Article
From the globe to the chiasm – combined extradural-intraorbital and intradural-intracranial resection of optic gliomas in children with NF1
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Authors
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Martin U. Schuhmann
- Sektion Pädiatrische Neurochirurgie, Klinik für Neurochirurgie, Universitätsklinikum Tübingen
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Toma Spiriev
- Sektion Pädiatrische Neurochirurgie, Klinik für Neurochirurgie, Universitätsklinikum Tübingen
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Dorothea Besch
- Sektion für Motilitätsstörungen, periokuläre Chirurgie und Kinderophthalmologie, Universitäts-Augenklinik Tübingen
| Published: | June 2, 2015 |
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Outline
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Objective: Optic gliomas are for the vast majority of cases no surgical tumors in NF1 due to their diffuse nature and involvement of optic nerve, chiasm and optic tract/hypothalamus. An exception are unilateral intraorbital to pre-chiasmatic tumors in case of blindness associated with increasing exophthalmus and orbital pain.
Method: We report on two girls aged 3 years 1 month and 4 years 11 months with unilateral large optic gliomas extending from the globe of the eye almost to the chiasm. Both exhibited the combination of blindness, increasing exophthamus and in one case excruciating pain. The presented video demonstrates both cases and didactically all steps of surgery.
Results: In both girls an extradural approach to the orbit via orbitotomy of the orbital roof was performed. The superior orbital fissure and the optic canal were extradurally exposed. The intraorbital tumor was resected from ist exit of the globe close to ist exit of optic canal. This was followed by intradural resection of the remaining tumor from the canal up to the chiasm. The optic canal was sealed with muscle to prevent CSF leak to the orbit. The orbital content and the orbital roof were fully reconstructed. Pain and exophthalmus were relieved. In both children the opthalmic artery and veins and the function of all orbital muscles was preserved with normal ocular movements after a recovery period. Both girls were discharged on day 6. One girl developed after 18 months a recurrence at the exit site of the optic nerve from the globe. A spread of the disease to the chiasma was prevented.
Conclusions: A combined interdisciplinary microsurgical intra-and extradural approach to unilateral optic nerve gliomas extending from the globe to the chiasm is possible when the combination of blindness, exophthalmus and pain indicates treatment. This can be achieved with preservation of all orbital function present at time of surgery and relieves exophthalmus and pain. Alternative treatment options would have been chemotherapy over 12-18 months known not to decrease the tumor volume dramatically and thus exophthalmus and pain would not have been directly addressed. Since resection at the optic nerve exit site of the globe has to be conservative to preserve the integrity of the eye ball, a local recurrence at this transition zone is possible.
