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Hydrocephalus in children: Management and prognosis

Authors
Abilash Haridas, MD
Tadanori Tomita, MD
Section Editor
Marc C Patterson, MD, FRACP
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

Hydrocephalus is a disorder in which an excessive amount of cerebrospinal fluid (CSF) accumulates within the cerebral ventricles and/or subarachnoid spaces, resulting in ventricular dilation and increased intracranial pressure (ICP) [1,2].

The management and prognosis of hydrocephalus in children will be reviewed here. The pathophysiology, etiology, clinical features, and diagnosis of hydrocephalus are discussed separately. (See "Hydrocephalus in children: Physiology, pathogenesis, and etiology" and "Hydrocephalus in children: Clinical features and diagnosis".)

This topic will focus on the management and prognosis of obstructive and communicating hydrocephalus, which are almost always associated with increased ICP. Normal pressure hydrocephalus, a condition seen predominantly in adults in which the cerebral ventricles are pathologically enlarged, but the ICP is not elevated, is discussed separately. (See "Normal pressure hydrocephalus".)

The prevention and initial management of hydrocephalus associated with intraventricular hemorrhage in preterm neonates is also discussed in greater detail separately. (See "Management and complications of intraventricular hemorrhage in the newborn".)

TERMINOLOGY

The following terms are used this topic:

                     

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Literature review current through: Jul 2017. | This topic last updated: Feb 28, 2017.
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References
Top
  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. 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.
  6. 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.
  7. 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.
  8. 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.
  9. Mazzola CA, Choudhri AF, Auguste KI, et al. Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 2: Management of posthemorrhagic hydrocephalus in premature infants. J Neurosurg Pediatr 2014; 14 Suppl 1:8.
  10. 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.
  11. Scarrow AM, Levy EI, Pascucci L, Albright AL. Outcome analysis of endoscopic III ventriculostomy. Childs Nerv Syst 2000; 16:442.
  12. 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.
  13. Kulkarni AV, Drake JM, Mallucci CL, et al. Endoscopic third ventriculostomy in the treatment of childhood hydrocephalus. J Pediatr 2009; 155:254.
  14. Kulkarni AV, Riva-Cambrin J, Holubkov R, et al. Endoscopic third ventriculostomy in children: prospective, multicenter results from the Hydrocephalus Clinical Research Network. J Neurosurg Pediatr 2016; 18:423.
  15. Limbrick DD Jr, Baird LC, Klimo P Jr, et al. Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 4: Cerebrospinal fluid shunt or endoscopic third ventriculostomy for the treatment of hydrocephalus in children. J Neurosurg Pediatr 2014; 14 Suppl 1:30.
  16. 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.
  17. 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.
  18. Flannery AM, Duhaime AC, Tamber MS, et al. Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 3: Endoscopic computer-assisted electromagnetic navigation and ultrasonography as technical adjuvants for shunt placement. J Neurosurg Pediatr 2014; 14 Suppl 1:24.
  19. Baird LC, Mazzola CA, Auguste KI, et al. Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 5: Effect of valve type on cerebrospinal fluid shunt efficacy. J Neurosurg Pediatr 2014; 14 Suppl 1:35.
  20. Chumas P, Tyagi A, Livingston J. Hydrocephalus--what's new? Arch Dis Child Fetal Neonatal Ed 2001; 85:F149.
  21. Drake JM, Kestle JR, Milner R, et al. Randomized trial of cerebrospinal fluid shunt valve design in pediatric hydrocephalus. Neurosurgery 1998; 43:294.
  22. Stein SC, Guo W. Have we made progress in preventing shunt failure? A critical analysis. J Neurosurg Pediatr 2008; 1:40.
  23. 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.
  24. Kahle KT, Kulkarni AV, Limbrick DD Jr, Warf BC. Hydrocephalus in children. Lancet 2016; 387:788.
  25. Forward KR, Fewer HD, Stiver HG. Cerebrospinal fluid shunt infections. A review of 35 infections in 32 patients. J Neurosurg 1983; 59:389.
  26. 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.
  27. Ratilal B, Costa J, Sampaio C. Antibiotic prophylaxis for surgical introduction of intracranial ventricular shunts. Cochrane Database Syst Rev 2006; :CD005365.
  28. Klimo P Jr, Van Poppel M, Thompson CJ, et al. Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 6: Preoperative antibiotics for shunt surgery in children with hydrocephalus: a systematic review and meta-analysis. J Neurosurg Pediatr 2014; 14 Suppl 1:44.
  29. Klimo P Jr, Thompson CJ, Baird LC, et al. Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 7: Antibiotic-impregnated shunt systems versus conventional shunts in children: a systematic review and meta-analysis. J Neurosurg Pediatr 2014; 14 Suppl 1:53.
  30. Agarwal N, Vernier E, Ravenscroft S, et al. Slit ventricle syndrome: a case report of intermittent intracranial hypertension. J Child Neurol 2013; 28:784.
  31. Cinalli G, Spennato P, Nastro A, et al. Hydrocephalus in aqueductal stenosis. Childs Nerv Syst 2011; 27:1621.
  32. 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.
  33. Third Ventriculostomy Versus Shunt for Children With Hydrocephalus. Study record detail. Available at: https://clinicaltrials.gov/ct2/show/NCT01801267 (Accessed on November 04, 2016).
  34. A Study Comparing Two Treatments for Infants With Hydrocephalus. Study record detail. Available at: https://clinicaltrials.gov/ct2/show/NCT00652470 (Accessed on November 04, 2016).
  35. Bouras T, Sgouros S. Complications of endoscopic third ventriculostomy: a systematic review. Acta Neurochir Suppl 2012; 113:149.
  36. Warf BC, Tracy S, Mugamba J. Long-term outcome for endoscopic third ventriculostomy alone or in combination with choroid plexus cauterization for congenital aqueductal stenosis in African infants. J Neurosurg Pediatr 2012; 10:108.
  37. Weil AG, Fallah A, Chamiraju P, et al. Endoscopic third ventriculostomy and choroid plexus cauterization with a rigid neuroendoscope in infants with hydrocephalus. J Neurosurg Pediatr 2015; :1.
  38. Stone SS, Warf BC. Combined endoscopic third ventriculostomy and choroid plexus cauterization as primary treatment for infant hydrocephalus: a prospective North American series. J Neurosurg Pediatr 2014; 14:439.
  39. Weil AG, Westwick H, Wang S, et al. Efficacy and safety of endoscopic third ventriculostomy and choroid plexus cauterization for infantile hydrocephalus: a systematic review and meta-analysis. Childs Nerv Syst 2016; 32:2119.
  40. Neurocognitive Outcomes and Changes in Brain and CSF Volume After Treatment of PIH in Ugandan Infants by Shunting vs ETV/CPC. Trial record. Available at: https://clinicaltrials.gov/ct2/show/NCT01936272?term=NCT01936272&rank=1 (Accessed on November 03, 2016).
  41. Nikas DC, Post AF, Choudhri AF, et al. Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 10: Change in ventricle size as a measurement of effective treatment of hydrocephalus. J Neurosurg Pediatr 2014; 14 Suppl 1:77.
  42. Rocque BG, Lapsiwala S, Iskandar BJ. Ventricular shunt tap as a predictor of proximal shunt malfunction in children: a prospective study. J Neurosurg Pediatr 2008; 1:439.
  43. Hoppe-Hirsch E, Laroussinie F, Brunet L, et al. Late outcome of the surgical treatment of hydrocephalus. Childs Nerv Syst 1998; 14:97.
  44. Klepper J, Büsse M, Strassburg HM, Sörensen N. Epilepsy in shunt-treated hydrocephalus. Dev Med Child Neurol 1998; 40:731.
  45. Bourgeois M, Sainte-Rose C, Cinalli G, et al. Epilepsy in children with shunted hydrocephalus. J Neurosurg 1999; 90:274.
  46. 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.
  47. 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.
  48. 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.
  49. Lindquist B, Carlsson G, Persson EK, Uvebrant P. Learning disabilities in a population-based group of children with hydrocephalus. Acta Paediatr 2005; 94:878.
  50. 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.
  51. 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.