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Nationwide population-based incidence and etiologies of pediatric and adult Horner syndrome

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To determine age- and sex-specific incidence and possible etiologies of pediatric and adult Horner syndrome in South Korea.


A nationwide, population-based, cohort study using data from the Korean National Health Claims database from 2007 to 2018. All patients with Horner syndrome from the entire Korean population (n = 51,629,512) were included. To find possible causes of Horner syndrome, we searched concurrent codes for systemic diseases, trauma, or surgical procedures.


A total of 139 pediatric patients (59.7% male) and 1331 adults (51.0% male) were newly diagnosed as having Horner syndrome. The cumulative incidence was 2.12 (95% CI 2.08–2.17) per 100,000 pediatric population and 2.95 (2.94–2.96) per 100,000 adults. The peak incidence occurred at 0–4 years of age in the pediatric population, and at 50–54 years in the adult population. A total of 835 (56.8%) patients had underlying conditions or related surgical procedures associated with Horner syndrome. The underlying causes were recognized in 695 (83.2%) patients before the diagnosis of Horner syndrome, in 75 (9.0%) patients at the same time as the Horner syndrome diagnosis, and in 65 (7.8%) patients after the diagnosis of Horner syndrome. There were four cases of Horner syndrome that preceded neuroblastoma diagnosis. The most common tumor related with Horner syndrome was that of the thyroid in the adult population.


This study determined the estimated incidence and possible causes of pediatric and adult Horner syndrome. As Horner syndrome with unknown etiologies may harbor serious malignancy, extensive evaluations are required, especially in children.

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This study was supported by the “Dongwha” Faculty Research Assistance Program of Yonsei University College of Medicine (6-2017-0169), and the faculty grant of Myongji Hospital (1902-09-01). The sponsor or funding organization had no role in the design or conduct of this research.

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Authors and Affiliations



JH contributed to study concept and design, analysis and interpretation of data, and draft and revision of the manuscript for content, and to obtain funding. Dr. Han reports no disclosure. S-YP contributed to supervision, interpretation of data and Critical revision of the manuscript for important intellectual content. Dr Park reports no disclosure. J-YL contributed to study concept and design, acquisition of data, analysis and interpretation of data, and draft and revision of the manuscript for content. Dr. Lee reports no disclosure.

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Correspondence to Ju-Yeun Lee.

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No conflicting relationship exists for any author.

Ethical standards

Approval from the Institutional Review Board/Ethics Committee from Myongji Hospital (2019–08-009) and Gangnam Severance (3–2019-0117) in Seoul, South Korea was obtained. The study adhered to principles of the Declaration of Helsinki.

Availability of all data and materials as well as software application or custom code

The outflow of all data to the outside is strictly prohibited by national security law. The raw data used in this study can be extracted by request from any qualified investigator through national HIRA system.

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Han, J., Park, S.Y. & Lee, JY. Nationwide population-based incidence and etiologies of pediatric and adult Horner syndrome. J Neurol 268, 1276–1283 (2021).

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