Orbital Heterotopic Glial Tissue Presenting as Exotropia

A 5-month-old female infant was noted to have difficulty in fixating with the left eye with accompanying progressive exo-deviation and axial proptosis. She also had optic disc hypoplasia with the double ring sign. Computed tomography showed left superomedial orbital mass without any orbital bony def...

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Bibliographic Details
Published in:Orbit (Amsterdam) 2008-01, Vol.27 (3), p.165-168
Main Authors: Kiratli, Hayyam, ekero lu, Mehmet Ali, Tezel, Gaye Güler
Format: Article
Language:English
Subjects:
Biopsy, Needle
Choristoma - diagnosis
Choristoma - pathology
Choristoma - surgery
desmin
Diagnosis, Differential
Ectopic brain
exotropia
Exotropia - diagnosis
Exotropia - pathology
Female
Follow-Up Studies
glial fibrillary acidic protein
Humans
Immunohistochemistry
Infant
Neuroglia
optic disc hypoplasia
orbit
Orbital Diseases - diagnosis
Orbital Diseases - pathology
Orbital Diseases - surgery
Risk Assessment
Severity of Illness Index
Tomography, X-Ray Computed
Treatment Outcome
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Summary:A 5-month-old female infant was noted to have difficulty in fixating with the left eye with accompanying progressive exo-deviation and axial proptosis. She also had optic disc hypoplasia with the double ring sign. Computed tomography showed left superomedial orbital mass without any orbital bony defect. Incisional biopsy through a medial orbitotomy allowed significant reduction in tumor burden except for the most apical portion. The affected eye resumed normal alignment and full motility. Histopathologically, the tumor was composed of glial tissue intermixed with muscle fibers. Immunohistochemically, desmin and glial fibrillary acidic protein were strongly expressed. Minimal proptosis of the left eye heralded the recurrence of the tumor 4 years later, also confirmed by magnetic resonance imaging studies. This patient embodies the rare occurrence of isolated heterotopic glial tissue in the orbit with skeletal muscle as one of its components and optic disc hypoplasia as the associated ocular anomaly. Late recurrence may occur following incomplete tumor removal.
ISSN:0167-6830
1744-5108
DOI:10.1080/01676830701523889