Strabismus in patients with neurofibromatosis type 1-associated optic pathway glioma

Reut Parness-Yossifon, Robert Listernick, Joel Charrow, Heath Barto, Janice Lasky Zeid*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

6 Scopus citations


Purpose To characterize the incidence, type, natural history, and treatment outcomes of strabismus in pediatric patients with neurofibromatosis type 1 (NF1) and optic pathway glioma (OPG). Methods The medical records of consecutive patients diagnosed with NF1 and OPG at our neurofibromatosis clinic since 1985 were reviewed retrospectively. We noted age at diagnosis, reason for referral to our clinic, ophthalmologic examination results, presence or absence of strabismus, strabismus type and treatment, and final results of treatment. Results A total of 76 patients were included. Of these, 22 (28.9%) had strabismus: 5 (22.7%) had esotropia and 17 (77.2%) had exotropia; of those with exotropia, 10 (45%) also had hypotropia. In 12 patients (54.5%), strabismus was present at the first ophthalmologic examination. The development of strabismus preceded the diagnosis of both NF1 and OPG in 4 (5.3%) patients and the diagnosis of OPG in another 2 (2.6%) patients with known NF1. In 16 (76%) patients, the strabismus was sensory. Five patients (22.7%) underwent surgery, which resulted in a deviation angle of <10Δ in 1 patient, <20Δ in 2 patients, and >20Δ in 2 patients. Conclusions Strabismus was the presenting symptom and led to the diagnosis of OPG in a subset of patients, including those who did not have a previous diagnosis of NF1. Exotropia, especially associated with a hypotropia, was the most common strabismic deviation seen in these patients. Sensory strabismus was the most common type seen in this population. Deviations of <10Δ may be difficult to achieve with strabismus surgery.

Original languageEnglish (US)
Article number2272
Pages (from-to)422-425
Number of pages4
JournalJournal of AAPOS
Issue number5
StatePublished - Oct 2015

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Ophthalmology


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