Orbital heterotopic glial tissue presenting as exotropia

Area of Science:

• Ophthalmology
• Pathology
• Pediatric Oncology

Background:

• Orbital tumors in infants are rare and can present with diverse clinical features.
• Heterotopic glial tissue in the orbit is an exceptionally uncommon finding.
• Optic disc hypoplasia is a congenital anomaly that can be associated with orbital abnormalities.

Observation:

• A 5-month-old infant presented with left eye fixation difficulty, progressive exodeviation, axial proptosis, and optic disc hypoplasia.
• Computed tomography revealed a superomedial orbital mass.
• Histopathology showed glial tissue with skeletal muscle fibers, positive for desmin and glial fibrillary acidic protein.

Findings:

• Surgical debulking partially reduced the tumor, with the eye regaining alignment and motility.
• A recurrence was noted 4 years later, indicated by minimal proptosis and confirmed by MRI.
• The tumor represents a rare instance of isolated orbital heterotopic glial tissue with muscular components.

Implications:

• This case underscores the potential for late recurrence of orbital glial tumors, even after initial surgical intervention.
• Accurate histopathological and immunohistochemical analysis is crucial for diagnosing rare orbital neoplasms.
• Associated ocular anomalies like optic disc hypoplasia may occur with these tumors.