Recurrent Orbital Apex Syndrome Presenting with Rapid Vision Loss and Ophthalmoplegia; a Case Report

doi:10.21203/rs.3.rs-4848421/v1

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Case Report

Recurrent Orbital Apex Syndrome Presenting with Rapid Vision Loss and Ophthalmoplegia; a Case Report

https://doi.org/10.21203/rs.3.rs-4848421/v1

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Background.

Orbital Apex Syndrome (OAS) is a rare and potentially debilitating condition that causes sudden, painful vision loss. Despite its diverse causes, prompt diagnosis and early intervention are crucial for restoring vision and preventing long-term damage. This report presents a unique case of recurrent OAS, highlighting the importance of timely treatment and careful management.

Case presentation.

A 55-year-old woman presented with sudden, painful vision loss in her right eye, accompanied by ptosis and ophthalmoplegia, similar to a presentation she had experienced five years ago. Clinical examination revealed reduced visual acuity and painful restriction of right eye movements, while diagnostic tests showed a mildly bulky right optic nerve in the optic canal on MRI and prolonged P100 latency in the right eye on VEP. Based on these findings, an Orbital Apex Syndrome (OAS) diagnosis was made. She was treated with intravenous steroids and supportive care. Her vision was restored to normal, and her ptosis and painful eye movements were resolved. The patient was discharged with a tapering dose of oral steroids and remained symptom-free at follow-up.

Conclusion.

This case report highlights the importance of prompt diagnosis and treatment of Orbital Apex Syndrome (OAS), as timely intervention can significantly impact patient outcomes. The patient's presentation and response to treatment suggest that her OAS was likely caused by inflammatory factors, underscoring the need for swift and targeted management to prevent long-term vision loss and other complications.

Orbital Apex Syndrome

Orbital Apex Disorders

OAS

Sudden Vision Loss

Painful Vision Loss

Orbital apex syndrome (OAS) is one of the orbital apex disorders (OAD), characterized by symptoms and signs resulting from the involvement of various structures in the region of the orbital apex (Superior orbital fissure and Optic canal) by a disease process. Once considered as separate disorders, other OAD disorders, such as the Superior orbital fissure syndrome (SOFS) and the Cavernous sinus syndrome (CSS), can progress to or coexist with OAS.^[1] OAS, also known as Jacod syndrome, usually presents with rapid onset defective vision and painful eye movements. Other symptoms include proptosis, abnormal corneal reflex or sensations, relative afferent pupillary defect (RAPD), anisocoria, disc edema, and optic atrophy.^[2] A prompt diagnosis and treatment is critical for restoring vision in OAS. Even though there are previous reports of OAS from India, recurrent OAS is rare. Here, we describe a case of recurrent OAS presented with sudden vision loss and painful eye movements who recovered completely because of early diagnosis and treatment. We took the patient's informed consent and followed the CARE guidelines for reporting this case report.^[3]

On ophthalmological examination, moderate ptosis was present in the right eye. Her best corrected visual acuity (BCVA) was 6/18 (right eye) and 6/9 (left eye); the color vision was normal in both eyes. Ocular movements were minimally restricted, and she reported pain during the movements. The corneal and conjunctival reflexes and other anterior segment findings were normal, with no RAPD. Tonometry revealed a normal intra-ocular pressure of 11 and 13 millimetres of mercury in the right and left eyes, respectively. Dilated fundus examination was normal. We did a visual field chart using the Humphrey field analyzer (HFA), which showed an enlarged blind spot bilaterally.

A 55-year-old woman presented to the emergency department with sudden onset defective vision in the right eye for one-week duration. She also reported pain while moving the right eye, right-sided headache, and difficulty in opening the right eye for the last four days. There was no history of redness, discharge, watering, diplopia, fever, trauma, or ocular surgery. The patient had a similar presentation five years back with defective vision and headache, which improved after a short course of systemic steroids. She was on treatment for Type 2 Diabetes, which was poorly controlled, and her history did not reveal any significant family history. She was on Glimepiride 2mg and Sitagliptin 100mg for her diabetes at presentation.

We did magnetic resonance imaging (MRI) of the brain and the orbit, which showed a mildly bulky right optic nerve in the optic canal, suggesting possible optic nerve inflammation (Fig. 1). Visual evoked potential (VEP) revealed prolonged P100 latency in the right eye, suggesting delayed optic nerve conduction (Fig. 2). A routine blood examination and markers for infection and autoimmune pathology were done, and they were normal except for elevated HbA1C (12%). We did a neurology evaluation to rule out any other causes of the presentation. We made a provisional diagnosis of OAS based on the history, eye examination findings, MRI, and VEP.

We started her on systemic steroids after discussing the possible causes of her symptoms and after getting informed consent. Intravenous Methyl Prednisolone 1gm was given for three consecutive days, followed by oral steroids in tapering dose. Oral analgesics were given for the pain. She reported improved pain and headache after one day of treatment. Her BCVA improved to 6/9 in her right eye after two days, and the ptosis disappeared on the fourth day, after which she was discharged with oral medications.

Her BCVA at discharge was 6/9 bilaterally, and there was no ptosis or ophthalmoplegia, thus showing a complete recovery to baseline. Her vision remains the same, and she no longer reports similar symptoms at one-month follow-up.

OAD can present with overlapping and progressing symptoms due to the anatomical proximity of the structures in the orbital apex. The symptoms of OAS are primarily due to the optic nerve's involvement and can also involve the Oculomotor nerve, Trochlear nerve, Abducens nerve, and the first division of the trigeminal nerve. Various etiological mechanisms can lead to OAS, such as inflammatory, infectious, neoplastic, iatrogenic/traumatic, or vascular.^[1] A rapid onset and progression of symptoms and painful ophthalmoplegia are often associated with an inflammatory cause and usually respond dramatically to corticosteroids, and symptoms usually resolve in 48–72 hours.^[4] Our patient also had a similar presentation pointing to the inflammatory cause despite normal blood investigations. The history of similar clinical features also suggests possible inflammatory causes.

Distinguishing other OAD disorders from OAS can be challenging. The SOFS is caused by lesions anterior to the orbital apex and presents with cranial nerve involvement but generally spares the optic nerve. In CSS, along with features of OAS, the sympathetic fibers and the maxillary division of the trigeminal nerve can be involved. A careful eye examination, MRI, and VEP can help distinguish these disorders, and in our case, the VEP findings were suggestive of optic nerve involvement. In inflammatory causes, the MRI often shows thickening and edema of the optic nerve in the orbital canal, similar to our patient.^[5] Early diagnosis and management are important predictors of OAS prognosis, and involvement of the optic nerve can have a potential risk for long-term deficits. The chance of recurrence is higher in the case of OAS associated with Tolosa Hunt syndrome (THS), and there can be residual neurological deficits. OAS, secondary to malignancies, often have a poor prognosis.^[6]

Conclusion and Takeaway Points.

Our case points to the need for rapid diagnosis and treatment of OAS for complete visual recovery. A detailed history, eye examination, and neuroimaging are key in diagnosing OAS. Since the patient had a similar presentation in the past, she may be at risk for a recurrence; hence, close follow-up might be needed. She might benefit from a detailed evaluation of any identifiable and correctable cases for OAS.

Ethics approval and consent to participate:

Written consent was taken from the patient. No ethics approval was taken, as this is a case report.

Consent for publication.

Informed written consent was taken from the patient for publication.

Availability of data and materials.

All data generated or analyzed during this study are included in this published article

Competing interests.

The authors declare that they have no competing interests.

Funding.

We have not received any funding or financial support for this manuscript.

Authors Contribution.

JVS: concept, design, definition of intellectual content, literature search, data acquisition, first draft manuscript preparation, manuscript editing, MJ: Data acquisition, manuscript editing, reviewing, JTJ: Concept, Design, literature search, Manuscript editing, manuscript reviewing.

Acknowledgments.

We acknowledge the support and advice of Dr. Jose Kunnel Paul (Consultant Neurologist), Dr Jospaul Lukas (Consultant Radiologist), and the management of Marsleeva Medicity Palai.

Pal K, Maurya* RP, Kadir SMU, Nuruddin M, Kumar A, Singh VP et al. Orbital apex syndrome: A review. IP Int J Ocular Oncol Oculoplasty 8(4):229–36.
Badakere A, Patil-Chhablani P. Orbital Apex Syndrome: A Review. Eye Brain. 2019;11:63.
Riley DS, Barber MS, Kienle GS, Aronson JK, von Schoen-Angerer T, Tugwell P, et al. CARE guidelines for case reports: explanation and elaboration document. J Clin Epidemiol. 2017;89:218–35.
Yeh S, Foroozan R. Orbital apex syndrome. Curr Opin Ophthalmol. 2004;15(6):490.
Goyal P, Lee S, Gupta N, Kumar Y, Mangla M, Hooda K, et al. Orbital apex disorders: Imaging findings and management. Neuroradiol J. 2018;31(2):104.
Mohankumar A, Gurnani B. Orbital Apex Syndrome [Internet]. In: StatPearls [Internet]. StatPearls Publishing; 2023 [cited 2024 Jun 19]. https://www.ncbi.nlm.nih.gov/books/NBK592386/

No competing interests reported.

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Recurrent Orbital Apex Syndrome Presenting with Rapid Vision Loss and Ophthalmoplegia; a Case Report

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Abstract

Introduction.

Clinical Findings.

Patient Information.

Diagnostic Assessments.

Therapeutic Interventions.

Follow-up and Outcomes

Discussion.

Declarations

References

Additional Declarations

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