New Ophthalmoplegia and Recurrent Ptosis After Ptosis Repair
Get authentic, real-time news that helps you fight COVID-19 better.
Install PlexusMD App for doctors. It's free.
The present case has been published in JAMA.

A man in his 60s with a history of surgery to repair right upper eyelid ptosis 1 year prior presented to a neuro-ophthalmology clinic with limited movement of the right eye of 1 year’s duration and worsening recurrent ptosis of the same eye. Results of previous single-fiber electromyography and serologic testing for acetylcholine receptor–binding antibodies were negative for myasthenia gravis.

His ocular history included amblyopia and nonarteritic ischemic optic neuropathy of the left eye. His medical history included obstructive sleep apnea, coronary artery disease, coronary artery bypass surgery, and prostate cancer, the last in remission for the past 4 years.

Visual acuities were 20/30 OD and hand motions OS. The patient had an Adie pupil in his right eye and a relative afferent pupillary defect in the left eye. The right eye demonstrated total ophthalmoplegia, and no deficit was found in the left.

Exophthalmometer measurements demonstrated enophthalmos of the right eye associated with facial asymmetry. Margin reflex distance was 0 mm in the right eye and 4 mm in the left. In-office ice and sleep testing did not improve the ptosis or ophthalmoplegia in the right eye. The remainder of his examination was remarkable for punctate epithelial erosions of the right eye and optic atrophy of the left.

MRI demonstrated atrophic optic chiasm and optic nerves bilaterally, right-sided extraocularmuscle atrophy, and right-sided facial hemiatrophy.

Final Dx: Parry-Romberg syndrome

Parry-Romberg syndrome is a rare degenerative disorder of unknown etiology characterized by progressive facial hemiatrophy. The disease process typically begins with fibrosis and atrophy of facial skin and subcutaneous tissue, with progression to underlying muscles, bone, and cartilage. In the natural course, the disease stabilizes after 2 to 20 years. Although classically unilateral, the disease can affect bilateral structures and is sometimes referred to as Barraquer-Simons syndrome.

Read more here: