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Bilateral chiasm and optic nerve micro-decompression in a child with extensive cranial fibrous dysplasia: how I do it

  • How I Do it - Neurosurgical technique evaluation
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Abstract

Background

Surgical decompression to the optic-chiasmatic region in craneofacial fibrous dysplasia (CFD) must be performed safely to improve or stabilize visual loss.

Method

We describe a technical nuance when facing on a huge, deformed skull with potentially imbricated dura mater. Craniectomy was performed in concentric arches allowing to expose surgical field and elevated step by step. Bilateral micro-decompression was performed after without difficulties.

Conclusions

Decompressing both optic nerves using this technique is safe and relatively simple to perform.

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Abbreviations

FD:

Fibrous dysplasia

CFD:

Craneofacial fibrous dysplasia

ON:

Optic nerve

LE:

Left eye

RE:

Right eye

VA:

Visual acuity

MRI:

Magnetic resonance imaging

CT:

Computer tomography

References

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Acknowledgements

We are grateful to Dr. Ariel Kaen for helping us with the video production, to Drs. Jorge Herrería and Cristina Romero for reviewing and editing, and to all authors for providing information, picture editing and text reviewing.

Author information

Authors and Affiliations

  1. Department of Pediatric Neurosurgery, Virgen del Rocío University Hospital, Av. Manuel Siurot, S/N, 41013, Seville, Spain

    Carlos Butrón-Díaz, Mónica Rivero-Garvia & Javier Márquez-Rivas

  2. Group of Applied Neuroscience, Biomedicine Institute of Seville, Seville, Spain

    Mónica Rivero-Garvia & Javier Márquez-Rivas

  3. Advanced Neurology Center, Seville, Spain

    Javier Márquez-Rivas

Authors
  1. Carlos Butrón-Díaz
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  2. Mónica Rivero-Garvia
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  3. Javier Márquez-Rivas
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Corresponding author

Correspondence to Carlos Butrón-Díaz.

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Key points

1. Preoperative study by MRI. Evaluate compromised structures, show the affectation of the superior orbital fissure, and look for atrophy of the ONs.

2. Do not operate patients with CFD without visual symptoms. Decompressing the optic canal has severe risks and is not indicated prophylactically.

3. Craniectomy in concentric arches. Removing the deformed bone in parts helps the surgeon to preserve the dura and easily pass the drills with direct control. (Figs. 3, 4).

4. Be aggressive. Wide resections are related to good cosmetic and definitive results, with lower rates of recurrence.

5. Bleeding. Fibrotic bone bleeds more than normal do. It could be controlled with no special maneuver. Avoid using electrocautery and reserve blood beforehand.

6. Extensive irrigation. This is the key to prevent damage of ONs. Do it through the micro-decompression [1,2] (Video 1).

7. Reshape the skull. In extensive CFDs, intervention only to decompress the ON is not indicated. Treat the aesthetic problem.

8. Add corticosteroids. Short therapies have been related to improvement in the VA before and after surgery and even with nerve atrophy.

9. Stop visual impairment. In cases where the VA does not improve, stopping the progression is also a therapeutic objective.

10. Evaluate the benefit of surgery. It is not always indicated.

This article is part of the Topical Collection on Neurosurgical technique evaluation

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Butrón-Díaz, C., Rivero-Garvia, M. & Márquez-Rivas, J. Bilateral chiasm and optic nerve micro-decompression in a child with extensive cranial fibrous dysplasia: how I do it. Acta Neurochir 164, 1485–1491 (2022). https://doi.org/10.1007/s00701-021-05036-2

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  • DOI: https://doi.org/10.1007/s00701-021-05036-2

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