Vision Biosystems Ltd, Newcastle Upon Tyne, UK), was used on paraffin sections for the detection of B (CD20/L-26: Novocastra Ltd, Newcastle Upon Tyne, UK) and T (CD3/PS1; Novocastra Ltd, Newcastle Upon Tyne, UK) lymphocytes on the Bond-automated system .

The research protocol was conducted after its approval by the departmental scientific and ethical review board. Informed parental consent was obtained from each eligible child before enrollment of the study.

3

Results

Nine children met our inclusion criteria in the PFAPA group, of which 5 were male (55.56%) and 4 were female (44.44%), with ages ranging between 2.5 and 5 years at the age of surgery (mean, 3.4 years). The mean duration of symptoms before surgery was 18.78 months (range, 12–30 months). Fever during the attack had a mean duration of 4.3 days (range, 3–6 days). Aphthous stomatitis was observed in 66.7% of children, cervical adenitis in 100%, pharyngitis in 66.7%, abdominal pain in 44.4%, and arthritis in 33.3% of children included in this study. Eight (88.89%) of 9 patients had complete remission of symptoms immediately after surgery. One child (followed up only by telephone) had 2 episodes of fever after surgery with signs and symptoms compatible with PFAPA syndrome; the first episode was 2 months and the second one was 7 months after surgery. The patient was treated with cimetidine. Since then, the child’s symptoms resolved completely. For the rest of PFAPA patients, the postoperative recovery was typical of other children who undergo this surgery for chronic tonsillitis or obstructive sleep disturbance. Table 1 lists details for all PFAPA patients.

Table 1

Study characteristics of children with PFAPA syndrome

Patient	Sex	Age at surgery (y)	TE	AE	Onset of PFAPA (age)	Recurrence of fever (wk)	Duration of fever (d)	Cervical adenopathy	Pharyngitis	Aphthous stomatitis	Abdominal pain	Arthritis	Follow-up (mo)	Recurrence of PFAPA after surgery	Therapy
															A	Co	Ci
1	Female	2.5	+	−	5 mo	3	3	+	+	+	+	−	19	−	+	+	+
2	Male	2.5	+	−	1.5 y	3	4	+	+	−	−	−	17	+	+	+	+
3	Female	2.5	+	−	10 mo	3	6	+	+	+	−	−	12	−	+	+	+
4	Male	2.5	+	−	1 y	4	5	+	+	+	+	−	15	−	+	+	−
5	Male	3	+	+	8 mo	2	3	+	−	+	+	−	14	−	+	+	−
6	Female	4	+	+	1.5 y	3	6	+	−	+	−	−	8	−	+	+	−
7	Female	4	+	−	3 y	2	3	+	+	+	+	+	10	−	+	−	+
8	Male	5	+	−	4 y	4	4	+	+	−	−	+	8	−	+	+	−
9	Male	5	+	−	4 y	3	5	+	+	−	−	+	6	−	+	+	−

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