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Optochiasmatic Cavernomas: Updated systematic review and proposal of a novel classification with surgical approaches

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Abstract

Cavernomas are histologically benign vascular malformations found at different sites in the brain. A rare site for such cavernomas, however, is the anterior optic pathway, comprising the optic nerve, chiasma, and optic tract—called optochiasmatic cavernomas (OCC). These lesions usually present with sudden onset or progressive vision loss, headache, and features mimicking pituitary apoplexy. In this paper, we describe a case of OCC operated at our center. We carry out an updated review of literature depicting cases of OCC, their clinical presentation, management, and postoperative complications. We also propose a novel classification system based on lesion location and further analyze these cavernoma types with respect to the surgical approach used and visual outcome. A 30-year-old lady had presented with a 3-week history of progressive bilateral vision loss and headache. Based on imaging, she was suspected to have a cavernous angioma of the chiasma and left optic tract. Due to progressive vision deterioration, the lesion was surgically excised using pterional craniotomy. Postoperatively, her visual symptoms improved, but she developed diabetes insipidus. Clinical and radiological follow-up has been done for 18 months after surgery. A total of 81 cases have been described in the literature, including the present case. Chiasmal apoplexy is the most common presentation. Surgical excision is the standard of care. Our analysis based on lesion location shows the most appropriate surgical approach to be used for each cavernoma type. Visual outcome correlates with the preoperative visual status. Visual outcome is good in patients presenting with acute chiasmal apoplexy, and when complete surgical excision is performed. The endonasal endoscopic approach was found to provide the best visual outcome. In addition to preoperative visual status, complete surgical excision predicts favorable visual outcomes in OCC. Our proposed classification system guides the appropriate surgical approach required for a particular location of the cavernoma.

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Acknowledgements

The authors are grateful to Ms. Himanshi Bhati (medical student) for her help in preparing the illustrations.

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Authors and Affiliations

  1. Department of Neurosurgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan, 342005, India

    Jigish Ruparelia, Rajnish Patidar, Jaskaran Singh Gosal, Mayank Garg, Deepak Kumar Jha & Suryanarayanan Bhaskar

  2. Department of Pathology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, 342005, India

    Vikarn Vishwajeet

  3. Department of Radiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, 342005, India

    Sarbesh Tiwari

  4. Department of Anesthesiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, 342005, India

    Manbir Kaur

  5. Department of Pharmacology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, 342005, India

    Surjit Singh

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Contributions

Authors JR and JSG prepared the main manuscript text. Authors RP, MG, MK and SB were part of the operating team. Authors DKJ and SB checked the manuscript. Author ST reported the radiological images. Author VV reported the pathology slides. Author SS did the statistical analysis and checked the manuscript. All authors did a final review of the manuscript.

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Correspondence to Jaskaran Singh Gosal.

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Ruparelia, J., Patidar, R., Gosal, J.S. et al. Optochiasmatic Cavernomas: Updated systematic review and proposal of a novel classification with surgical approaches. Neurosurg Rev 47, 53 (2024). https://doi.org/10.1007/s10143-024-02288-1

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