Hydrocephalus in the Child with Cerebral Palsy

  • Jeffrey CampbellEmail author
Living reference work entry


Approximately 15% of children with cerebral palsy (CP) have concomitant hydrocephalus, which can be both a cause of CP and a result of disorders that independently lead to CP. While CP by definition is a static neurologic deficit, hydrocephalus and its treatment is a dynamic process that can create new disability or even death throughout the life span. It is vital for the provider to recognize hydrocephalus that benefits from treatment and be able to offer the full range of treatment options, including shunts and endoscopic third ventriculostomies. Unfortunately, even with the best of care, current treatments of hydrocephalus are fallible, and many patients live under the constant threat of new neurological injury from a malfunctioning shunt.


Hydrocephalus Shunt Endoscopic third ventriculostomy Ventricle Cerebrospinal fluid Aqueductal stenosis Dandy Walker malformation Intraventrentricular hemorrhage of prematurity Meningitis 


  1. Al-Jolou W, Yew A, Boomsaad Z, Garton H, Muraszko K, Maher C (2010) Prevalence and natural history of arachnoid cysts in children. J Neurosurg Pediatr 5:578–585PubMedGoogle Scholar
  2. Arrington C, Ware AL, Ahmed Y, Kulesz PA, Dennis M, Fletcher JM (2016) Are shunt revisions associated with IQ in congenital hydrocephalus? A meta-analysis. Neuropsychol Rev 26:329–339CrossRefGoogle Scholar
  3. Ballabh P (2010) Intraventricular hemorrhage in premature infants: mechanism of disease. Pediatr Res 67:1–8CrossRefGoogle Scholar
  4. Benda C (1954) The Dandy-Walker syndrome or the so-called atresia of the foramen of Magendie. J Neuropathol Exp Neurol 13:14–29Google Scholar
  5. Beuriat P-A, Puget S, Cinalli G, Blauwblomme T, Beccaria K, Zerah M, Sainte-Rose C (2017) Hydrocephalus treatment in children: long-term outcome in 975 consecutive patients. J Neurosurg Pediatr 20:10–18PubMedGoogle Scholar
  6. Bouras T, Sgouros S (2012) Complications of endoscopic third ventriculostomy: a systematic review. In: Aygok G, Rekate H (eds) Hydrocephalus. Acta neurochirurgica supplementum, vol 113. Springer, Vienna, pp 149–153Google Scholar
  7. Castro-Cago M, Alonso A, Eiris-Punal J (1996) Autosomal recessive hydrocephalus with aqueductal stenosis. Childs Nerv Syst 12:188–191CrossRefGoogle Scholar
  8. Duncan C, Change V (2008) Intraventricular hemorrhage and posthemorrhagic hydrocephalus. In: Albright A, Pollack I, Adelson P (eds) Pediatric neurosurgery, 2nd edn. Thieme Medical Publishers, Inc, New York, pp 145–161Google Scholar
  9. Edwards J (1961) The syndrome of sex-linked hydrocephalus. Arch Dis Child 36:486–493CrossRefGoogle Scholar
  10. Hahn JS, Plawner LL (2004) Evaluation and management of children with holoprosencephaly. Pediatr Neurol 31:79–88CrossRefGoogle Scholar
  11. Hirsch J, Hirsch E, Sainte Rose C, Renier D, Pierre-Khan A (1986) Stenosis of the aqueduct of Sylvius. Etiology and treatment. J Neurosurg Sci 30:29–39Google Scholar
  12. Jackson J (2010) Neuropsychological symptoms associated with multiple shunts. Loma Linda University Electronic theses, dissertations & projects, Loma LindaGoogle Scholar
  13. Kulkarni AV, Drake JM, Kestle JR, Mallucci CL, Sgouros S, Constantini S (2010) Predicting who will benefit from endoscopic third ventriculostomy compared with shunt insertion in childhood hydrocephalus using the ETV Success Score. J Neurosurg Pediatr 6:310–315PubMedGoogle Scholar
  14. Kulkarni A, Schiff SJ, Mbabazi-Kabachelor E, Mugamba J, Ssenyonga P, Donnelly R, … Warf BC (2017) Endoscopic treatment versus shunting for infant hydrocephalus in Uganda. N Engl J Med 377:2456–2464CrossRefGoogle Scholar
  15. Levy M, Masri L, McComb J (1997) Outcome for preterm infants with germinal matrix hemorrhage and progressive hydrocephalus. Neurosurgery 41:1111–1117CrossRefGoogle Scholar
  16. Marin R, Ley-Martos M, Gutierrez G, Rodriguez-Sanchez F, Arroyo D, Mora-Lopez F (2015) Three cases with L1 syndrome and two novel mutations in the L1CAM gene. Eur J Pediatr 174:1541–1544CrossRefGoogle Scholar
  17. Mohanty A, Biswas A, Satish S, Praharaj S, Sastry K (2006) Treatment options for Dandy-Walker malformation. J Neurosurg 105(5 Suppl):348–356Google Scholar
  18. Murphy B, Inder T, Rooks V, Taylor G, Anderson N, Mogridge N, … Volpe J (2002) Posthaemorrhagic ventricular dilatation in the premature infant: natural history and predictors of outcome. Arch Dis Child Fetal Neonatal Ed 87:F37–F41CrossRefGoogle Scholar
  19. Ozaras N, Yalcin S, Ofluoglu D, Gureri B, Cabukoglu C, Erol B (2005) Are some cases of spina bifida combined with cerebral palsy? A study of 28 cases. Eura Medicophys 41:239–242Google Scholar
  20. Paulsen AH, Lundar T, Lindegaard K-F (2015) Pediatric hydrocephalus: 40-year outcomes in 128 hydrocephalic patients treated with shunts during childhood. Assessment of surgical outcome, work participation, and health-related quality of life. J Neurosurg Pediatr 16:633–641PubMedGoogle Scholar
  21. Raam MS, Muenke M, Solomon BD (2011) Holoprosencephaly: a guide to diagnosis and clinical management. Indian Pediatr 48:457–466CrossRefGoogle Scholar
  22. Senat M, Bernard J, Delezoide A, Saugier-Veber P, Hillion Y, Roume J, Ville Y (2001) Prenatal diagnosis of hydrocephalus-stenosis of the aqueduct of Sylvius by ultrasound in the first trimester of pregnancy. Report of two cases. Prenat Diagn 21:1129–1132CrossRefGoogle Scholar
  23. Simon E, Hevner R, Pinter J, Clegg N, Delgado M, Kinsman S, … Barkovich A (2001) The dorsal cyst in holoprosencephaly and the role of the thalamus in its formation. Neuroradiology 43:787–791CrossRefGoogle Scholar
  24. Simon TD, Hall M, Riva-Cambrin J, Albert JE, Jeffries HE, LaFleur B, … Kestle JR (2009) Infection rates following initial cerebrospinal fluid shunt placement across pediatric hospitals in the United States. J Neurosurg Pediatr 4:156–165PubMedGoogle Scholar
  25. Stoll BJ, Hansen NI, Bell EF, Shankaran S, Laptook AR, Walsh MC, … Higgins RD (2010) Neonatal outcomes of extremely preterm infants from the NICHD Neonatal Research Network. Pediatrics 126:443–456Google Scholar
  26. van Haastert I, Groenendaal F, Uiterwaal C, Termote J, van der Heide-Jalving M, Eijsermans M, … de Vries L (2011) Decreasing incidence and severity of cerebral palsy in prematurely born children. J Pediatr 159:86–91Google Scholar
  27. Westermaier T, Schweitzer T, Ernestus R-I (2012) Arachnoid cysts. Adv Exp Med Biol 724:37–50Google Scholar
  28. Yasuda T, Tomita T, McClone DM, Donovon M (2002) Measurement of cerebrospinal fluid output through external ventricular drainage in one hundred infants and children: correlation with cerebrospinal fluid production. Pediatr Neurosurg 36:22–28CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Nemours/A.I. duPont Hospital for ChildrenWilmingtonUSA

Section editors and affiliations

  • Steven J. Bachrach
    • 1
    • 2
  1. 1.Department of Pediatrics (Emeritus)Nemours/Alfred I. duPont Hospital for ChildrenWilmingtonUSA
  2. 2.Sidney Kimmel Medical College of Thomas Jefferson UniversityPhiladelphiaUSA

Personalised recommendations