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Neuronavigated endoscopic aqueductoplasty with panventricular stent plus septostomy for isolated fourth ventricle in complex hydrocephalus and syringomyelia associated with myelomeningocele: how I do it

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Abstract

Background

Isolated fourth ventricle (IFV) is a challenging entity to manage. In recent years, endoscopic treatment for aqueductoplasty has been on the rise. However, in patients with complex hydrocephalus and distorted ventricular system, its implementation can be complex.

Methods

We present a 3-year-old patient with myelomeningocele and postnatal hydrocephalus treated by ventriculoperitoneal shunt. In follow-up, a progressive IFV and isolated lateral ventricle with symptoms of the posterior fossa developed. An endoscopic aqueductoplasty (EA) with panventricular stent plus septostomy guided with neuronavigation was decided due to the complexity of the ventricular system.

Conclusion

In IFV associated with complex hydrocephalus with distortion of the ventricular system, navigation can be of great help for planning and as a guide for performing EA.

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Abbreviations

IFV :

Isolated fourth ventricle

EA :

Endoscopic aqueductoplasty

CT :

Computer tomography

MRI :

Magnetic resonance imaging

EVD :

External ventricular drain

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Authors and Affiliations

  1. Department of Neurosurgery, Torrecárdenas University Hospital, Calle Hermandad Donantes de Sangre s, /n 04009, Almería, Spain

    José Javier Guil-Ibáñez, Mario Gomar-Alba, José María Narro-Donate & José Masegosa-González

  2. Department of Health Science, University of Almería, Almería, Spain

    José Javier Guil-Ibáñez & Tesifón Parrón-Carreño

Authors
  1. José Javier Guil-Ibáñez
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  2. Tesifón Parrón-Carreño
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  3. Mario Gomar-Alba
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  4. José María Narro-Donate
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  5. José Masegosa-González
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Corresponding author

Correspondence to José Javier Guil-Ibáñez.

Ethics declarations

Ethics approval

All procedures performed in the studies involving human participants were in accordance with ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standard.

Consent to participate and consent for publication

Informed consent to participate and consent for publication was obtained from the patient included in this report.

Conflict of interest

The authors declare no competing interests.

Additional information

Key points

Determine the indications for surgery. Patients with clinical symptoms should undergo surgery. In cases where the patient is asymptomatic but has radiological progression, risk/benefit assessment is necessary.

Establish the approach route. If an endoscopic aqueductoplasty is being considered, the patient must have a ventricular size large enough to accommodate the system. Otherwise, alternatives such as EVD placement or cranial expansion may be considered.

Study preoperative images and develop a surgical plan by identifying limiting structures and the best surgical route.

In complex anatomies, consider the use of neuronavigation.

Explain to the patient and family the possibility of transient neurological alterations, particularly in eye mobility.

Knowledge of ventricular anatomy and experience in intraventricular endoscopy are crucial to obtain good outcomes.

The use of stents appears to show a lower rate of restenosis in the literature.

A proper technique for closing the cortical-subcortical tract is ideal to avoid the risk of cerebrospinal fluid leak.

Periodic follow-up with MRI is recommended for patients. We have protocolized the performance of a control magnetic resonance imaging before discharge, another 1 month after the procedure, at 3 months, 6 months, and then annually. During the interpretation of these images, we investigate possible signs of system failure (e.g., signs of hyperdrainage) and combine it with the information provided by the parents about the patient’s neurological (cognitive, school, motor, etc.) evolution.

In patients with shunts and a history of obstructive hydrocephalus, consideration should be given to endoscopic third ventriculostomy to remove the ventricular shunt.

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Guil-Ibáñez, J.J., Parrón-Carreño, T., Gomar-Alba, M. et al. Neuronavigated endoscopic aqueductoplasty with panventricular stent plus septostomy for isolated fourth ventricle in complex hydrocephalus and syringomyelia associated with myelomeningocele: how I do it. Acta Neurochir 165, 2333–2338 (2023). https://doi.org/10.1007/s00701-023-05649-9

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