About the Craniofacial Service
The Craniofacial Unit at Great Ormond Street Hospital (GOSH) is one of four specialist services in England that treat children with congenital and acquired craniofacial conditions.
Around 250 patients are treated each year and approximately 200 surgical procedures undertaken. About 10 per cent of the surgery is complex midfacial surgery including fronto-facial distraction. The unit sees children from newborn through to 18 years old and link in with the adolescent services at University College Hospital (UCH), which includes maxillofacial surgery.
The multidisciplinary team at GOSH is comprised of plastic surgeons, neurosurgeons, neurologists, dentists, ENT (ear, nose and throat) colleagues, ophthalmologists, audiologists, genetics colleagues, speech and language therapists, psychologists, as well as two clinical nurse specialists supporting the children and their families. The unit aims to streamline the service to ensure the child and family receive care and treatment in line with current best practice and which responds early to any problems, to facilitate the best functional, cognitive and psychological outcomes.
Craniosynostosis clinical outcome measures
Craniosynostosis is quite a rare condition that affects how the bones in the skull grow. ‘Cranio’ means head; ‘synostosis’ means fusion. There are different types of craniosynostosis. They all affect the shape of the head. In most cases, it is just head shape that is affected. But in complex cases, the bones in the face – and sometimes hands and feet – are also affected and fuse together too early.
At birth, a baby’s skull is made up of bony plates separated by seams known as ‘sutures’, similar to those in clothing. The sutures are there to help with moulding of the head during delivery, and play a role in shaping the head as it grows. The sutures eventually fuse, forming a protective helmet for the brain – the skull. But with craniosynostosis, a single suture or a number of sutures fuse much earlier than they are supposed to. This can restrict the growth and development of the brain, as well as the shape of the head.
When only one suture is affected, it is called simple or single suture craniosynostosis but when more than one suture is affected, it is called complex craniosynostosis. This may happen as part of a syndrome (collection of symptoms often seen together), and so may be referred to as ‘syndromic’ as well.
Single suture craniosynostosis
When a child has craniosynostosis, the plates fuse together too soon causing an abnormal head shape. Single suture craniosynostosis is the condition where only one suture has fused too soon.
At GOSH, we developed a Patient-Reported Outcome Measure (PROM) and a Patient-Reported Experience Measures (PREM) for conditions comprising non-syndromic single suture craniosynostosis. Measures were designed with specialist colleagues at GOSH and parents/carers to explore parental concerns and decision-making reasons for surgery, and to understand patient and parent experience. The tools were refined and improved using a phased iterative approach. Parents and carers are asked to complete the PROM before surgery and at six weeks and one year after surgery, and to complete the PREM six weeks after surgery.
1. Motivators for surgery
Parents and carers were asked before surgery the importance to them of four outcomes. Fig. 1 shows that almost all said that head shape (n=41/41), forehead shape (n=38/41) and reducing chance of bullying (n=40/41) were important. Of those that answered, around 90 per cent (n=23/25) said developmental progress was important.
Fig. 1 Motivators for surgery
2. Concern score
Before and after surgery, parents/carers were asked to rate their concerns between
- 0 – Not at all
- 1 – A little bit
- 2 – Quite a bit
- 3 – Very much.
Fig. 2 shows the combined concern scores. The combined score can be a maximum of 21 and a minimum of zero. Overall there is a decrease in the concern score for all parents/carers after surgery.
Fig. 2 Concern scores
3. Post-operative outcomes
Parents/carers were asked if overall, surgery had achieved what they hoped it would. Fig. 3.1 shows 90 per cent thought the overall outcome was ‘More than expected’ (47%) or ‘As expected’ (43%), with six per cent saying ‘Less than expected’, and four percent did not answer.
Fig. 3.1 Overall outcomes of surgery
Fig 3.2 shows that over 90 per cent of parents/carers said the surgery achieved improved head shape either ‘Very much’ or ‘Quite a bit’. No parents/carers said ‘Not at all’.
Fig. 3.2 Head shape outcomes
Fig. 3.3 shows that 79 per cent of parent/carers said the surgery achieved improved forehead shape either ‘Very much’ or ‘Quite a bit’. Eight per cent said ‘Not at all’.
Fig 3.3 Forehead shape outcomes
Fig. 3.4 shows that 83 per cent of parents/carers said the surgery had reduced chances of their child being bullied or teased. Six per cent said ‘Not at all’.
Fig 3.4 Bullying/teasing outcomes
Overall, 90 per cent of parents/carers reported that the surgery was worthwhile.
Fig. 3.5 Surgery worthwhile
Six weeks after the operation parents/carers were asked about their experience of care. The results indicate that our communication and care is found to be very good. It also showed us that we can specifically make improvements in supporting our families and patients in feeling more prepared before the operation. We will be using these results to make the identified improvements in our care.
Fig. 4 Experience measures
This information was published in October 2018 and will be updated in October 2019.