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Cranial expansion and aqueductoplasty for combined isolated fourth ventricle and slit-ventricle syndrome: a surgical alternative

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Abstract

Introduction

An isolated fourth ventricle (IFV) is a rare entity observed in shunted patients and its treatment is still uncertain. Endoscopic aqueductoplasty has shown good results for restoring CSF flux between the third and fourth ventricles. However, it needs some grade of ventricular dilation to be performed. Some patients affected by IFV show slit-ventricle morphology in CT/MRI. Usually, the rise of opening pressure or the shunt externalization gets enough ventricular dilation. However, the lack of intracranial compliance in some patients makes these options unsuitable and high-ICP symptoms are developed without ventricular dilation.

Methods

We present a two cases series affected by IFV with no ventricular dilation in radiological exams. ICP sensors were implanted, observing high-ICP and establishing the diagnosis of craniocerebral disproportion. A two-stage surgical plan based on a dynamic cranial expansion followed by a supratentorial endoscopic aqueductoplasty was performed. A physical and mathematical model explaining our approach was also provided.

Results

Chess-table cranial expansion technique was performed in both patients. Six/seven days after the first surgery, respectively, ventricular dilation was observed in CT. Endoscopic precoronal aqueductoplasty was then performed. No postoperative complications were described. IFV symptoms improved in both patients. Eighteen and 12 months after the two-stage surgical plan, the patients remain symptom-free and void of flow is still observed between the third and the fourth ventricles in MRI.

Conclusion

The two-stage approach was a suitable option for the treatment of these complex patients affected by both craniocerebral disproportion and isolated fourth ventricle.

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Abbreviations

IFV:

Isolated fourth ventricle

CSF:

Cerebrospinal fluid

VPS:

Ventriculoperitoneal shunt

CCD:

Craniocerebral disproportion

ICP:

Intracranial pressure

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Authors and Affiliations

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

    Jorge Tirado-Caballero, Mónica Rivero-Garvia, Gloria Moreno-Madueño & Javier Márquez-Rivas

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

    Jorge Tirado-Caballero, Mónica Rivero-Garvia & Javier Márquez-Rivas

  3. Group of Interdisciplinary Physics, Engineering School, Universidad de Sevilla, Seville, Spain

    Emilio Gómez-González

  4. Advanced Neurology Center, Seville, Spain

    Javier Márquez-Rivas

Authors
  1. Jorge Tirado-Caballero
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  2. Mónica Rivero-Garvia
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  4. Emilio Gómez-González
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Correspondence to Jorge Tirado-Caballero.

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Tirado-Caballero, J., Rivero-Garvia, M., Moreno-Madueño, G. et al. Cranial expansion and aqueductoplasty for combined isolated fourth ventricle and slit-ventricle syndrome: a surgical alternative. Childs Nerv Syst 37, 885–894 (2021). https://doi.org/10.1007/s00381-020-04939-2

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