Hydrocephalus in children: Physiology, pathogenesis, and etiology
- Abilash Haridas, MD
Abilash Haridas, MD
- Pediatric & Cerebrovascular Neurosurgery, Children’s Hospital of Michigan
- Assistant Professor of Neurosurgery, Wayne State University
- Tadanori Tomita, MD
Tadanori Tomita, MD
- Yeager Professor and Division Head of Pediatric Neurosurgery,
- Director, Falk Brain Tumor Center
- Ann & Robert H. Lurie Children’s Hospital of Chicago;
- Professor and Vice-Chair, Department of Neurosurgery,
- Northwestern University Feinberg School of Medicine
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 physiology, pathogenesis, and etiology of hydrocephalus will be reviewed here. The clinical features, diagnosis, management, and prognosis of hydrocephalus are discussed separately. (See "Hydrocephalus in children: Clinical features and diagnosis" and "Hydrocephalus in children: Management and prognosis".)
Normal pressure hydrocephalus, a condition seen predominantly in adults in which the cerebral ventricles are pathologically enlarged, but the ICP is not elevated, is also discussed separately. (See "Normal pressure hydrocephalus".)
The following terms are used this topic:
●Obstructive hydrocephalus – Obstructive hydrocephalus (also called noncommunicating hydrocephalus) refers to excess accumulation of cerebrospinal fluid (CSF) due to structural blockage of CSF flow within the ventricular system. This is the most common form of hydrocephalus in children and is almost always associated with increased intracranial pressure (ICP). (See 'Obstruction' below.)
- Fishman MA. Hydrocephalus. In: Neurological pathophysiology, Eliasson SG, Prensky AL, Hardin WB (Eds), Oxford, New York 1978.
- 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.
- Hellbusch LC. Benign extracerebral fluid collections in infancy: clinical presentation and long-term follow-up. J Neurosurg 2007; 107:119.
- Bateman GA, Napier BD. External hydrocephalus in infants: six cases with MR venogram and flow quantification correlation. Childs Nerv Syst 2011; 27:2087.
- Fernell E, Hagberg G, Hagberg B. Infantile hydrocephalus epidemiology: an indicator of enhanced survival. Arch Dis Child Fetal Neonatal Ed 1994; 70:F123.
- Jeng S, Gupta N, Wrensch M, et al. Prevalence of congenital hydrocephalus in California, 1991-2000. Pediatr Neurol 2011; 45:67.
- 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.
- Persson EK, Anderson S, Wiklund LM, Uvebrant P. Hydrocephalus in children born in 1999-2002: epidemiology, outcome and ophthalmological findings. Childs Nerv Syst 2007; 23:1111.
- Tully HM, Capote RT, Saltzman BS. Maternal and infant factors associated with infancy-onset hydrocephalus in Washington State. Pediatr Neurol 2015; 52:320.
- Tully HM, Dobyns WB. Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet 2014; 57:359.
- Munch TN, Rostgaard K, Rasmussen ML, et al. Familial aggregation of congenital hydrocephalus in a nationwide cohort. Brain 2012; 135:2409.
- 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.
- 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.
- Schrander-Stumpel C, Fryns JP. Congenital hydrocephalus: nosology and guidelines for clinical approach and genetic counselling. Eur J Pediatr 1998; 157:355.
- 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.
- 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.
- Fransen E, Van Camp G, Vits L, Willems PJ. L1-associated diseases: clinical geneticists divide, molecular geneticists unite. Hum Mol Genet 1997; 6:1625.
- 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.
- 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.
- Akins PT, Guppy KH, Axelrod YV, et al. The genesis of low pressure hydrocephalus. Neurocrit Care 2011; 15:461.
- CSF production
- CSF circulation
- CSF absorption
- Impaired absorption
- Excessive production
- - Neural tube defects
- - Isolated hydrocephalus
- - X-linked hydrocephalus
- - CNS malformations
- - Syndromic forms
- - Intrauterine infection
- - Choroid plexus papilloma or carcinoma
- Acquired hydrocephalus
- - Posthemorrhagic hydrocephalus
- - CNS tumors
- - CNS infections
- Low pressure hydrocephalus
- INFORMATION FOR PATIENTS