Child's Nervous System

, Volume 35, Issue 1, pp 19–27 | Cite as

Conservative and operative management of iatrogenic craniocerebral disproportion—a case-based review

  • Thomas BeezEmail author
  • Christopher Munoz-Bendix
  • Sebastian Alexander Ahmadi
  • Martina Messing-Jünger
  • Hans-Jakob Steiger
  • Andreas Röhrig
Case-Based Update



Craniocerebral disproportion (CCD) can occur as a sequela after shunting in early infancy. It can be understood as a disorder closely related to slit ventricle syndrome and chronic overdrainage syndrome. Here, we present two exemplary cases and summarize the pathophysiological, diagnostic, and therapeutic approaches to CCD.

Clinical presentation

Two premature babies underwent shunting for posthemorrhagic hydrocephalus and presented in later childhood with recurrent episodes of symptomatic raised intracranial pressure (ICP) at 2 and 8 years of age, respectively.

Diagnosis and management

Both patients had unchanged ventricular size on cranial imaging and fulfilled the clinical diagnostic criteria of CCD. After confirming shunt patency, ICP monitoring was performed to diagnose intermittent intracranial hypertension. Different treatment pathways were pursued: While readjustment of a programmable shunt valve was sufficient to alleviate the raised ICP in the first case, a cranial expansion surgery was necessary in the second case.

Outcome and conclusions

Both children were treated successfully after thorough assessment and careful choice of treatment approaches. This review provides detailed insight into CCD and highlights the importance of individual and critical decision-making in these complex patients.


Intracranial hypertension Shunt Chronic overdrainage Slit ventricles 


Compliance with ethical standards

Conflict of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.


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Copyright information

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

Authors and Affiliations

  1. 1.Department of Neurosurgery, Medical FacultyHeinrich-Heine-UniversityDüsseldorfGermany
  2. 2.Department of NeurosurgeryAsklepios Children’s HospitalSankt AugustinGermany

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