Management of esotropia: hypotropia in neurofibromatosis type 1 - simulating myopic strabismus fixus

A 28-year-old man presented with a progressive inward deviation of the left eye in the last 4 years. Examination revealed -3 abduction and elevation deficit in the left eye with 50 prism diopters (PD) esotropia and 12 PD of hypotropia. The patient had multiple fibromas on the forearms with pulsatile globe and was diagnosed as neurofibromatosis type 1. Myopic strabismus fixus was suspected. MRI revealed left temporal lobe herniation through a dysplastic sphenoid wing, compressing the posterior half of the superior rectus and lateral rectus muscles, resulting in an esotropia-hypotropia complex. Surgical treatment involved suture myopexy (Yokoyama's technique) of the left superior rectus and lateral rectus muscles with a 6.5 mm left medial rectus recession. Two months postoperatively, the patient had minimal residual esotropia and hypotropia. MRI orbits should always be performed in high myopes with strabismus to assess extraocular muscle pathways.