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Orbital Apex Syndrome Secondary to Middle Cranial Fossa Meningocele: A Rare Pediatric Case
Konstantin Gushansky1, Mohamad Zaghal1
1Ophthalmology, Shaare Zedek Medical Center, Jerusalem, Israel.
Insights
Orbital apex syndrome (OAS) in a child was successfully treated. A rare middle cranial fossa meningocele compressing orbital structures was identified and surgically repaired, restoring vision and eye movement.
Area of Science:
- Neuro-ophthalmology
- Pediatric Neurology
- Radiology
Background:
- Orbital apex syndrome (OAS) is a rare condition affecting neurovascular structures.
- It can cause sight-threatening and potentially life-threatening complications.
- Prompt diagnosis and intervention are crucial for preserving vision.
Purpose of the Study:
- To report a pediatric case of OAS.
- To highlight a rare etiology: middle cranial fossa meningocele (MEC).
- To emphasize multidisciplinary management and outcomes.
Main Methods:
- Case report of a 10-year-old child with acute OAS symptoms.
- Diagnostic imaging (CT/MRI) to identify MEC and associated sinusitis/meningitis.
- Multidisciplinary team approach involving ophthalmology, neurology, ENT, and radiology.
- Treatment with intravenous antibiotics followed by surgical repair (cranial osteoplasty).
Main Results:
- The patient presented with proptosis, ophthalmoplegia, and optic disc edema.
- Imaging confirmed MEC extending into the orbital apex.
- Intravenous antibiotics led to rapid systemic and ocular improvement.
- Surgical repair resulted in normal visual acuity and largely recovered extraocular movements at follow-up.
Conclusions:
- Middle cranial fossa meningoceles are a rare but important cause of OAS.
- Early recognition and collaborative, multidisciplinary management are key.
- Successful outcomes are achievable with timely intervention and surgical repair.
Background:
Orbital apex syndrome (OAS) is a rare, sight-threatening condition caused by injury or compression of neurovascular structures in the orbital apex, which, depending on the underlying etiology, may even pose a life-threatening risk. Prompt recognition and intervention are essential to prevent permanent visual loss and systemic complications.
Case Presentation:
We report a 10-year-old child presenting with acute left-sided proptosis, eyelid edema, ophthalmoplegia, and optic disc edema, following a history of orbital cellulitis and meningitis in early childhood. Imaging revealed a middle cranial fossa meningocele (MEC) extending into the orbital apex, associated with sinusitis and purulent meningitis. Ophthalmic examination demonstrated severe restriction of left-eye movements, proptosis, and a relative afferent pupillary defect. A multidisciplinary team - including ophthalmology, neurology, ENT, and radiology - initiated urgent intravenous antibiotics, resulting in rapid systemic and ocular improvement. The patient subsequently underwent cranial osteoplasty to repair the skull base defect. At discharge and four-month follow-up, visual acuity was normal, extraocular movements had largely recovered, and imaging showed intact retinal nerve fiber layers.
Conclusion:
This case underscores the importance of early recognition and collaborative management in OAS. Middle cranial fossa meningoceles, though rare, should be considered among potential structural causes of orbital apex compression.
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