Spontaneous globe luxation in iatrogenic Cushing syndrome

We report a rare case of spontaneous eyeball luxation associated with exophthalmos due to iatrogenic Cushing syndrome (CS). The normalization of serum hormones led to the regression of the picture.

A 64-year-old man presented with spontaneous globe luxation of the left eye after a 6-month history of bilateral, painless, and slowly progressive exophthalmos. The patient had been receiving weekly infusions of methylprednisone over the previous 6 months. His best-corrected visual acuity (BCVA) at presentation was 20/40 in the right eye and 20/20 in the left eye. The patient demonstrated full extraocular motility. The intraocular pressure (IOP) was elevated in the right eye (24 mHg) and normal in the left eye (18 mmHg). Exophthalmometry demonstrated bilateral proptosis with measurements of 27 mm in the right eye and 28 mm in the left eye. Computed tomography scan of the brain and orbits revealed increased orbital and cervical fat. Clinical, radiographic and serologic findings ruled out potential diagnoses including orbital metastasis, thyroid orbitopathy, carotid-cavernous fistula, and idiopathic orbital pseudotumor. Clinical suspicion of iatrogenic CS was high, and additional serologic testing confirmed the diagnosis.

Exophthalmos is an uncommon sign of CS, but to our knowledge, this is the first reported case of spontaneous globe luxation secondary to CS. In our case, normalization of cortisol was sufficient to resolve the clinical symptoms and eliminated the need for surgical intervention such as orbital decompression surgery.