Abstract ID: 26-558
A Rare Etiology in a Patient Presenting with a Suspected Orbital Mass: Unilateral Exotropic Heavy Eye Syndrome
Author: Gonca Inci Base Hospital / Institution: Istanbul University Istanbul Faculty of Medicine, Department of Ophthalmology
Presentation Type: ePoster Presentation
Purpose
To present a rare and atypical clinical manifestation of unilateral Heavy Eye Syndrome (HES) characterized by exotropia and significant proptosis, which initially raised suspicion for an orbital space-occupying lesion in a patient with extreme axial myopia.
Methods
A 42-year-old male with a history of Hemophilia A was referred to our ocular oncology department for the evaluation of right-sided proptosis and chronic visual impairment. The clinical assessment included Hertel exophthalmometry, prism cover test, ocular motility evaluation, and optical biometry. Contrast-enhanced orbital Magnetic Resonance Imaging (MRI) was performed to rule out intraorbital mass. The right eye (OD) exhibited an extreme axial length of 34.55 mm, profound chorioretinal atrophy, and a posterior staphyloma. Visual acuity in the OD was limited to finger counting while lefte ye (OS) was 20/20 Snellen. Contrary to the classic esotropic “Myopic Strabismus Fixus” pattern, the orthoptic exam revealed a 5-degree exotropia and minimal hypotropia, accompanied by mild limitation in adduction, abduction, and elevation. MRI findings confirmed a superotemporal prolapse of the globe through the muscle cone, resulting in the nasal displacement of the superior rectus (SR) muscle. The absence of an orbital mass, combined with these characteristic radiological findings, established the diagnosis of an atypical, early, unilateral variant of HES. The atypical exotropic deviation was considered secondary to sensory loss from dense amblyopia and posterior segment pathologies.
Results
The patient was informed that the potential for visual recovery following cataract surgery would be limited due to extensive chorioretinal atrophy and profound amblyopia in the right eye and a conservative management was planned.
Conclusion
This case underscores that HES can present with a high degree of clinical variability, including unilateral proptosis and exotropia. Clinicians should maintain a suspicion for HES in the differential diagnosis of unilateral proptosis in highly myopic patients to avoid unnecessary invasive procedures.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| MEHMET SERHAT | MANGAN | Istanbul University Istanbul Faculty of Medicine, Department of Ophthalmology |
| NIHAN | AKSU CEYLAN | Istanbul University Istanbul Faculty of Medicine, Department of Ophthalmology |