Abstract
Hydrocephalus presents specific challenges – in both diagnosis and management – in patients with craniosynostosis. Hydrocephalus has been recognized as a feature of the syndromic forms, in particular the more phenotypically severe, for a long time. Controversy still exists regarding the pathophysiology, prevalence, and optimal management strategy of hydrocephalus in children affected by syndromic craniosynostosis. In this chapter, we review the literature and our experience regarding each of these important facets, with an attempt made to offer some unifying theories and practical guides to management. In addition, we will review specific considerations, including the association of hydrocephalus, craniosynostosis, and Chiari 1, as well as the use of craniofacial surgery for skull deformity induced either by hydrocephalus itself or its treatment.
Similar content being viewed by others
References
Abu-Sittah GS, Jeelani O, Dunaway D et al (2016) Raised intracranial pressure in Crouzon syndrome: incidence, causes, and management. J Neurosurg Pediatr 17(4):469–475
Bhadkamkar MA, Albright SB, Wolfswinkel EM et al (2015) Posterior cranial vault distraction in the treatment of shunt-induced craniosynostosis. J Craniofac Surg 26(1):e70–e72
Booth CD, Figueroa RE, Lehn A et al (2011) Analysis of the jugular foramen in pediatric patients with craniosynostosis. J Craniofac Surg 22(1):285–288
Boulet SL, Rasmussen SA, Honein MA (2008) A population-based study of craniosynostosis in metropolitan Atlanta, 1989–2003. Am J Med Genet A 146A(8):984–991
Brooks ED, Yang J, Beckett JS et al (2016) Normalization of brain morphology after surgery in sagittal craniosynostosis. J Neurosurg Pediatr 17(4):460–468
Cinalli G, Renier D, Sebag G et al (1995) Chronic tonsillar herniation in Crouzon’s and Apert’s syndromes: the role of premature synostosis of the lambdoid suture. J Neurosurg 83(4):575–582
Cinalli G, Sainte-Rose C, Kollar EM et al (1998a) Hydrocephalus and craniosynostosis. J Neurosurg 88(2):209–214
Cinalli G, Chumas P, Arnaud E et al (1998b) Occipital remodeling and suboccipital decompression in severe craniosynostosis associated with tonsillar herniation. Neurosurgery 42(1):66–71
Cinalli G, Spennato P, Sainte-Rose C et al (2005) Chiari malformation in craniosynostosis. Childs Nerv Syst 21(10):889–901
Cohen MM Jr (1993) Pfeiffer syndrome update, clinical subtypes, and guidelines for differential diagnosis. Am J Med Genet 45(3):300–307
Cohen MM Jr, Kreiborg S (1990) The central nervous system in the Apert syndrome. Am J Med Genet 35(1):36–45
Coll G, Di Rocco F (2015) Morphology of the foramen magnum in syndromic and non-syndromic brachycephaly: letter to the editor. Childs Nerv Syst 31(8):1213–1214
Collmann H, Sörensen N, Krauss J (2005) Hydrocephalus in craniosynostosis: a review. Childs Nerv Syst 21(10):902–912
Collmann H, Sörensen N, Krauss J et al (1988) Hydrocephalus in craniosynostosis. Childs Nerv Syst 4(5):279–285
Da Costa AC, Walters I, Savarirayan R et al (2006) Intellectual outcomes in children and adolescents with syndromic and nonsyndromic craniosynostosis. Plast Reconstr Surg 118(1):175–181
Davis C, Lauritzen CG (2008) Spring-assisted remodeling for ventricular shunt-induced cranial deformity. J Craniofac Surg 19(3):588–592
de Jong T, Rijken BF, Lequin MH et al (2012) Brain and ventricular volume in patients with syndromic and complex craniosynostosis. Childs Nerv Syst 28(1):137–140
Di Ieva A, Bruner E, Davidson J et al (2013) Cranial sutures: a multidisciplinary review. Childs Nerv Syst 29(6):893–905
Di Rocco C, Frassanito P, Massimi L et al (2011) Hydrocephalus and Chiari type I malformation. Childs Nerv Syst 27(10):1653–1664
Di Rocco F, Jucá CE, Arnaud E et al (2010) The role of endoscopic third ventriculostomy in the treatment of hydrocephalus associated with faciocraniosynostosis. J Neurosurg Pediatr 6(1):17–22
Doorenbosch X, Molloy CJ, David DJ et al (2009) Management of cranial deformity following ventricular shunting. Childs Nerv Syst 25(7):871–874
Drake JM, Kestle JR, Milner R et al (1998) Randomized trial of cerebrospinal fluid shunt valve design in pediatric hydrocephalus. Neurosurgery 43(2):294–303
Dwyer CM, Prelog K, Owler BK (2013) The role of venous sinus outflow obstruction in pediatric idiopathic intracranial hypertension. J Neurosurg Pediatr 11(2):144–149
Ehni G (1982) Reduction of head size in advanced hydrocephalus: a case report. Neurosurgery 11(2):223–228
Erdinçler P, Kaynar MY, Canbaz B et al (1998) Two different surgical techniques for reduction cranioplasty. Childs Nerv Syst 14(8):372–377
Farb RI, Vanek I, Scott JN et al (2003) Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology 60(9):1418–1424
Fearon JA, Dimas V, Ditthakasem K (2016) Lambdoid Craniosynostosis: the relationship with Chiari deformations and an analysis of surgical outcomes. Plast Reconstr Surg 137(3):946–951
Fearon JA, Rhodes J (2009) Pfeiffer syndrome: a treatment evaluation. Plast Reconstr Surg 123(5):1560–1569
Fishman MA, Hogan GR, Dodge PR (1971) The concurrence of hydrocephalus and craniosynostosis. J Neurosurg 34(5):621–629
Florisson JM, Barmpalios G, Lequin M et al (2015) Venous hypertension in syndromic and complex craniosynostosis: the abnormal anatomy of the jugular foramen and collaterals. J Craniomaxillofac Surg 43(3):312–318
Golabi M, Edwards MS, Ousterhout DK (1987) Craniosynostosis and hydrocephalus. Neurosurgery 21(1):63–67
Greitz D (2004) The hydrodynamic hypothesis versus the bulk flow hypothesis. Neurosurg Rev 27(4):299–300
Hanieh A, David DJ (1993) Apert’s syndrome. Childs Nerv Syst 9(5):289–291
Hayward R (2005) Venous hypertension and craniosynostosis. Childs Nerv Syst 21(10):880–888
Hayward R, Britto JA, Dunaway D et al (2015) Raised intracranial pressure and nonsyndromic sagittal craniosynostosis. J Neurosurg Pediatr 16(3):346–348
Hill CA, Martínez-Abadías N, Motch SM et al (2013) Postnatal brain and skull growth in an Apert syndrome mouse model. Am J Med Genet A 161A(4):745–757
Hogan GR, Bauman ML (1971) Hydrocephalus in Apert’s syndrome. J Pediatr 79(5):782–787
Hörmann D (1980) Premature craniosynostosis after ventricular shunting in hydrocephalic children (author’s translation). Zentralbl Chir 105(19):1270–1282
Jeevan DS, Anlsow P, Jayamohan J (2008) Abnormal venous drainage in syndromic craniosynostosis and the role of CT venography. Childs Nerv Syst 24(12):1413–1420
Kollar CD, Johnston IH, Sholler GF (2001) Communicating hydrocephalus secondary to a cardiac tumour compressing the superior vena cava. Childs Nerv Syst 17(3):117–120
Levine DN (2004) The pathogenesis of syringomyelia associated with lesions at the foramen magnum: a critical review of existing theories and proposal of a new hypothesis. J Neurol Sci 220(1–2):3–21
Liasis A, Nischal KK, Walters B et al (2006) Monitoring visual function in children with syndromic craniosynostosis: a comparison of 3 methods. Arch Ophthalmol 124(8):1119–1126
Machado G, Di Rocco F, Sainte-Rose C et al (2011) Cloverleaf skull deformity and hydrocephalus. Childs Nerv Syst 27(10):1683–1691
Martínez-Lage JF, Ruiz-Espejo Vilar A, Pérez-Espejo MA et al (2006) Shunt-related craniocerebral disproportion: treatment with cranial vault expanding procedures. Neurosurg Rev 29(3):229–235
Marucci DD, Dunaway DJ, Jones BM et al (2008) Raised intracranial pressure in Apert syndrome. Plast Reconstr Surg 122(4):1162–1168
Mathews MS, Loudon WG, Muhonen MG et al (2007) Vault reduction cranioplasty for extreme hydrocephalic macrocephaly. J Neurosurg 107(4 Suppl):332–337
McLaughlin JF, Loeser JD, Roberts TS (1997) Acquired hydrocephalus associated with superior vena cava syndrome in infants. Childs Nerv Syst 13(2):59–63
Moore MH, Hanieh A (1994) Hydrocephalus in Pfeiffer syndrome. J Clin Neurosci 1(3):202–204
Morriss-Kay GM, Wilkie AO (2005) Growth of the normal skull vault and its alteration in craniosynostosis: insights from human genetics and experimental studies. J Anat 207(5):637–653
Mulliken JB, Steinberger D, Kunze S et al (1999) Molecular diagnosis of bilateral coronal synostosis. Plast Reconstr Surg 104(6):1603–1615
Murovic JA, Posnick JC, Drake JM et al (1993) Hydrocephalus in Apert syndrome: a retrospective review. Pediatr Neurosurg 19(3):151–155
Mursch K, Brockmann K, Lang JK et al (1998) Visually evoked potentials in 52 children requiring operative repair of craniosynostosis. Pediatr Neurosurg 29(6):320–323
Nilsson D, Svensson J, Korkmaz BA et al (2013) Decreased head circumference in shunt-treated compared with healthy children. J Neurosurg Pediatr 12(5):483–490
Noetzel MJ, Marsh JL, Palkes H et al (1985) Hydrocephalus and mental retardation in craniosynostosis. J Pediatr 107(6):885–892
Okudera T, Huang YP, Ohta T et al (1994) Development of posterior fossa dural sinuses, emissary veins, and jugular bulb: morphological and radiologic study. Am J Neuroradiol 15(10):1871–1883
Proudman TW, Clark BE, Moore MH et al (1995) Central nervous system imaging in Crouzon’s syndrome. J Craniofac Surg 6(5):401–405
Raybaud C (2016) MR assessment of pediatric hydrocephalus: a road map. Childs Nerv Syst 32(1):19–41
Raybaud C, Di Rocco C (2007) Brain malformation in syndromic craniosynostoses, a primary disorder of white matter: a review. Childs Nerv Syst 23(12):1379–1388
Reid S, Ferretti P (2003) Differential expression of fibroblast growth factor receptors in the developing murine choroid plexus. Brain Res Dev Brain Res 141(1–2):15–24
Rekate HL (2008) The definition and classification of hydrocephalus: a personal recommendation to stimulate debate. Cerebrospinal Fluid Res 5:2
Renier D, Arnaud E, Cinalli G et al (1996) Mental prognosis of Apert syndrome. Arch Pediatr 3(8):752–760
Rice DP (2008) Developmental anatomy of craniofacial sutures. Front Oral Biol 12:1–21
Rich PM, Cox TC, Hayward RD (2003) The jugular foramen in complex and syndromic craniosynostosis and its relationship to raised intracranial pressure. Am J Neuroradiol 24:45–51
Richtsmeier JT, Flaherty K (2013) Hand in glove: brain and skull in development and dysmorphogenesis. Acta Neuropathol 125(4):469–489
Rijken BF, den Ottelander BK, van Veelen ML et al (2015a) The occipitofrontal circumference: reliable prediction of the intracranial volume in children with syndromic and complex craniosynostosis. Neurosurg Focus 38(5):E9
Rijken BF, Lequin MH, van der Lijn F et al (2015b) The role of the posterior fossa in developing Chiari I malformation in children with craniosynostosis syndromes. J Craniomaxillofac Surg 43(6):813–819
Rollins N, Booth T, Shapiro K (2000) MR venography in children with complex craniosynostosis. Pediatr Neurosurg 32:308–315
Sainte-Rose C, LaCombe J, Pierre-Kahn A et al (1984) Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants? J Neurosurg 60(4):727–736
Sgouros S, Goldin JH, Hockley AD et al (1999a) Intracranial volume change in childhood. J Neurosurg 91(4):610–616
Sgouros S, Hockley AD, Goldin JH et al (1999b) Intracranial volume change in craniosynostosis. J Neurosurg 91(4):617–625
Sharma VP, Fenwick AL, Brockop MS et al (2013) Mutations in TCF12, encoding a basic helix-loop-helix partner of TWIST1, are a frequent cause of coronal craniosynostosis. Nat Genet 45(3):304–307
Strahle J, Muraszko KM, Buchman SR et al (2011) Chiari malformation associated with craniosynostosis. Neurosurg Focus 31(3):E2
Strenger L (1963) Complications of ventriculovenous shunts. J Neurosurg 20:219–224
Sun P, Shin JH, Persing JA (1997) Management of the ventricular shunt in posterior deformities of the skull in craniosynostosis. J Craniofac Surg 8(1):38–41
Sundine MJ, Wirth GA, Brenner KA et al (2006) Cranial vault reduction cranioplasty in children with hydrocephalic macrocephaly. J Craniofac Surg 17(4):645–655
Taylor WJ, Hayward RD, Lasjaunias P et al (2001) Enigma of raised intracranial pressure in patients with complex craniosynostosis: the role of abnormal intracranial venous drainage. J Neurosurg 94:377–385
Thompson DA, Liasis A, Hardy S et al (2006) Prevalence of abnormal pattern reversal visual evoked potentials in craniosynostosis. Plast Reconstr Surg 118(1):184–192
Thompson DNP, Jones BM, Harkness WJF et al (1997a) Consequences of cranial vault expansion surgery for craniosynostosis. Pediatr Neurosurg 26(6):296–303
Thompson DNP, Harkness WJF, Jones BM et al (1997b) Aetiology of herniation of the hindbrain in craniosynostosis. An investigation incorporating intracranial pressure monitoring and magnetic resonance imaging. Pediatr Neurosurg 26(6):288–295
Thompson DNP, Hayward RD, Harkness WJ et al (1995) Lessons from a case of kleeblattschädel. Case report. J Neurosurg 82(6):1071–1074
Tubbs RS, Sharma A, Griessenauer C et al (2012) Kleeblattschädel skull: a review of its history, diagnosis, associations, and treatment. Childs Nerv Syst 29(5):745–748
Twigg SR, Vorgia E, McGowan SJ et al (2013) Reduced dosage of ERF causes complex craniosynostosis in humans and mice and links ERK1/2 signaling to regulation of osteogenesis. Nat Genet 45(3):308–313
Twigg SR, Wilkie AO (2015a) A genetic-pathophysiological framework for craniosynostosis. Am J Hum Genet 97(3):359–377
Twigg SR, Wilkie AO (2015b) New insights into craniofacial malformations. Hum Mol Genet 24(R1):R50–R59
Vries JK, Habal MB (1979) Cranio-orbital correction for massive enlargement of the cranial vault. Plast Reconstr Surg 63(4):466–472
Wall SA, Thomas GP, Johnson D et al (2014) The preoperative incidence of raised intracranial pressure in nonsyndromic sagittal craniosynostosis is underestimated in the literature. J Neurosurg Pediatr 14(6):674–681
Wilkie AO, Byren JC, Hurst JA et al (2010) Prevalence and complications of single-gene and chromosomal disorders in craniosynostosis. Pediatrics 126(2):e391–e400
Yacubian-Fernandes A, Palhares A, Giglio A et al (2004) Apert syndrome: analysis of associated brain malformations and conformational changes determined by surgical treatment. J Neuroradiol 31(2):116–122
Yeh E, Fanganiello RD, Sunaga DY et al (2013) Novel molecular pathways elicited by mutant FGFR2 may account for brain abnormalities in Apert syndrome. PLoS One 8(4):e60439
Zuurbier SM, van den Berg R, Troost D et al (2015) Hydrocephalus in cerebral venous thrombosis. J Neurol 262(4):931–937
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Section Editor information
Rights and permissions
Copyright information
© 2018 Springer International Publishing AG, part of Springer Nature
About this entry
Cite this entry
James, G., Thompson, D.N. (2018). Cerebrospinal Fluid hydrodynamics in Craniosynostosis. In: Cinalli, G., Ozek, M., Sainte-Rose, C. (eds) Pediatric Hydrocephalus. Springer, Cham. https://doi.org/10.1007/978-3-319-31889-9_64-1
Download citation
DOI: https://doi.org/10.1007/978-3-319-31889-9_64-1
Received:
Accepted:
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-31889-9
Online ISBN: 978-3-319-31889-9
eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences