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Paediatric hydrocephalus

Nature Reviews Disease Primers volume 10, Article number: 35 (2024) Cite this article

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Abstract

Hydrocephalus is classically considered as a failure of cerebrospinal fluid (CSF) homeostasis that results in the active expansion of the cerebral ventricles. Infants with hydrocephalus can present with progressive increases in head circumference whereas older children often present with signs and symptoms of elevated intracranial pressure. Congenital hydrocephalus is present at or near birth and some cases have been linked to gene mutations that disrupt brain morphogenesis and alter the biomechanics of the CSF–brain interface. Acquired hydrocephalus can develop at any time after birth, is often caused by central nervous system infection or haemorrhage and has been associated with blockage of CSF pathways and inflammation-dependent dysregulation of CSF secretion and clearance. Treatments for hydrocephalus mainly include surgical CSF shunting or endoscopic third ventriculostomy with or without choroid plexus cauterization. In utero treatment of fetal hydrocephalus is possible via surgical closure of associated neural tube defects. Long-term outcomes for children with hydrocephalus vary widely and depend on intrinsic (genetic) and extrinsic factors. Advances in genomics, brain imaging and other technologies are beginning to refine the definition of hydrocephalus, increase precision of prognostication and identify nonsurgical treatment strategies.

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Fig. 1: The cerebral ventricular system and cerebrospinal fluid homeostasis.
Fig. 2: Clustering of post-infectious hydrocephalus cases in Uganda.
Fig. 3: Convergence of cellular and molecular pathogenesis in post-haemorrhagic and post-infectious hydrocephalus models.
Fig. 4: Fetal ultrasonography images.
Fig. 5: Primary signs and symptoms of paediatric hydrocephalus by age group and aetiology.

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Acknowledgements

The authors thank the Hydrocephalus Association, Rudi Schulte Research Institute, NIH and patients with hydrocephalus and their families who have participated in research efforts. A.V.K. is supported by NIH 5U01NS107486-04 and NIH 5R01HD085853-10. K.T.K. is supported by the Rudi Schulte Research Institute, NIH 7R01NS109358-05 and NIH 5R01NS111029-04. J.M.S. is supported by the Rudi Schulte Research Institute and NIH 5R01NS110793-05. N.M. is supported by Novo Nordisk Foundation Tandem grant NNF17OC0024718. S.J.S. is supported by NIH Director’s Transformative Award R01AI145057, NIH 5R01HD085853-07, NIH 1U01NS107486, the NeuroKids foundation and the Ruddy Lifesaving Fund. S.R. is supported by the Rudi Schulte Research Institute, NIH 5R01HD104673-02 and DOD HT94252310296.

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Authors and Affiliations

  1. Department of Neurosurgery, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Kristopher T. Kahle & Jenna E. Koschnitzky

  2. Broad Institute of Harvard and MIT, Cambridge, MA, USA

    Kristopher T. Kahle

  3. Department of Neurosurgery and Division of Genetics and Genomics, Boston Children’s Hospital, Boston, MA, USA

    Kristopher T. Kahle

  4. Department of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical School of Brown University, Providence, RI, USA

    Petra M. Klinge

  5. Division of Paediatric Neurosurgery, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada

    Abhaya V. Kulkarni

  6. Department of Neuroscience, University of Copenhagen, Copenhagen, Denmark

    Nanna MacAulay

  7. Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Shenandoah Robinson

  8. Department of Paediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Shenandoah Robinson

  9. Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Shenandoah Robinson

  10. Department of Neurosurgery, Yale University, New Haven, CT, USA

    Steven J. Schiff

  11. Department of Epidemiology of Microbial Diseases, Yale University, New Haven, CT, USA

    Steven J. Schiff

  12. Department of Neurosurgery, Washington University School of Medicine, Saint Louis, MO, USA

    Jennifer M. Strahle

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J.E.K. is an independent contractor for Rhaeos, Inc., a start-up company that is developing a noninvasive, wireless and wearable shunt flow sensor, currently in clinical trials. All other authors declare no competing interests.

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Kahle, K.T., Klinge, P.M., Koschnitzky, J.E. et al. Paediatric hydrocephalus. Nat Rev Dis Primers 10, 35 (2024). https://doi.org/10.1038/s41572-024-00519-9

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