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Undiagnosed sagittal synostosis as cause of “idiopathic” intracranial hypertension

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Abstract

Purpose

Idiopathic intracranial hypertension (IIH) is a rare condition in children, but if diagnosed needs to be promptly treated to avoid clinical sequalae. The main purpose of this paper was to test our clinical experience with a cohort of normocephalic children with craniosynostosis who do not present in the routine way to craniofacial services, due to the normal head shape and age, diagnosed with IIH.

Methods

We retrospectively reviewed all children who were referred to neurosurgery from 2012 to 2022 for management of IIH on our prospectively kept database. We determined what treatments were offered and if there was an associated craniosynostosis.

Results

In total, 19 children were identified with an average age at referral of 11.5 years (st dev 4.0 years) with 11 male and 8 female. The most common presenting symptoms and signs were papilloedema (18/19), headaches (15/19), visual deterioration (9/19), nausea and vomiting (7/19) and diplopia (4/19). Five out of 19 children (26.3%) had a sagittal suture fused that was not identified at the time of treatment and all children were normocephalic.

Conclusion

There is a cohort of children with IIH who will have concomitant craniosynostosis and ideally would benefit from cranial vault expansion as primary surgery rather than cerebrospinal fluid (CSF) diversion. We suggest all children with IIH requiring neurosurgical intervention have cross-sectional imaging to look for occult craniosynostosis prior to intervention.

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Data availability

No datasets were generated or analyzed during the current study.

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Funding

No funding was obtained for this study.

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Authors and Affiliations

  1. Department of Paediatric Neurosurgery, Birmingham Women’s and Children’s NHS Foundation Trust, Birmingham, UK

    Joshua Pepper & Pasquale Gallo

  2. Medical School, University of Birmingham, Birmingham, UK

    Saloni Bhattacharyya

  3. Supra-Regional Craniofacial National Centre, Birmingham Women’s and Children’s NHS Foundation Trust, Birmingham, UK

    Pasquale Gallo

Authors
  1. Joshua Pepper
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  2. Saloni Bhattacharyya
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  3. Pasquale Gallo
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Contributions

J.P. drafted the manuscript and prepared Table 1, Figs. 1 and 4. S.B. helped with data collection and analysis, P.G. designed the study and contributed to the manuscript drafting. Prepared Figs. 2 and 3. Revised the manuscript. All authors reviewed the final rmanuscript.

Corresponding author

Correspondence to Pasquale Gallo.

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Pepper, J., Bhattacharyya, S. & Gallo, P. Undiagnosed sagittal synostosis as cause of “idiopathic” intracranial hypertension. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06308-9

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