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Head Circumference

Head Circumference. Head Circumference. Average at birth 35cm Normal Growth: 1cm/month for 1 st 6 months ½ cm/month from 6 months to 1 year Especially important first 3 years of age. Head Circumference. Most concerning: 3 Standard Deviations above or below the mean

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Head Circumference

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  1. Head Circumference

  2. Head Circumference • Average at birth 35cm • Normal Growth: • 1cm/month for 1st 6 months • ½ cm/month from 6 months to 1 year • Especially important first 3 years of age

  3. Head Circumference • Most concerning: 3 Standard Deviations above or below the mean • >97th Percentile: Macrocephaly • <3rd Percentile: Microcephaly • Special Charts • Down Syndrome • Williams Syndrome • Achondroplasia • VLBW

  4. Head Circumference • Microcephaly: Small Head • Macrocephaly: Large Head • Micrencephaly: Small Brain • Megalencephaly: Large Brain • Brain growth determines ultimate cranium size

  5. Microcephaly • 2.5% of all children • Some with no neurologic problems • Asymptomatic familial microcephaly • Family history of small head size • Normal development • Normal neuro exam

  6. Microcephaly • Primary (genetic) • Usually present at birth • Secondary (environmental) • Present at birth • Develop later from postnatal insult

  7. Primary Microcephaly Vera • Autosomal Recessive • Brain is small • Otherwise normal architecture • Mental retardation • Otherwise unremarkable neuro exam

  8. Microcephaly w Anatomic Abnormalities • Abnormal neuroimaging • Holoprosencephaly- failure of forebrain to divide into hemispheres. • Midline facial defects • Disruption of hypothalamic-pituitary axis • Lissencephaly- smooth brain • Seizures, spasticity, global dev delay

  9. Microcephaly: Syndromes Rett Syndrome

  10. Microcephaly: Syndromes Rubenstein Taybi Syndrome Broad Thumb-Hallux Syndrome

  11. Microcephaly: Syndromes Cornelia de Lange

  12. Microcephaly: Syndromes Miller Dieker

  13. Secondary Microcephaly • Prenatal Causes • Drugs and alcohol, irradiation, intrauterine infections • Perinatal asphyxia • Severe malnutrition

  14. Macrocephaly

  15. Hydrocephalus • Presenting signs/symptoms vary with age • Communicating vsnoncommunicating

  16. Hydrocephalus • Benign hydrocephalus • H.C. normal or large at birth • Increases to 98%ile, then parallels chart • Normal neuro exam and development

  17. Megalencephaly • Benign or idiopathic (AKA Familial Macrocephaly) • Normal neuro exam • No increased ICP • No fluid collection • Family history of large heads • May have some subtle difficulty with coordination

  18. Megalencephaly • Metabolic • Inborn errors of metabolism • Most autosomal recessive • Significant dev delay with regression • Mucopolysaccharidoses

  19. Megalencephaly • Anatomic • Increased number of cells • Sotos syndrome (overgrowth syndrome) • Neurocutaneous syndromes • Most with developmental delay

  20. Macrocephaly

  21. Macrocephaly • Must entertain the worst • Space-occupying lesions • Tumor • AVM • Hemorrhage

  22. Imaging • Open fontanelle • Ultrasound • MRI • Gives best information on brain parynchyma • Best at posterior fossa • CT • Intracranial calcifications • Hemorrhage • Skull

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