head shape or size abnormalities - NICE guidance - suspected neurological conditions - recognition and referral

Last edited 05/2019 and last reviewed 04/2022

Head shape or size abnormalities

Summary points from NICE guidance relating to head shape and size abnormalities in children are:

Children with dysmorphic features and developmental delay

• refer urgently to paediatric services children with dysmorphic features and developmental delay.

Children aged under 4 years

For all children aged under 4 years with suspected abnormal head shape or size:

• take 3 consecutive measurements of the child's head circumference at the same appointment, using a disposable paper tape measure
• plot the longest of the 3 measurements on a standardised growth chart, corrected for gestational age
• if the child's head circumference is below the 2nd centile, refer for paediatric assessment.

Offer follow-up measurements if needed, according to clinical judgement and taking the child's age into account.

For children with a head circumference measurement that differs by 2 or more centile lines from a previous measurement on a standardised growth chart (for example, an increase from the 25th to the 75th centile, or a decrease from the 50th to the 9th centile):

refer to paediatric services for assessment and cranial imaging to exclude progressive hydrocephalus or microcephaly or

refer urgently to paediatric services if the child also has any of the following signs or symptoms of raised intracranial pressure:

• tense fontanelle
• sixth nerve palsy
• failure of upward gaze ('sunsetting')
• vomiting
• unsteadiness (ataxia)
• headache

For children with a head circumference above the 98th centile that has not changed by more than 2 centile lines from the previous measurement on a standardised growth chart, who are developing normally and who have no symptoms of raised intracranial pressure:

• note the head size of the biological parents, if possible, to check for familial macrocephaly
  
  
• if familial macrocephaly is likely, do not routinely refer the child in the absence of any other problem.

Babies aged under 1 year with plagiocephaly

For babies aged under 1 year whose head is flattened on one side (plagiocephaly):

be aware that positional plagiocephaly (plagiocephaly caused by pressure outside the skull before or after birth) is the most common cause of asymmetric head shape

measure the distance between the outer canthus of the baby's eye and the tragus of their ear on each side

if the measurements differ, confirm positional plagiocephaly and do not routinely refer if the baby is developing normally

if the measurements are the same, suspect unilateral premature closure of lambdoid suture and refer to paediatric services.

Advise parents or carers of babies with positional plagiocephaly that it is usually caused by the baby sleeping in one position and can be improved by changing the baby's position when they are lying, encouraging the baby to sit up when awake, and giving the baby time on their tummy.

Reference:

• NICE (May 2019). Suspected neurological conditions: recognition and referral