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Reprinted by permission of Int J Pediatr Otorhinolaryngol. 2016; 89:149-153.

International Journal of Pediatric Otorhinolaryngology 89 (2016) 149 e 153

Contents lists available at ScienceDirect

International Journal of Pediatric Otorhinolaryngology

journal homepage: http://www.ijporlonline.com/

PANDAS: A systematic review of treatment options * Zachary Farhood, Adrian A. Ong, Christopher M. Discolo * Department of Otolaryngology e Head and Neck Surgery, Medical University of South Carolina, United States

a r t i c l e i n f o

a b s t r a c t

Article history: Received 26 April 2016 Received in revised form 9 August 2016 Accepted 10 August 2016 Available online 12 August 2016

Introduction: Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus (PANDAS) is a rare but important condition for pediatric otolaryngologists to recognize. Several treatment options exist including tonsillectomy, antibiotic treatment/prophylaxis, intravenous immunoglobulin (IVIG), and psychiatric medications/therapy. Methods: A systematic review of the PubMed, EMBASE, and Scopus databases was performed searching for articles that focused exclusively on the aforementioned treatment modalities in the PANDAS popu- lation. Review articles, single patient case reports, and studies examining the natural history or diag- nostic strategies were excluded. Results: Five articles regarding tonsillectomy treatments with level of evidence (LOE) 4 were found but no clear bene fi t could be determined. Three articles were selected involving the use of antibiotic therapy. One prospective study and one double-blind randomized control trial (DB RCT) supported the use of antibiotics but a separate DB RCT showed no bene fi t. Two selected articles described the use of IVIG: one unblinded RCT and one retrospective study. One prospective study on cognitive-behavioral therapy (CBT) showed bene fi t in PANDAS. Conclusion: There is a paucity of high-level studies regarding this rare disorder and no hard treatment recommendations can be made. Tonsillectomy should only be performed in those who are surgical candidates based on current published guidelines. Antibiotics are an option but provide uncertain bene fi t. CBT remains a low-risk option. Studies support the use of IVIG, however more investigation is needed prior to widespread adoption of this treatment given its potential risks. © 2016 Elsevier Ireland Ltd. All rights reserved.

Keywords: Pediatric autoimmune neuropsychiatric disorders associated with streptococcal

infections Treatment

1. Introduction

medical therapy, and cognitive behavioral treatment. The objective of this study was to systematically review the literature for treat- ment options of PANDAS.

Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus (PANDAS) is a rare pediatric disorder with otolaryngic ties that was described by Swedo et al., in 1998 with speci fi c criteria ( Table 1 ) [1] . The criteria continue to be re fi ned but the course of the disease involves tics and/or obsessions/compul- sions that worsen in relation to acute infection caused by group A b - hemolytic (GABHS) streptococcus. This is a waxing and waning disorder with an incompletely understood pathophysiology. Several treatment options have been proposed including surgery,

2. Methods

Systematic reviews of published literature are deemed exempt by our institutional review board. A systematic review of the PubMed, EMBASE, and Scopus databases was performed in August 2015, searching for articles focusing exclusively on treatment mo- dalities in the PANDAS population. The primary outcome was improvement in symptoms. There were no date restrictions. The speci fi c search strategy is detailed in Table 2 . Articles in the English or Spanish language were included. Re- view articles, single patient case reports, and studies examining the natural history or diagnostic strategies were excluded. Two in- dividuals (Z.F. and A.A.O) performed the search and analyzed each article for appropriateness to include in the systematic review. The

* This manuscript was presented at the 2015 Society for Ear, Nose, and Throat Advancement in Children Meeting, December 4 e 6, 2015, San Antonio, TX. * Corresponding author. 135 Rutledge Ave, MSC 550, Charleston, SC 29425-5500, United States. E-mail address: discolo@musc.edu (C.M. Discolo).

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