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Ocular motor dysfunction in Parry-Romberg syndrome: four cases.

Christina R Prescott¹, M Joshua Hasbani, Andrew J Levada

¹Department of Ophthalmology and Visual Science, Yale University School of Medicine, New Haven, Connecticut, USA. christina.prescott@gmail.com

Journal of Pediatric Ophthalmology and Strabismus

|December 14, 2011

Summary

Parry-Romberg syndrome (PRS) can cause varied eye movement issues and facial changes, often delaying diagnosis. Muscle fibrosis, not nerve damage, likely explains these complex ophthalmic symptoms in PRS patients.

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Area of Science:

Ophthalmology
Neurology
Genetics

Background:

Parry-Romberg syndrome (PRS) is a rare disorder characterized by progressive hemifacial atrophy.
Ophthalmic manifestations in PRS are diverse and can complicate diagnosis.

Observation:

Four patients with PRS presented with distinct ophthalmic abnormalities, including abnormal eye movements, ptosis, and facial hemiatrophy.
Diagnostic delays were common, necessitating extensive imaging before PRS was identified.
Specific cases involved lateral rectus paresis, medial rectus weakness, dry eye, hemifacial atrophy, enophthalmos, and restricted up gaze.

Findings:

The ocular motor defects observed in these patients are best attributed to muscle fibrosis.
The variability of ophthalmic signs underscores the diagnostic challenges associated with PRS.

Implications:

Recognizing the spectrum of ophthalmic findings is crucial for timely PRS diagnosis.
Understanding the role of muscle fibrosis may guide future therapeutic strategies for PRS-related ocular motility disorders.