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Hydrocephalus Secondary to Spina Bifida

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Cerebrospinal Fluid Disorders

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Abstract

Hydrocephalus is the most consistent and significant neurologic sequelae of myelomeningocele (MM), tightly correlated with cognitive function and quality of life. While debated, the pathologic mechanism likely relates to CSF flow dynamics altered by foramen crowding from cerebellar tonsillar descent (Chiari II malformation). Hydrocephalus may develop and be detected prenatally, at the time of MM closure or weeks or months after the spinal defect repair. The mainstay of hydrocephalus treatment has been ventriculoperitoneal shunt (VPS) insertion, though evidence to support the efficacy of endoscopic third ventriculostomy (ETV) with or without choroid plexus cauterization (CPC) continues to grow. In a select subpopulation of patients treated at high volume centers, intrauterine spina bifida repair (IUSBR) has been shown to dramatically decrease the proportion of patients requiring permanent CSF diversion. As with other hydrocephalus variants, patients suffering from hydrocephalus secondary to MM are living longer and require long-term surveillance and management by a multidisciplinary team of surgical, neurologic, and physiatric specialists.

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Authors and Affiliations

  1. Vanderbilt University Medical Center, Monroe Carell Jr. Children’s Hospital at Vanderbilt, Department of Neurological Surgery, Nashville, TN, USA

    Michael C. Dewan

  2. Monroe Carell Jr. Children’s Hospital at Vanderbilt, Neurological Surgery, Division of Pediatric Neurosurgery, Nashville, TN, USA

    John C. Wellons III & Robert P. Naftel

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  1. Michael C. Dewan
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  2. John C. Wellons III
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  3. Robert P. Naftel
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Correspondence to Robert P. Naftel .

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Editors and Affiliations

  1. Department of Neurological Surgery, Washington University School of Medicine, St. Louis Children’s Hospital, St. Louis, MO, USA

    David D. Limbrick Jr.

  2. Nationwide Children’s Hospital, Department of Neurosurgery, The Ohio State University College of Medicine, Columbus, OH, USA

    Jeffrey R. Leonard

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Dewan, M.C., Wellons, J.C., Naftel, R.P. (2019). Hydrocephalus Secondary to Spina Bifida. In: Limbrick Jr., D., Leonard, J. (eds) Cerebrospinal Fluid Disorders . Springer, Cham. https://doi.org/10.1007/978-3-319-97928-1_10

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  • DOI: https://doi.org/10.1007/978-3-319-97928-1_10

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