Neurodevelopmental profile in children with benign external hydrocephalus syndrome. A pilot cohort study



The management of children with benign external hydrocephalus (BEH) remains controversial. Most BEH children do well in the long-term, but a substantial number have temporary or permanent psychomotor delays. The study aims to assess the prevalence and pattern of neurodevelopmental delay in a cohort of children with BEH.


We conducted a cohort study of 42 BEH children (30 boys and 12 girls, aged 6 to 38 months). A pediatric neurosurgeon performed a first clinical evaluation to confirm/reject the diagnosis according to the clinical features and neuroimaging studies. Two trained evaluators assessed the child’s psychomotor development using the third edition of the Bayley Scales of Infant and Toddler Development (Bayley-III). Developmental delay was defined as a scaled score < 7 according to the simple scale and/or a composite score < 85.


Eighteen children (43%) presented statistically lower scores in the gross motor and composite motor of the Bayley-III scales compared to their healthy peers.


In BEH, it is important to establish a diagnostic algorithm that helps to discriminate BEH patients that have self-limiting delays from those at risk of a persistent delay that should be referred for additional studies and/or interventions that might improve the natural evolution of a disease with high impact on the children and adult’s quality of life.

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Fig. 1

Data availability

The data that support the findings of this study are available from the corresponding author, upon reasonable request.

Code availability

Not applicable.


Bayley III:

Third Edition of Bayley Scales of Infant and Toddler Development


benign external hydrocephalus


cerebrospinal fluid


head circumference


intracranial pressure






standard deviation


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We want to especially thank the families who collaborated positively by bringing their children to the evaluations and participating during the test administration and our young patients who did the effort of doing their best performance. Without their implication this work would not have been possible. The authors acknowledge Ester Carceller Delgado and the nurses of the maternal investigation unit for their help in the management of the clinical spaces.


UNINN is supported by a Grant from the Departament d'Universitats, Recerca i Societat de la Informació de la Generalitat de Catalunya (SGR 2014-844). The work has been supported by the European Union’s Horizon 2020 research and innovation programme under the Marie Sklodowska-Curie grant agreement No 675332; Fondo de Investigación Sanitaria (Instituto de Salud Carlos III) grant PI18/00468 and DTS16/00087, co-financed by the European Regional Development (ERDF); ministerio de Economía y Competitividad /FEDER (PHOTODEMENTIA, DPI2015-64358-C2-1-R); the “Severo Ochoa” Programme for Centers of Excellence in R&D (SEV-2015-0522); the Obra social “la Caixa” Foundation (LlumMedBcn); Institució CERCA, AGAUR-Generalitat (2017 SGR 1380), RIS3CAT (001-P-001682 CECH); FEDER EC and LASERLAB-EUROPE IV; KidsBrainIT (ERA-NET NEURON); Fundació La Marató de TV3 (201709.31 and 201724.31).

Author information




MA. Poca and J. Sahuquillo had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Author contributions to the study and manuscript preparation include the following. Conception and design: Poca and Sahuquillo. Clinical supervision of the patients’ recruitment: Durduran, Maruccia, Paredes, Poca, Rosas, and Sahuquillo. Acquisition of data: Gomàriz, Maruccia, Poca, and Rosas. Analysis and interpretation of data: Durduran, Maruccia, Poca, and Sahuquillo. Statistical analysis: Maruccia, Poca, and Sahuquillo. Drafting the article: Durduran, Maruccia, Poca, and Sahuquillo. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Poca, Sahuquillo. Study supervision: Poca, Sahuquillo.

Corresponding author

Correspondence to Federica Maruccia.

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The study received ethical approval from the VHUH Ethics Committee (PR-ATR-402/2017) and was carried out in accordance with the Code of Ethics of the World Medical Association (Declaration of Helsinki).

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Written informed consent was obtained from parents/guardians prior to the child’s enrolment in the study.

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The authors declare no competing interest.

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Maruccia, F., Gomáriz, L., Rosas, K. et al. Neurodevelopmental profile in children with benign external hydrocephalus syndrome. A pilot cohort study. Childs Nerv Syst (2021).

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  • Bayley III scales
  • Child development
  • Psychomotor assessment
  • Macrocephaly