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

ABSTRACT

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.

KEYWORDS:

• Congenital orbital fibrosis
• congenital fibrosis syndrome
• congenital fibrosis of the extraocular muscles

Competing interests

None.

Ethics approval

This retrospective study was approved by the Institutional Review Board of the Seoul National University Bundang Hospital.

Additional information

Notes on contributors

Jeong-Min Hwang

The following contributions were made by the coauthors. Design of the study (NK, JMH); Conduct of the study (NK, HKY, JHK); Manuscript preparation (NK, HKY, JHK); and Review or approval of manuscript (JMH, JHK).