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Adult long-term outcome of patients after congenital hydrocephalus shunt therapy

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Abstract

Introduction

Even though shunt surgery has been an established and widely accepted treatment for congenital hydrocephalus for five decades, long-term follow-up and functional outcome data is rare.

Patients and methods

Sixty-nine patients were examined after congenital hydrocephalus had been treated between 1971 and 1987 at the Department of Pediatric Surgery (University of Leipzig) within the first 12 months of life. Median age of patients was 32 years (range 25–42 years). Patients agreed to undergo a contemporary hydrocephalus assessment.

Results

Seven patients (10.1 %) became shunt-independent before adulthood. By the age of 20, 82 % of the patients had needed at least one shunt revision, 100 % by the age of 30. 21.7 % of the primary valves (Spitz-Holter) remained intact without revision until today up to 35 years (mean functional intactness 23 years). Shunt infections occurred in 4.3 % of primary implantations.

48 % of the patients had a good functional outcome (mRS = 0–1). 40.9 % of the patients attended regular school. In summary, 75 % patients work on a daily basis. 44.6 % live an independent life, 35.4 % rely on parental supervision, and 20 % depend on professional care institutions.

Conclusion

Compared to the pre-shunt era, surgery within the first year of life is advantageous regarding visual function, educational progress, and social results. The outcome achieved throughout childhood remains stable during adult life as long as catastrophic events of shunt malfunction can be prevented.

Epilepsy, motor deficits, acute shunt dysfunction, and problems of social integration as well as aging parental caregivers seem to be prominent factors of morbidity in adulthood.

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Acknowledgments

We acknowledge the huge and highly esteemed contribution of the pediatric surgical staff and surgeons, who diagnosed, treated, and operated the many children under very different technical circumstances at their time at University Leipzig, compared to today. Many of them are only known to us by their thorough and detailed history taking and archiving of the medical records, in this way enabling us to analyze the long-term results.

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Authors and Affiliations

  1. Department of Neurosurgery, Pediatric Neurosurgery, University Hospital Leipzig, Liebigstrasse 20, 04103, Leipzig, Germany

    M. Preuss, A. Kutscher, J. Meixensberger & U. Nestler

  2. Department of Pediatric Surgery, University Hospital Leipzig, Leipzig, Germany

    R. Wachowiak

  3. Division of Neuropediatrics, University Hospital Leipzig, Leipzig, Germany

    A. Merkenschlager & M. K. Bernhard

  4. Division of Neuroradiology, University Hospital Leipzig, Leipzig, Germany

    M. Reiss-Zimmermann

Authors
  1. M. Preuss
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  2. A. Kutscher
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  3. R. Wachowiak
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  4. A. Merkenschlager
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  5. M. K. Bernhard
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  6. M. Reiss-Zimmermann
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  7. J. Meixensberger
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  8. U. Nestler
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Correspondence to M. Preuss.

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Preuss, M., Kutscher, A., Wachowiak, R. et al. Adult long-term outcome of patients after congenital hydrocephalus shunt therapy. Childs Nerv Syst 31, 49–56 (2015). https://doi.org/10.1007/s00381-014-2571-8

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  • DOI: https://doi.org/10.1007/s00381-014-2571-8

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