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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 Most concerning: 3 Standard Deviations above or below the mean >97th Percentile: Macrocephaly <3rd Percentile: Microcephaly Special Charts Down Syndrome Williams Syndrome Achondroplasia VLBW Microcephaly: Small Head Macrocephaly: Large Head Micrencephaly: Small Brain Megalencephaly: Large Brain Brain growth determines ultimate cranium size 2.5% of all children Some with no neurologic problems Asymptomatic familial microcephaly Family history of small head size Normal development Normal neuro exam Primary (genetic) Usually present at birth Secondary (environmental) Present at birth Develop later from postnatal insult Autosomal Recessive Brain is small Otherwise normal architecture Mental retardation Otherwise unremarkable neuro exam 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 Rett Syndrome Rubenstein Taybi Syndrome Broad Thumb-Hallux Syndrome Cornelia de Lange Miller Dieker Prenatal Causes Drugs and alcohol, irradiation, intrauterine infections Perinatal asphyxia Severe malnutrition Presenting signs/symptoms vary with age Communicating vs noncommunicating Benign hydrocephalus H.C. normal or large at birth Increases to 98%ile, then parallels chart Normal neuro exam and development 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 Metabolic Inborn errors of metabolism Most autosomal recessive Significant dev delay with regression Mucopolysaccharidoses Anatomic Increased number of cells Sotos syndrome (overgrowth syndrome) Neurocutaneous syndromes Most with developmental delay Must entertain the worst Space-occupying lesions Tumor AVM Hemorrhage Open fontanelle Ultrasound MRI Gives best information on brain parynchyma Best at posterior fossa CT Intracranial calcifications Hemorrhage Skull