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Macrocephalies--a differentiated approach.

R H Gooskens1, J Willemse, J A Faber

  • 1Department of Child Neurology, University Hospital Utrecht, The Netherlands.

Neuropediatrics
|August 1, 1989
PubMed
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Macrocephaly in children with normal head growth requires careful assessment. Differentiating megalencephaly from hydrocephalus is crucial for appropriate investigation and management.

Area of Science:

  • Pediatrics
  • Neurology
  • Medical Imaging

Background:

  • Macrocephaly, characterized by an abnormally large head, can be associated with various underlying conditions.
  • Accurate assessment of intracranial volume is essential for diagnosing the cause of macrocephaly.
  • Standard head measurements like occipito-frontal circumference (OFC) may not fully represent intracranial volume.

Purpose of the Study:

  • To evaluate existing criteria for diagnosing macrocephaly in children with normal head growth.
  • To develop a differentiated approach for investigating macrocephalic children.
  • To assess the utility of head measurements in estimating intracranial volume.

Main Methods:

  • A clinical study involving 43 macrocephalic children with normal head growth.

Related Experiment Videos

  • Comparison of head measurements (OFC, Utrecht Head Measure) with CT-scan-derived intracranial volume.
  • Subgroup classification of macrocephalic children based on CT-scan length measurements.

    • Main Results:

    • Occipito-frontal circumference (OFC) explained only about 30% of the variance in intracranial volume.
    • The "Utrecht Head Measure" (OFC-squared x head height) provided a better estimation of intracranial volume.
    • Three subgroups were identified: megalencephaly (n=15), extraventricular obstructive hydrocephalus (n=5), and communicating hydrocephalus (n=23).
    • Megalencephalic children exhibited fewer neurological and physical abnormalities and better cognitive/developmental outcomes compared to hydrocephalic children.

    Conclusions:

    • Head circumference alone is insufficient for accurately assessing intracranial volume in macrocephalic children.
    • The Utrecht Head Measure offers improved estimation of intracranial volume.
    • Distinct clinical presentations differentiate megalencephaly from hydrocephalus, guiding further investigation.
    • Guidelines for assessing macrocephaly should incorporate a differentiated approach for megalencephalic and hydrocephalic patients.