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Ocular manifestations and long-term complications of rhabdomyosarcoma in children

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Abstract

Background/objectives

The purpose of the study was to describe the ocular manifestations of rhabdomyosarcoma in a large cohort of children.

Subject/methods

This was a retrospective observational cohort study. The medical records of all pediatric patients with head and neck rhabdomyosarcoma diagnosed between 1997 and 2021 at a tertiary-care pediatric hospital were analyzed. The main outcome measures were the incidence and prognostic role of ocular findings at presentation and long-term ocular complications.

Results

There were 77 children with head and neck rhabdomyosarcoma in the study cohort with 38 patients showing ocular manifestations at presentation. Median age at diagnosis was 6.0 years, the median follow-up was 5.7 years and 54.5% were male. At last follow-up, 70.1% had no evidence of progression, 26.0% were deceased, and 2.6% were on palliative treatment. Orbital signs were common (44.2%). The most common ocular findings were proptosis (18.2%), restriction of extraocular motility (28.6%), strabismus/diplopia (22.1%) and ptosis (16.9%). The most common long-term complications were bony hypoplasia/facial asymmetry (40.3%) and keratopathy/dry eye (31.2%). Poor visual acuity (≤20/200) was noted in 13 (16.9%) patients with 5 (6.5%) patients requiring an exenteration. Survival was 100% in primary orbital RMS (p = 0.02), whereas any or a combination of cranial nerve palsies carried a poor prognosis (42% survival, p = 0.008).

Conclusions

In our cohort, half of children with rhabdomyosarcoma had ocular manifestations at presentation with about one-third showing orbital tumor involvement. Cranial nerve involvement carried a significantly worse prognosis for survival.

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Fig. 1: Kaplan–Meier curve of paediatric patients with head and neck rhabdomyosarcoma (RMS).

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Data availability

Data available on request from the authors.

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Authors and Affiliations

  1. Department of Ophthalmology and Vision Sciences, University of Toronto and Hospital for Sick Children, Toronto, ON, Canada

    Sybille Graef, Dan DeAngelis & Michael J. Wan

  2. Department of Pediatrics, Division of Hematology/Oncology, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

    Abha A. Gupta

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  1. Sybille Graef
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  2. Dan DeAngelis
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  4. Michael J. Wan
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Contributions

Sybille Graef—responsible for concept/design of article, data acquisition, data analysis, drafting of article, approved version to be published, accountable for all aspects of the work. Dan DeAngelis—responsible for concept/design of article, drafting of article, approved version to be published, accountable for all aspects of the work. Abha A. Gupta—responsible for concept/design of article, drafting of article, approved version to be published, accountable for all aspects of the work. Michael J. Wan—responsible for concept/design of article, data analysis, drafting of article, approved version to be published, accountable for all aspects of the work.

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Correspondence to Michael J. Wan.

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Graef, S., DeAngelis, D., Gupta, A.A. et al. Ocular manifestations and long-term complications of rhabdomyosarcoma in children. Eye (2024). https://doi.org/10.1038/s41433-024-03175-1

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