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Retinal Pigment Epithelium Transplantation in a Non-human Primate Model for Degenerative Retinal Diseases

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Ependymal tissue in microphthalmia with cyst.

Birgit Lorenz¹, Heike M Elflein, Ferdinand Hofstädter

¹Department of Pediatric Ophthalmology and Ophthalmic Genetics, Klinikum, University of Regensburg, Regensburg, Germany. birgit.lorenz@klinik.uni-regensburg.de

Ophthalmic Genetics

|December 7, 2006

Summary

This study reports on a male infant with microphthalmia and optic nerve coloboma. Ectopic ependymal cells in the cyst may produce cerebrospinal fluid, not indicating a CNS connection.

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Area of Science:

Ophthalmology
Pediatric Neurology
Developmental Biology

Background:

Microphthalmia and optic nerve coloboma are congenital ocular abnormalities.
Orbital cystic masses can present as significant challenges in pediatric patients.

Observation:

A male infant presented with a large, progressively enlarging orbital cyst causing proptosis and obscuring the globe.
MRI revealed a microphthalmic globe and a multi-compartmented cystic mass.
The fellow eye exhibited a large optic nerve coloboma.

Findings:

Aspiration of the cyst yielded fluid resembling cerebrospinal fluid (CSF).
Histopathological examination of the cyst wall revealed ectopic ependymal cells, suggesting local CSF production.
No direct communication between the cyst and the central nervous system (CNS) was identified.

Implications:

The presence of CSF in such orbital cysts does not necessitate a CNS connection.
Ectopic ependymal cells are a potential source of CSF within orbital cystic masses.
This finding has implications for the diagnosis and management of congenital orbital anomalies.