Comparison of Clinical and Radiological Findings between Congenital Orbital Fibrosis and Congenital Fibrosis of the Extraocular Muscles.

Purpose/Aims: To compare the clinical and radiological findings of the two rare entities of congenital orbital fibrosis and congenital fibrosis of the extraocular muscles (CFEOM). Materials

Methods

Clinical characteristics and magnetic resonance imaging (MRI) findings of three congenital orbital fibrosis patients were analyzed and compared to 13 CFEOM patients.

Results

In all three patients with congenital orbital fibrosis, unilateral EOM limitation and enophthalmos were found with a retrobulbar infiltrating mass and normal oculomotor and abducens nerves on MRI. Conversely, most of the patients with CFEOM showed bilateral involvement with hypoplasia of oculomotor nerves and/or abducens nerves and hypotrophic EOM on MRI.

Conclusion

All the patients with congenital orbital fibrosis had normal oculomotor and abducens nerves with a retrobulbar mass infiltrating between EOMs, suggesting that congenital orbital fibrosis is a unique disease entity distinguished from CFEOM.