Child's Nervous System

, Volume 35, Issue 2, pp 251–256 | Cite as

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

  • Laura V. SainzEmail author
  • Julian Zipfel
  • Susanne R. Kerscher
  • Annette Weichselbaum
  • Andrea Bevot
  • Martin U. Schuhmann
Original Paper



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.


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.


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.


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.


Benign idiopathic macrocephaly Subarachnoid space Venous sinuses MR phlebography 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Pediatric Neurosurgery, Department of NeurosurgeryUniversity Hospital of TuebingenTuebingenGermany
  2. 2.Department of NeurosurgeryUniversity Hospital Fundación Jiménez Díaz of MadridMadridSpain
  3. 3.Department of Pediatric NeurologyUniversity Children’s Hospital of TuebingenTuebingenGermany

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