The Risk of Neuroblastoma in Paediatric Horner Syndrome—A Systematic Reappraisal of the Literature

Abstract

Paediatric Horner syndrome is associated with neuroblastoma. The strength of this association is unclear, however, creating uncertainty regarding how intensively to investigate such cases. We systematically reviewed the literature to better elucidate the risk of neuroblastoma in cases of paediatric Horner syndrome and to assist clinicians in formulating investigation protocols. The quality of the evidence base is poor. Estimates of the risk of neuroblastoma varied widely, but the best estimate that can be derived from the literature is a risk of 1 in 10 for cases presenting to large institutions. Furthermore, including other mass lesions increases the risk to around 1 in 7. The only population-based study addressing this topic is not at odds with these findings, despite previous authors’ conclusions to the contrary. The risk of neuroblastoma in paediatric Horner syndrome is significant and, furthermore, in view of the low incidence of the syndrome, it would imply negligible health economic burden to image every case without a known cause.

KEYWORDS::

• Horner syndrome
• Investigation
• Neuroblastoma
• Paediatric

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. No funding was obtained for this study.