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Abilash Haridas, MD
Tadanori Tomita, MD
Section Editors
Marc C Patterson, MD, FRACP
Leonard E Weisman, MD
Deputy Editor
Carrie Armsby, MD, MPH


Hydrocephalus is a disorder in which an excessive amount of cerebrospinal fluid (CSF) accumulates within the cerebral ventricles and/or subarachnoid spaces, which are dilated [1,2].

In children, hydrocephalus is almost always associated with increased intracranial pressure (ICP). In most cases, this is caused by excess CSF accumulating in the cerebral ventricles due to disturbances of CSF circulation (known as obstructive or non-communicating hydrocephalus). Less often, the CSF accumulates because of impaired absorption (known as communicating hydrocephalus). These types of hydrocephalus will be the focus of this topic review.

By contrast, in normal pressure hydrocephalus, the cerebral ventricles are pathologically enlarged, but the ICP is within the normal range. This condition is usually caused by impaired CSF absorption. This type of hydrocephalus is more often seen in adults and is discussed separately. (See "Normal pressure hydrocephalus".)

These forms of hydrocephalus are distinct from two radiographic findings that include the same word. The term "hydrocephalus ex-vacuo" refers to dilatation of the ventricles secondary to brain atrophy or loss of brain tissue secondary to an insult; hydrocephalus ex-vacuo is not accompanied by increased ICP. The term "external hydrocephalus" or "benign enlargement of the extra-axial spaces" refers to excessive fluid, usually CSF, in the subarachnoid spaces and is associated with familial macrocephaly [3,4].


The prevalence of congenital and infantile hydrocephalus in the United States and Europe has been estimated as 0.5 to 0.8 per 1000 live and still births [5-7]. Approximately 15 to 25 percent of these cases are associated with myelomeningocele (spina bifida) [6,8].


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Literature review current through: Nov 2016. | This topic last updated: Mon May 11 00:00:00 GMT+00:00 2015.
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  1. Fishman MA. Hydrocephalus. In: Neurological pathophysiology, Eliasson SG, Prensky AL, Hardin WB (Eds), Oxford, New York 1978.
  2. Carey CM, Tullous MW, Walker ML. Hydrocephalus: Etiology, Pathologic Effects, Diagnosis, and Natural History. In: Pediatric Neurosurgery, 3 ed, Cheek WR (Ed), WB Saunders Company, Philadelphia 1994.
  3. Hellbusch LC. Benign extracerebral fluid collections in infancy: clinical presentation and long-term follow-up. J Neurosurg 2007; 107:119.
  4. Bateman GA, Napier BD. External hydrocephalus in infants: six cases with MR venogram and flow quantification correlation. Childs Nerv Syst 2011; 27:2087.
  5. Fernell E, Hagberg G, Hagberg B. Infantile hydrocephalus epidemiology: an indicator of enhanced survival. Arch Dis Child Fetal Neonatal Ed 1994; 70:F123.
  6. Jeng S, Gupta N, Wrensch M, et al. Prevalence of congenital hydrocephalus in California, 1991-2000. Pediatr Neurol 2011; 45:67.
  7. Garne E, Loane M, Addor MC, et al. Congenital hydrocephalus--prevalence, prenatal diagnosis and outcome of pregnancy in four European regions. Eur J Paediatr Neurol 2010; 14:150.
  8. Tully HM, Capote RT, Saltzman BS. Maternal and infant factors associated with infancy-onset hydrocephalus in Washington State. Pediatr Neurol 2015; 52:320.
  9. Munch TN, Rostgaard K, Rasmussen ML, et al. Familial aggregation of congenital hydrocephalus in a nationwide cohort. Brain 2012; 135:2409.
  10. Yasuda T, Tomita T, McLone DG, Donovan M. Measurement of cerebrospinal fluid output through external ventricular drainage in one hundred infants and children: correlation with cerebrospinal fluid production. Pediatr Neurosurg 2002; 36:22.
  11. Beni-Adani L, Biani N, Ben-Sirah L, Constantini S. The occurrence of obstructive vs absorptive hydrocephalus in newborns and infants: relevance to treatment choices. Childs Nerv Syst 2006; 22:1543.
  12. Schrander-Stumpel C, Fryns JP. Congenital hydrocephalus: nosology and guidelines for clinical approach and genetic counselling. Eur J Pediatr 1998; 157:355.
  13. Munch TN, Rasmussen ML, Wohlfahrt J, et al. Risk factors for congenital hydrocephalus: a nationwide, register-based, cohort study. J Neurol Neurosurg Psychiatry 2014; 85:1253.
  14. Graf WD, Born DE, Sarnat HB. The pachygyria-polymicrogyria spectrum of cortical dysplasia in X-linked hydrocephalus. Eur J Pediatr Surg 1998; 8 Suppl 1:10.
  15. Fransen E, Van Camp G, Vits L, Willems PJ. L1-associated diseases: clinical geneticists divide, molecular geneticists unite. Hum Mol Genet 1997; 6:1625.
  16. Sasaki-Adams D, Elbabaa SK, Jewells V, et al. The Dandy-Walker variant: a case series of 24 pediatric patients and evaluation of associated anomalies, incidence of hydrocephalus, and developmental outcomes. J Neurosurg Pediatr 2008; 2:194.
  17. Frawley GP, Dargaville PA, Mitchell PJ, et al. Clinical course and medical management of neonates with severe cardiac failure related to vein of Galen malformation. Arch Dis Child Fetal Neonatal Ed 2002; 87:F144.
  18. Akins PT, Guppy KH, Axelrod YV, et al. The genesis of low pressure hydrocephalus. Neurocrit Care 2011; 15:461.
  19. Kirkpatrick M, Engleman H, Minns RA. Symptoms and signs of progressive hydrocephalus. Arch Dis Child 1989; 64:124.
  20. Alexander E Jr, Davis CH. Macewen's sign--"the cracked pot sound". Surg Neurol 1987; 27:519.
  21. Löppönen T, Saukkonen AL, Serlo W, et al. Accelerated pubertal development in patients with shunted hydrocephalus. Arch Dis Child 1996; 74:490.
  22. Rekate HL. Treatment of Hydrocephalus. In: Pediatric Neurosurgery, 3, Cheek WR (Ed), WB Saunders Company, Philadelphia 1994.
  23. Brunetti MA, Mahesh M, Nabaweesi R, et al. Diagnostic radiation exposure in pediatric trauma patients. J Trauma 2011; 70:E24.
  24. Dinçer A, Özek MM. Radiologic evaluation of pediatric hydrocephalus. Childs Nerv Syst 2011; 27:1543.
  25. Cavalheiro S, Moron AF, Almodin CG, et al. Fetal hydrocephalus. Childs Nerv Syst 2011; 27:1575.
  26. Jea A, Bradshaw TJ, Whitehead WE, et al. The high risks of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood: case report and literature review. Pediatr Neurosurg 2010; 46:141.
  27. Schneider SJ, Wisoff JS, Epstein FJ. Complications of ventriculoperitoneal shunt procedures or hydrocephalus associated with vein of Galen malformations in childhood. Neurosurgery 1992; 30:706.
  28. Cinalli G, Spennato P, Nastro A, et al. Hydrocephalus in aqueductal stenosis. Childs Nerv Syst 2011; 27:1621.
  29. Chumas P, Tyagi A, Livingston J. Hydrocephalus--what's new? Arch Dis Child Fetal Neonatal Ed 2001; 85:F149.
  30. Drake JM, Kestle JR, Milner R, et al. Randomized trial of cerebrospinal fluid shunt valve design in pediatric hydrocephalus. Neurosurgery 1998; 43:294.
  31. Stein SC, Guo W. Have we made progress in preventing shunt failure? A critical analysis. J Neurosurg Pediatr 2008; 1:40.
  32. Langley JM, LeBlanc JC, Drake J, Milner R. Efficacy of antimicrobial prophylaxis in placement of cerebrospinal fluid shunts: meta-analysis. Clin Infect Dis 1993; 17:98.
  33. Forward KR, Fewer HD, Stiver HG. Cerebrospinal fluid shunt infections. A review of 35 infections in 32 patients. J Neurosurg 1983; 59:389.
  34. Casey AT, Kimmings EJ, Kleinlugtebeld AD, et al. The long-term outlook for hydrocephalus in childhood. A ten-year cohort study of 155 patients. Pediatr Neurosurg 1997; 27:63.
  35. Ratilal B, Costa J, Sampaio C. Antibiotic prophylaxis for surgical introduction of intracranial ventricular shunts. Cochrane Database Syst Rev 2006; :CD005365.
  36. Agarwal N, Vernier E, Ravenscroft S, et al. Slit ventricle syndrome: a case report of intermittent intracranial hypertension. J Child Neurol 2013; 28:784.
  37. Siomin V, Cinalli G, Grotenhuis A, et al. Endoscopic third ventriculostomy in patients with cerebrospinal fluid infection and/or hemorrhage. J Neurosurg 2002; 97:519.
  38. Scarrow AM, Levy EI, Pascucci L, Albright AL. Outcome analysis of endoscopic III ventriculostomy. Childs Nerv Syst 2000; 16:442.
  39. Javadpour M, Mallucci C, Brodbelt A, et al. The impact of endoscopic third ventriculostomy on the management of newly diagnosed hydrocephalus in infants. Pediatr Neurosurg 2001; 35:131.
  40. Drake JM, Kulkarni AV, Kestle J. Endoscopic third ventriculostomy versus ventriculoperitoneal shunt in pediatric patients: a decision analysis. Childs Nerv Syst 2009; 25:467.
  41. Kulkarni AV, Drake JM, Mallucci CL, et al. Endoscopic third ventriculostomy in the treatment of childhood hydrocephalus. J Pediatr 2009; 155:254.
  42. Kulkarni AV, Drake JM, Kestle JR, et al. Predicting who will benefit from endoscopic third ventriculostomy compared with shunt insertion in childhood hydrocephalus using the ETV Success Score. J Neurosurg Pediatr 2010; 6:310.
  43. Ogiwara H, Dipatri AJ Jr, Alden TD, et al. Endoscopic third ventriculostomy for obstructive hydrocephalus in children younger than 6 months of age. Childs Nerv Syst 2010; 26:343.
  44. Bouras T, Sgouros S. Complications of endoscopic third ventriculostomy: a systematic review. Acta Neurochir Suppl 2012; 113:149.
  45. Whitelaw A. Repeated lumbar or ventricular punctures in newborns with intraventricular hemorrhage. Cochrane Database Syst Rev 2001; :CD000216.
  46. Berger A, Weninger M, Reinprecht A, et al. Long-term experience with subcutaneously tunneled external ventricular drainage in preterm infants. Childs Nerv Syst 2000; 16:103.
  47. Heep A, Engelskirchen R, Holschneider A, Groneck P. Primary intervention for posthemorrhagic hydrocephalus in very low birthweight infants by ventriculostomy. Childs Nerv Syst 2001; 17:47.
  48. Hoppe-Hirsch E, Laroussinie F, Brunet L, et al. Late outcome of the surgical treatment of hydrocephalus. Childs Nerv Syst 1998; 14:97.
  49. Klepper J, Büsse M, Strassburg HM, Sörensen N. Epilepsy in shunt-treated hydrocephalus. Dev Med Child Neurol 1998; 40:731.
  50. Bourgeois M, Sainte-Rose C, Cinalli G, et al. Epilepsy in children with shunted hydrocephalus. J Neurosurg 1999; 90:274.
  51. Caraballo RH, Bongiorni L, Cersósimo R, et al. Epileptic encephalopathy with continuous spikes and waves during sleep in children with shunted hydrocephalus: a study of nine cases. Epilepsia 2008; 49:1520.
  52. Kulkarni AV, Donnelly R, Shams I. Comparison of Hydrocephalus Outcome Questionnaire scores to neuropsychological test performance in school-aged children. J Neurosurg Pediatr 2011; 8:396.
  53. Kulkarni AV, Drake JM, Rabin D, et al. Measuring the health status of children with hydrocephalus by using a new outcome measure. J Neurosurg 2004; 101:141.
  54. Lindquist B, Carlsson G, Persson EK, Uvebrant P. Learning disabilities in a population-based group of children with hydrocephalus. Acta Paediatr 2005; 94:878.
  55. Brookshire BL, Fletcher JM, Bohan TP, et al. Verbal and nonverbal skill discrepancies in children with hydrocephalus: a five-year longitudinal follow-up. J Pediatr Psychol 1995; 20:785.
  56. Adams-Chapman I, Hansen NI, Stoll BJ, et al. Neurodevelopmental outcome of extremely low birth weight infants with posthemorrhagic hydrocephalus requiring shunt insertion. Pediatrics 2008; 121:e1167.