Cleft clinical outcomes

Clinical outcomes are measurable changes in health, function or quality of life that result from our care. Constant review of our clinical outcomes establishes standards against which to continuously improve all aspects of our practice.

Great Ormond Street Hospital (GOSH) hosts the North Thames Cleft Lip and Palate Service jointly with Broomfield Hospital in Essex. The service is the largest in the UK and the team works across both sites providing treatment to an average of 150–170 new babies per year born with a cleft lip and/or palate.

The multidisciplinary team at GOSH is comprised of surgeons (plastics, maxillofacial and ear, nose and throat); paediatricians; speech and language therapists; orthodontists; paediatric dentists; audiologists; geneticists, psychologists and nurse specialists. We see and treat patients from birth to 21 years as part of the package of care.

The service also treats adults with clefts of the lip and palate from all over the North Thames region at Broomfield Hospital. This includes North London, Essex and South and West Hertfordshire.

We also see children with complex and multiple disorders associated with cleft lip and palate, and have a large caseload of children without a cleft who have speech disorders due to dysfunction of the palate.

A national standard for cleft services is that every baby with a cleft lip and/or palate must be seen within 24 hours of the cleft service receiving the referral.

The visit is an important one, with potential for significant impact on outcome. The visit involves a clinical assessment of the cleft type and a detailed feeding assessment for the new-born baby by the attending nurse, who is a specialist in cleft conditions. The nurse specialist will then instruct caregivers and especially breastfeeding mothers on how to safely feed their baby, and will develop an individually tailored feeding management plan for the child. It is sometimes necessary for infants with cleft conditions to be transferred to hospital for specialist care if there are significant feeding issues. But, advice on feeding given within the first 24 hours can prevent this.

Information gathered at this appointment also helps to identify any additional medical anomalies that may be present, including cardiac and respiratory issues, so that appropriate investigations and onward referrals to other services can be coordinated.

In addition to the benefit of the feeding advice, practical support, and information about what to expect for future treatment, families describe the importance of the reassurance they feel from this first visit.

The table shows that we have maintained our results since 2014/15 with an overall average of 79%. We continue to strive to see every family within 24 hours of referral.

Figure 1.1 Percentage of patients with a cleft who are visited by a clinical nurse specialist within 24 hours, 2014/15 to 2018/19

Table 1.1 Percentage of patients with a cleft who are visited by a clinical nurse specialist within 24 hours, 2014/15 to 2018/19

Timely assessment of hearing in children with a cleft palate enables early intervention such as hearing aids for those who are found to have a correctable hearing impairment. Importantly, early intervention means that the impact on the child’s speech and language development can be minimised. Table 2.1 shows how many of our children with a cleft palate are assessed by the age of one. Some are assessed a little after one year of age but all will be assessed prior to surgery. However, we aim to test all children by the age of one and while our rates are improving, we continue to work towards every child in our service being assessed for hearing impairment before their first birthday.

Figure 2.1 Percentage of children born each year with a cleft palate, and treated at GOSH, who had their hearing assessed before they turned one year old, by year of birth 2011 to 2017

Table 2.1 Percentage of children born each year with a cleft palate, and treated at GOSH, who had their hearing assessed before they turned one year old

The aim of cleft palate repair surgery is to:

• Restore the possibility of normal speech
• Help with feeding
• Help with drainage of the middle ear

Sometimes further surgery is required after a cleft repair. Reasons for further surgery include an unhealed area (fistula or wound breakdown) from the first operation, a palate that is too short or one that has poor muscle function. The need for further surgery is more likely if the cleft is severe or if the child has other medical problems or a syndrome. A syndrome is simply a collection of abnormalities which characteristically occur together in one patient. We record and publish our outcomes for all our patients with a cleft, regardless of whether the cleft occurs in association with a syndrome or not.

The chart below shows patients who had their primary (first) repair between 2010 and 2014. We show data up to 2014 because a proportion of patients operated on after 2014 may have revision surgery. Showing the data in this way enables us to be as accurate as possible about our revision surgery data. The data includes all UK NHS patients with cleft palate, unilateral cleft lip and palate, or bilateral cleft lip and palate who were under 18 months of age at the time of the primary repair.

Figure 3.1 Percentage of patients at North Thames Regional Cleft Service who required secondary or more speech surgery, 2010 to 2014

These figures show that our secondary or more speech surgery (eg cleft palate re-repair or lengthening for scarred or short palates) rate was between 16.5% and 21.4%. This data excludes patients that are not in follow-up at GOSH.

It is also important to show the data by cleft types, as outcomes vary between the types of cleft. The chart below shows patients who had their primary repair between January 2005 and June 2012 (so all children counted are now over five years old). The data includes all our NHS patients with cleft palate, unilateral cleft lip and palate, or bilateral cleft lip and palate who were under 18 months of age at the time of the primary repair.

Figure 3.2 Percentage of children who required secondary or more surgery by 5 years of age dependent upon type of cleft, patients who had their primary repair between Jan 2005 and June 2012

These figures are for patients who had their primary repair between January 2005 and June 2012, all with a 5 year audit follow-up at GOSH. The figures show that the more severe the original cleft eg bilateral cleft lip and palate, the higher the chance of needing more speech surgery in later years to attain the best speech outcomes. This data excludes patients that are not in follow-up at GOSH.

Facial growth can be affected in children with a cleft lip and/or palate. However, surgery to repair the cleft can also hinder the growth of the face due to scarring that may adversely affect the downward and forward growth of the upper jaw. It is therefore important to assess the growth outcome in patients with a cleft to allow the cleft team, and especially the surgeons, to get an early indication of how treatment should progress.

Assessment of the dental bite in 5 year old unilateral cleft lip and palate patients provides jaw outcomes by showing the position of the upper teeth in relation to the lower teeth. A favourable outcome is indicated by upper teeth that bite relatively forward. Upper teeth that bite relatively backwards may require further intervention and it is likely that the cleft team will consider the child for further surgery when they are a young adult.

Dental models taken from 5 year old patients are compared to the Atack 5 year index (Atack et al 1997) and are ranked from 1 (good growth) to 5 (poor growth). All models are sent off and assessed independently for consistency. This index is used nationally in the UK so that cleft units can get feedback about their outcomes and can gauge their outcomes against national averages.

This chart was produced by the Craniofacial Society of Great Britain and Ireland. The numbers along the right of the chart shows the number of models collected.

The chart shows growth outcomes from GOSH of children born with unilateral cleft lip and palate between 2000 and 2012. The groups are determined from benchmarking against historic data for a centre in Oslo, where in the 1990s growth results were regarded as very good:

• Groups I and II are coloured green, representing good growth.
• Groups III are coloured orange, representing moderate growth.
• Groups IV and V are coloured red, representing poor growth. 

Figure 4.1 Growth outcomes, 2000 to 2012

The North Thames Centre has good dental arch growth outcome scores for patients born with unilateral cleft lip and palate between 2000 and 2011 when compared with other UK centres. These scores are consistently higher than the national average (mean).

4.2 National funnel plot of five-year olds (born between 2004-2011) with poor Five Year Old Index scores according to the number of children at each region / unit with index scores

This funnel plot is from the Cleft Registry and Audit NEtwork (CRANE) Annual Report on Cleft Lip and/or Palate 2018 and can be found on page 29 of the report. It shows the proportion of poor Five Year Old Index scores (IV and V) by centre for comparison. GOSH and Chelmsford, which together provide services for the North Thames region, have amongst the lowest rates of poor scores. This comparative national chart and the GOSH chart above both indicate that a high proportion of children now aged five who were treated by our teams had good facial growth and development and are unlikely to need further surgery as young adults.

Referenced with permission of the CRANE Database.

The Peer Assessment Rating (PAR) index is a valid and reliable measure of orthodontic treatment outcome and is the most widely accepted index of its kind.

Assessment of outcomes with the PAR index requires the examination of pre-treatment and post-treatment orthodontic study models. Beginning with the pre-treatment models, a score is given to each feature that does not meet an ideal occlusion (including contact points between upper and lower teeth and how well the back teeth fit together). The scores are then added together to give a total that represents the severity of the malocclusion. The process is then repeated with the post-treatment models. The percentage reduction in PAR score is found by dividing the reduction in PAR score by the pre-treatment PAR score and multiplying by 100. The resulting percentage is then assigned to one of four categories of improvement:

• Great improvement: 70% to 100%
• Improvement: 50% to 69%
• Little improvement: 30% to 49%
• No improvement: <30%

Table 5.1 PAR scores for 2011 to 2018

All patients treated at GOSH who have completed their orthodontic treatment and been assessed at the end of this treatment period are categorised as showing great improvement in their orthodontic occlusion.

One of the primary aims of cleft palate repair is to achieve normal speech by the time of school entry at age five. Routine audit of speech at age five for children with a cleft involving the palate is undertaken by all UK cleft centres, with speech rated through consensus judgement using the Cleft Audit Protocol for Speech – Augmented (CAPS-A) scores. The 16 parameters of speech assessed by CAPS-A include palate function during speech related to structural factors such as the ability of the palate to properly close off the mouth from the nose while speaking, and individual cleft speech characteristics that can affect the clarity and intelligibility of speech.

We report on the proportion of five year-olds meeting four of the nationally agreed Speech Outcome Standards. Data is shown for children who are fully consented, able to participate and have all 16 CAPS-A scores recorded. This includes some children who are not registered in The Cleft Registry and Audit Network (CRANE) database, and therefore data reported elsewhere may differ. For children born since 2010, the data includes children whose cleft is associated with a syndrome.

Our aim is to have as many children as possible with normal speech, and this outcome is achieved when children have normal scores across all 16 CAPS-A speech parameters.

Figure 6.1 Percentage of children with a cleft palate achieving normal speech, children born 2005 to 2011

Table 6.1 Percentage of children with a cleft palate achieving normal speech, children born 2005 to 2011

* Data is for all children who are able to participate in a speech assessment, including those with a cleft associated with a syndrome.

The national standard is for 50 per cent or more of children to achieve normal speech. For children born between 2005 and 2011 this has been met and exceeded every year.

Most children with a cleft palate develop normal speech. However, in some children the palate is unable to completely seal off the nasal cavity during speech and/or there is a small hole (fistula) that does not heal after surgery. This allows air to leak out of the nose while speaking and can lead to difficulties with articulation of sounds or nasal sounding speech. Children with speech issues related to structural problems can require additional surgical treatment and / or speech therapy.

Our aim is to achieve for as many children as possible a good velopharyngeal function during speech (ie the palate sealing off against the back wall of the throat) and to have speech with no evidence of a structure-related problem. This outcome is achieved when children have not needed additional surgery for speech and normal scores for the six parameters in the CAPS-A assessment relating to structural outcomes (resonance, nasal airflow and three individual cleft speech characteristics relating to structural causes).

Figure 6.2 Percentage of children with a cleft palate with no evidence of a structural problem and no history of additional surgery for speech, children born 2005 to 2011

Table 6.2 Percentage of children with a cleft palate with no evidence of a structural problem and no history of additional surgery for speech, children born 2005 to 2011

* Data is for all children who are able to participate in a speech assessment, including those with a cleft associated with a syndrome.

The national standard is for more than 70 per cent of children to have no history of additional surgery for speech and with no evidence of a structural problem. For children born between 2005 and 2011 this has been met and exceeded every year.

For children with speech affected by a structural problem, our aim is that following surgery children have speech with no evidence of a structurally related problem.

Our aim is to have as many children as possible with no cleft-related articulation difficulties (ie speech that is clear and understandable) that requires therapy or surgery. This outcome is achieved when children have normal scores for ten parameters in the CAPS-A assessment relating to clarity and intelligibility.

Figure 6.3 Percentage of children with a cleft palate with no articulation difficulties at five years of age that requires therapy or surgery, children born 2005 to 2011

Table 6.3 Percentage of children with a cleft palate with no articulation difficulties at five years of age that requires therapy or surgery, children born 2005 to 2011

* Data is for all children who are able to participate in a speech assessment, including those with a cleft associated with a syndrome.

The national standard is for more than 50 per cent of children to have no cleft-type articulation difficulties at five years of age that require either therapy or surgery. For children born between 2005 and 2011 this has been met and exceeded each year.

6.4 National funnel plot of five-year olds (born between 2009-2011) with scores suggesting normal speech, according to the number of children at each region / unit with CAPS-A scores

This funnel plot is from the Cleft Registry and Audit NEtwork (CRANE) Annual Report on Cleft Lip and/or Palate 2018 and can be found on page 34 of the report. It shows the proportion of children at each region / unit with scores for all 16 CAPS-A speech parameters. All centres fall within the expected range of results, with GOSH achieving high levels of normal speech in our patients. Additional revision surgery may have contributed to our speech outcomes.

Referenced with permission of the CRANE Database.

This information was published in November 2019.

1. Atack, N.E., Hathorn, I.S., Mars, M. and Sandy, J.R., (1997) The use of study models to assess surgical outcome in cleft lip and palate subjects at the age of 5. European Journal of Orthodontics, 19, pp.165-170.

2. John A., Sell D., Sweeney T., Harding-Bell A. and Williams A. (2006) The Cleft Audit Protocol for Speech—Augmented: A Validated and Reliable Measure for Auditing Cleft Speech. The Cleft Palate–Craniofacial Journal, Vol. 43, No. 3, pp. 272-288

3. Britton L., Albery L., Bowden M., Harding-Bell A., Phippen G. and Sell D. (2014) A Cross-Sectional Cohort Study of Speech in Five-Year-Olds With Cleft Palate +/- Lip to Support Development of National Audit Standards: Benchmarking Speech Standards in the United Kingdom. The Cleft Palate-Craniofacial Journal: Vol. 51, No. 4, pp. 431-451