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Cerebro-venous hypertension: a frequent cause of so-called “external hydrocephalus” in infants

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Abstract

Introduction

External hydrocephalus (eHC) is commonly defined as a subtype of infant “hydrocephalus” consisting of macrocepahly associated with enlarged subarachnoid space and no or mild ventriculomegaly. This status is thought to be related to impaired CSF absorption because of arachnoid villi immaturity. However, other factors like the venous system might be involved in the development of the clinical picture.

Methods

All patients diagnosed with eHC received prospectively contrast-enhanced 3D MR phlebography. Venous sis abnormalities were graded depending on the number of affected sinus segments and type. External CSF space volume was quantified planimetrically.

Results

Seventeen patients with the typical clinical feature of eHC were included. In 15, venous sinus abnormalities were found. There was a significant correlation between the volume of the widened cortical subarachnoid space (CSAS) and the number of venous sinus segments affected. Conversely, ventricular volume was not correlated.

Conclusion

These results support the hypothesis that impaired venous outflow plays a major role in external hydrocephalus development. Raised venous pressure increases intracranial pressure accelerating head growth, resulting in an enlargement of the cortical subarachnoid space. Increased venous pressure increases the capillary bed pressure and brain turgor preventing ventricular space to enlarge forcing displacement of ventricular CSF to the subarachnoid space. As a result, ventriculomegaly is rarely found. The descriptive term “external hydrocephalus” implying a primary etiology within the CSF system is misleading and this work supports the notion that venous hypertension is the leading cause of the clinical picture.

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References

  1. Barlow CF (1984) CSF dynamics in hydrocephalus—with special attention to external hydrocephalus. Brain Dev 6:119–127

    Article  CAS  PubMed  Google Scholar 

  2. Bateman GA (2010) Hyperemic hydrocephalus: a new form of childhood hydrocephalus analogous to hyperemic intracranial hypertension in adults. J Neurosurg Pediatr 5(1):20–26

    Article  PubMed  Google Scholar 

  3. Bateman GA, Alber M, Schuhmann MU (2014) An association between external hydrocephalus in infants and reversible collapse of the venous sinuses. Neuropediatrics 45:183–187

    PubMed  Google Scholar 

  4. Bateman GA, Brown KM (2011) The measurement of CSF flow through the aqueduct in normal and hydrocephalic children: from where does it come, to where does it go? Childs Nerv Syst 28:55–63

    Article  PubMed  Google Scholar 

  5. Bateman GA, Napier BD (2011) External hydrocephalus in infants: six cases with MR venogram and flow quantification correlation. Childs Nerv Syst 27:2087–2096

    Article  Google Scholar 

  6. Brinker T, Stopa E, Morrison J, Klinge P (2014) A new look at cerebrospinal fluid circulation. Fluids Barriers CNS 11:10

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. De Bonis P, Pompucci A, Mangiola A, Rigante L, Anile C (2010) Post-traumatic hydrocephalus after decompressive craniectomy: an underestimated risk factor. J Neurotrauma 27:1965–1970

    Article  PubMed  Google Scholar 

  8. De Simone R, Ranieri A, Montella S, Bilo L, Cautiero F (2014) The role of dural sinus stenosis in idiopathic intracranial hypertension pathogenesis: the self-limiting venous collapse feedback-loop model. Panminerva Med 56(3):201–209

    PubMed  Google Scholar 

  9. Dillon T, Berman W Jr, Yabek SM, Seigel R, Akl B, Wernly J (1986) Communicating hydrocephalus: a reversible complication of the mustard operation with serial hemodynamics and long-term follow-up. Ann Thorac Surg 41:146–149

    Article  CAS  PubMed  Google Scholar 

  10. Frydrychowski AF, Winklewski PJ, Guminski W (2012) Influence of acute jugular vein compression on the cerebral blood flow velocity, pial artery pulsation and width of subarachnoid space in humans. PLoS One 7(10):e48245

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Hanlo PW, Gooskens RJ, van Schooneveld M, Tulleken CA, van der Knaap MS, Faber JA, Willemse J (1997) The effect of intracranial pressure on myelination and the relationship with neurodevelopment in infantile hydrocephalus. Dev Med Child Neurol 39:286–291

    Article  CAS  PubMed  Google Scholar 

  12. Hellbush LC (2007) Benign extracerebral fluid collections in infancy: clinical presentation and long-term follow-up. J Neurosurg 107(2 Suppl Pediatrics):119–125

    Google Scholar 

  13. Kapoor KG, Katz SE, Grzybowski DM, Lubow M (2008) Cerebrospinal fluid outflow: an evolving perspective. Brain Res Bull 77:327–334

    Article  PubMed  Google Scholar 

  14. Kumar R (2006) External hydrocephalus in small children. Childs Nerv Syst 22:1237–1241

    Article  PubMed  Google Scholar 

  15. Kendall B, Holland I (1981) Benign communicating hydrocephalus in children. Neuroradiology 21:93–96

    Article  CAS  PubMed  Google Scholar 

  16. Leliefeld PH, Gooskens RHJM, Vicken KL, Ramos LM, van der Grond J, Tulleken CAF et al (2008) Magnetic resonance imaging for quantitative flow measurement in infants with hydrocephalus: a prospective study. J Neurosurg Pediatrics 2:163–170

    Article  Google Scholar 

  17. Libicher M, Tröger J (1992) US measurement of the subarachnoid space in infants: normal values. Radiology 184(3):749–751

    Article  CAS  PubMed  Google Scholar 

  18. Maki Y, Kokubo Y, Nose T, Yoshii Y (1976) Some characteristic findings of isotope cisternograms in children. J Neurosurg 45:56–59

    Article  CAS  PubMed  Google Scholar 

  19. Maytal J, Alvarez LA, Elkin CM, Shinnar S (1987) External hydrocephalus: radiologic spectrum and differentiation from cerebral atrophy. AJR Am J Roentgenol 148:1223–1230

    Article  CAS  PubMed  Google Scholar 

  20. Muenchberger H, Assaad N, Joy P, Brunsdon R, Shores EA (2006) Idiopathic macrocephaly in the infant: long-term neurological and neuropsychological outcome. Childs Nerv Syst 22(10):1242–1248

    Article  PubMed  Google Scholar 

  21. Norrell H, Wilson C, Howieson J et al (1969) Venous factors in infantile hydrocephalus. J Neurosurg 31:561–569

    Article  CAS  PubMed  Google Scholar 

  22. O’Hayon BB, Drake JM, Ossip MG, Tuli S, Clarke M (1998) Frontal and occipital horn ratio: a linear estimate of ventricular size for multiple imaging modalities in pediatric hydrocephalus. Pediatr Neurosurg 29(5):245–249

    Article  Google Scholar 

  23. Oi S, Di Rocco C (2006) Proposal of “evolution theory in cerebrospinal fluid dynamics” and minor pathway hydrocephalus in developing immature brain. Childs Nerv Syst 22:662–669v

    Article  PubMed  Google Scholar 

  24. Rekate HL, Nadkarni TD, Wallace D (2008) The importance of the cortical subarachnoid space in understanding hydrocephalus. J Neurosurg Pediatr 2(1):1–11

    Article  PubMed  Google Scholar 

  25. Turner L (1961) The structure of arachnoid granulations with observations on their physiology and pathophysiological significance. Ann R Coll Surg Engl 29:237–264

    CAS  PubMed  PubMed Central  Google Scholar 

  26. Wiig US, Zahl SM, Egge A, Helseth E, Wester K (2017) Epidemiology of benign external hydrocephalus in Norway-a population-based study. Pediatr Neurol 73:36–41

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Department of Neurosurgery, University Hospital of Tuebingen, Tuebingen, Germany

    Laura V. Sainz, Julian Zipfel, Susanne R. Kerscher & Martin U. Schuhmann

  2. Department of Neurosurgery, University Hospital Fundación Jiménez Díaz of Madrid, Madrid, Spain

    Laura V. Sainz

  3. Department of Pediatric Neurology, University Children’s Hospital of Tuebingen, Tuebingen, Germany

    Annette Weichselbaum & Andrea Bevot

Authors
  1. Laura V. Sainz
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  2. Julian Zipfel
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  3. Susanne R. Kerscher
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  4. Annette Weichselbaum
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  5. Andrea Bevot
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  6. Martin U. Schuhmann
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Correspondence to Laura V. Sainz.

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Sainz, L.V., Zipfel, J., Kerscher, S.R. et al. Cerebro-venous hypertension: a frequent cause of so-called “external hydrocephalus” in infants. Childs Nerv Syst 35, 251–256 (2019). https://doi.org/10.1007/s00381-018-4007-3

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  • DOI: https://doi.org/10.1007/s00381-018-4007-3

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