Namju Kim1, Hee Kyung Yang1, Jae Hyoung Kim2, Jeong-Min Hwang1. 1. a Department of Ophthalmology , Seoul National University College of Medicine, Seoul National University Bundang Hospital , Seongnam , Korea. 2. b Department of Radiology , Seoul National University College of Medicine, Seoul National University Bundang Hospital , Seongnam , Korea.
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.
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 fibrosispatients 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.
Entities:
Keywords:
Congenital orbital fibrosis; congenital fibrosis of the extraocular muscles; congenital fibrosis syndrome