Microcephaly Clinical Presentation

Updated: Jun 10, 2016
  • Author: James J Reese, Jr, MD, MPH; Chief Editor: Amy Kao, MD  more...
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Presentation

History

The head circumference is measured at birth as well as at regular checks by primary care providers. Microcephaly can present at various ages and can be either static or progressive, depending on the etiology. Additionally, it may or may not be associated with syndromic features, depending on the etiology.

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Physical Examination

For a concern of abnormal head size, the exam should comment on both head shape and head size (in comparison to age and gender matched controls). The head should be measured formally in the standard method. According to the AAN Practice Parameter, “Accurate head circumference (HC) measurement is obtained with a flexible non-stretchable measuring tape pulled tightly across the most prominent part on the back (occiput) and front (supraorbital ridges) of the head.” [3]

The clinician should also note the percentile of head circumference at birth, and if the current head circumference has remained consistent or has changed over time. (For details on the types of head shape changes, please see the Medscape Reference article Pediatric Craniosynostosis.) A head circumference of more than 2 standard deviations below the mean is considered microcephaly; a circumference of more than 3 standard deviations below the mean is classified as severe microcephaly, which should prompt consideration of neuroimaging. [4]  (See image below.)

Illustration of typical head size, Microcephaly, a Illustration of typical head size, Microcephaly, and Severe Microcephaly

Furthermore, comment about syndromic features, developmental level, and presence (or lack of) neurologic deficits should also be made since those features can help dictate which further workup, if any, should be performed.

 

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