Congenital fibrosis of the extraocular muscles: magnetic resonance imaging findings and surgical treatment.

PURPOSE

We analyzed findings of orbital and cranial magnetic resonance imaging (MRI) in patients with congenital fibrosis of the extraocular muscles (CFEOM). We described surgery and its outcome.

MATERIAL AND METHOD

Nine out of 10 patients with clinical findings of CFEOM underwent orbital and cranial MRI to perform a study of the extraocular muscles and cranial nerves. A multimodality workstation platform developed by the imaging laboratory of our hospital for PC computer allowed us to visualize and measure the cross sections of the extraocular muscles in a coronal section. Surgery was indicated to resolve strabismus. Outcome was considered favorable if the final deviation was < 10 pd in the primary position without head turn.

RESULTS

In 8 cases (6 males, 5 unilateral [3 left eye]), MRI revealed atrophy of at least 1 of the extraocular muscles supplied by the third nerve. Five patients had third nerve aplasia or hypoplasia. Clinical findings were compatible with a probable diagnosis of CFEOM in all 10 patients. Four patients underwent ptosis surgery before being diagnosed with CFEOM. Four patients underwent surgery to correct strabismus and, of these, 2 required multiple interventions (1 needed 4 interventions). Outcome was successful in only 2 cases.

CONCLUSION

Orbital and cranial MRI provided useful information about extraocular muscles and cranial nerves in CFEOM. Surgery must be performed on an individual basis; the number of reoperations is high. The outcome of surgery was favorable in half of the cases.