Large Head

Large head
Is the head actually large?

• The clinician should accurately measure and plot occipito-frontal circumference
(OFC) using appropriate non-stretch tape measure.
• The OFC extends from the most prominent part of the glabella to the most
prominent posterior area of the occiput or the largest measureable circumference.
• The OFC can be affected by thick hair and cranial bone deformations or
hyperostosis.
• Height and weight should be plotted simultaneously as the OFC may be within
normal centiles, but significantly discordant with body growth.
• If OFC is within normal centiles, but the head seems apparently large, then
consider late intrauterine growth retardation, skeletal dysplasias for example,
osteogenesis imperfecta, and conditions causing frontal bossing, for example, ricket
Are there any serial measurements?
• Often the birth OFC and subsequent measurements are available from
neonatal records and parent-held child health surveillance records.
• Is the OFC crossing centiles upwards? This does not necessarily
indicate a pathological cause as it may occur in benign
FM/megalencephaly but should raise concern regarding raised ICP
Are there any high-risk factors that are known to be associated
with underlying causes of a large head in infancy?
• ▸ History of other immediate family member/s having asymptomatic large head
• ▸ Ex-preterm baby with history of intraventricular hemorrhage
• ▸ Previous history of subdural hemorrhage
• ▸ Neonatal meningitis
• ▸ Presence of non-cranial congenital anomalies can increase risk of brain
anomalies and/or hydrocephalus, for example, spina bifida
• ▸ Family history of genetic syndromes associated with macrocephaly for
example, neurofibromatosis type 1
• ▸ Relevant family history of developmental problems, for example, autism or
learning disability
• Ask three questions:
• 1. Is the child totally normal?
• 2. Is he neurologically normal but having evidence of systemic disease?
• 3. Does he have abnormal neurology?
Are there any signs or symptoms of raised
ICP
• Large bones
• better referred to as ‘bony disorders’:
•
Achondroplasia.
•
Rickets.
.
Osteogenesis imperfecta (OI).

•
Chronic haemolytic anaemias.

• Large brain
• Large ventricles and/or subarachnoid spaces
• Large brain (increased brain substance)—generalised megalencephaly:
•
Sotos syndrome (cerebral gigantism).

1.
Neurocutaneous syndromes:
2.
•
Neurofibromatosis type 1 (NF-1).
(a)
•
Tuberous sclerosis (TS).
(b)
•
Klippel–Trenauney–Weber syndrome (K–T–W).
(c)
•
Sturge–Weber syndrome (S–W).
(d)
Inherited metabolic disorders:

3.
Lipidoses (e.g. Tay–Sachs disease).

(a)
•
Mucopolysaccharidoses (MPS).
(b)
•
Leukodystrophies; for example, Alexander disease or Canavan disease (also
(c)
• called ‘spongy degeneration’, actually a mitochondrial encephalomyelopathy).

• Large brain (increased brain substance)—localised enlargement:
•
1. Cerebral tumours; for example, glioma or ependymoma.
•
2. Cerebral abscess
• Large ventricles and/or subarachnoid spaces: due to excessive
amounts of CSF;that is, hydrocephalus.
Obstructive (‘non-communicating’ hydrocephalus); for example, aqueduct
• stenosis or posterior fossa tumours.
• Failure of CSF absorption (‘communicating hydrocephalus’); for
example,
• meningeal adhesions after meningitis.
• Overproduction of CSF (also ‘communicating’); for example, choroid
plexus papilloma
• Large bleed: subdural haematoma (unilateral or bilateral), not
infrequently due to non-accidental injury (NAI).
General observations
• Note the child’s level of alertness, movement
• any obvious features of syndromal diagnoses or neurocutaneous
disorders
• Look at the head for signs suggestive of hydrocephalus (e.g. ‘sun-
setting’ of the eyes, and prominent scalp veins
• Skeletal anomalies
• Growth parameters
• Movement
• Skin
• Palpate carefully for shunts
• there may be more than one
• if present,
• trace shunt tubing,
• inspect the chest and abdomen for scars indicating ventriculoatrial or ventriculoperitoneal
shunts.
• While palpating, interact with the child, watching eye movements and
responsiveness
Head
• Inspect
• Measure
• Percentiles
• Shape
• *Look at back at this point*
• Palpate
• Auscultate
Eyes
• Inspect
• • Visual acuity
• • Visual fields
• • External ocular movements
• • Pupils
• • Fundi
Back
• Inspect
• • Midline scar (spina bifida)
• • Scoliosis (associated spina bifida,NF-1)
Lower limbs
• Full examination of motor system

• • Upper motor neuron signs
• (hydrocephalus, intracranial tumor)
• • Lower motor neurone signs (spina bifida, leukodystrophies)
• • Cerebellar signs (D-W)
Upper limbs
• Full motor examination (as with lower limbs)

Developmental assessment
• Gross motor
• Fine motor
• Hearing
Abdomen
• Hepatosplenomegaly (MPS)
• The most important things to remember in this case are the following:
• Always measure the head yourself, until a constant result around the largest diameter is
obtained (usually three times is enough).
•
Always measure the parents’ heads (in a similar fashion).
.
•
Always request the progressive percentiles of the child (the parents’ percentile charts will
.
not be available).
•
Always examine the back, to avoid missing spinal dysraphism.
.
•
Always examine the lower limbs before the upper limbs, as the lower limbs are first
.
affected in hydrocephalus, because the tracts supplying them run closer to the ventricles.
•
Always examine the eye movements, in particular the upward gaze (for Parinaud
.
syndrome) and lateral rectus function (for raised intracranial pressure compressing the
sixth nerve).

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Large head

Is the head actually large?

Osteogenesis imperfecta (OI).

Chronic haemolytic anaemias.

Sotos syndrome (cerebral gigantism).

Inherited metabolic disorders:

Lipidoses (e.g. Tay–Sachs disease).

• called ‘spongy degeneration’, actually a mitochondrial encephalomyelopathy).

• Full examination of motor system

• Full motor examination (as with lower limbs)

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