Neurology: Plagiocephaly and Brachycephaly Flashcards
What is meant by plagiocephaly and brachycephaly? [1]
What are they a risk of ? [1]
Plagiocephaly and brachycephaly are very common conditions that cause abnormal head shapes in otherwise normal health babies.
- Plagio- translates as oblique, or slanted. Plagiocephaly refers to flattening of one area of the baby’s head.
- Brachy- translates as short. Brachycephaly refers to flattening at the back of the head, resulting in a short head from back to front.
Why does plagiocephaly and brachycephaly typically occur? [1]
These conditions occur where a baby had a tendency to rest their head on a particular point
- resulting in the skull bones and sutures moulding with gravity to create an abnormal head shape. This is called positional plagiocephaly
When investigating plagio / brachycephaly, which muscle should you look to see is shortened? [1] Why? [1]
Look for congenital muscular torticollis (CMT), which is a shortening of the sternocleidomastoid muscle on one side.
- This may be the reason the child always rests on one side of their head.
- Physiotherapy can help with movement exercises to treat the torticollis.
What is meant by spinal muscular atrophy? [1]
How do patients typically present? [4]
Spinal muscular atrophy (SMA) is a rare autosomal recessive condition that causes a progressive loss of motor neurones, leading to progressive muscular weakness.
- Spinal muscular atrophy affects the lower motor neurones in the spinal cord.
- This means there will be lower motor neurone signs: such as fasciculations, reduced muscle bulk, reduced tone, reduced power and reduced or absent reflexes.
There are four categories of spinal muscular atrophy that are numbered from most to least severe. Describe the difference between them [4]
SMA type [] is the most common type.
There are four categories of spinal muscular atrophy that are numbered from most to least severe. SMA type 2 is the most common type.
SMA type 1 has an onset in the first few months of life, usually progressing to death within 2 years.
SMA type 2 has an onset within the first 18 months. Most never walk, but survive into adulthood.
SMA type 3 has an onset after the first year of life. Most walk without support, but subsequently loose that ability. Respiratory muscles are less affected and life expectancy is close to normal.
SMA type 4 has an onset in the 20s. Most will retain the ability to walk short distances but require a wheelchair for mobility. Everyday tasks can lead to significant fatigue. Respiratory muscles and life expectancy are not affected.
Describe the respiratory support that children with SMA type 1 may need [2]
Respiratory support with non-invasive ventilation may be required to prevent hypoventilation and respiratory failure, particularly during sleep.
Children with SMA type 1 may require a tracheostomy with mechanical ventilation, which can dramatically extend life by supporting failing respiratory muscles.
