Original article
Pediatric Horner Syndrome: Etiologies and Roles of Imaging and Urine Studies to Detect Neuroblastoma and Other Responsible Mass Lesions

https://doi.org/10.1016/j.ajo.2006.05.047Get rights and content

Purpose

To evaluate the frequency of etiologies of Horner syndrome in children and suggest an imaging and laboratory diagnostic protocol to evaluate for neuroblastoma and other lesions in a child presenting with Horner syndrome and no known cause.

Design

Retrospective chart and data review.

Methods

A retrospective review of all children seen at a large pediatric neuro-ophthalmology referral center with a diagnosis of Horner syndrome between 1993 and 2005 with particular attention to underlying etiologies and the results of imaging and urine catecholamine studies.

Results

Fifty-six children met criteria for Horner syndrome and further review. Twenty-eight children (50%) had no previously identified cause for Horner syndrome. Of these children, 24 (85.7%) had urine catecholamine metabolite studies, and all had negative results. Twenty (71.4%) had complete modern imaging of the brain, neck, and chest. Of the 18 children who had complete imaging and urine studies, responsible mass lesions were found in six (33%). Four had neuroblastoma, one had Ewing sarcoma, and the other had juvenile xanthogranuloma. Of all patients (diagnosis known and unknown), neoplasm was the etiology in 13 of 56 (23%) of patients.

Conclusions

We confirm that Horner syndrome in a child of any age without a surgical history requires a complete examination to exclude a mass lesion. In such patients, we recommend brain, neck, and chest magnetic resonance imaging (MRI) with and without contrast as well as urinary catecholamine metabolite testing. However, imaging is more sensitive than urine testing in this setting.

Section snippets

Patient ascertainment and clinical review

Approval from the Children’s Hospital of Philadelphia Institutional Review Board was obtained for this study. The Neuro-ophthalmologic examination records, imaging reports, and pertinent laboratory data were reviewed for all children with a diagnosis of Horner syndrome seen by the Children’s Hospital of Philadelphia Neuro-ophthalmology service from July 1993 through July 2005. For the purposes of this study, Horner syndrome was defined as unilateral miosis with or without ipsilateral ptosis and

Results

Fifty-seven pediatric patients fulfilled criteria for Horner syndrome between 1993 and 2005 and were entered for further review. One six-year-old patient with a positive cocaine test and signs of Horner syndrome noted in infant photos was lost to follow-up and was not included in this study. Patient characteristics are included in Table 2. One patient was diagnosed with bilateral oculosympathetic pareses.15 Half of the patients had no previously established or readily identifiable etiology at

Discussion

A striking number (6/18 [33%]) of children presenting with Horner syndrome without a known etiology, but who subsequently had a complete workup including urine screening and head, neck, and chest imaging were found to have a responsible mass. Of these six children, four had a neuroblastoma found on MRI imaging despite the absence of elevated urine catecholamines.

Nicholas R. Mahoney, MD, graduated from the University of Pennsylvania School of Medicine in May 2006. After completing his internship, he will be an ophthalmology resident at the Scheie Eye Institute, University of Pennsylvania 2007–2010.

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    Nicholas R. Mahoney, MD, graduated from the University of Pennsylvania School of Medicine in May 2006. After completing his internship, he will be an ophthalmology resident at the Scheie Eye Institute, University of Pennsylvania 2007–2010.

    Grant T. Liu, MD, is a Professor of Neurology and Ophthalmology at the University of Pennsylvania. He completed a residency at the Harvard-Longwood Neurology Program and a neuro-ophthalmology fellowship at the Bascom-Palmer Eye Institute. Although he sees both adult and children with neuro-ophthalmic problems, Dr Liu’s special interest is in pediatric neuro-ophthalmology.

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