Beckwith-Wiedemann syndrome 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.

About the Beckwith-Wiedemann syndrome service

Beckwith-Wiedemann syndrome (BWS) is a congenital (present at birth) overgrowth syndrome that occurs in approximately one in 15,000 births. A syndrome is a collection of features often seen together. BWS is variable – some children have a number of features of the condition, others have only a few. The condition is named after the two doctors who recognised and described it in the 1960s.

One of the most common features of the condition is macroglossia (large tongue size). Macroglossia may cause difficulties with feeding, speech, the development of the teeth and jaws, and increased drooling.

GOSH provides a national specialised service for children who have macroglossia associated with BWS. The aim of our service is to prevent or resolve the problems associated with macroglossia and to enable the best functional outcome. Some children may require tongue reduction surgery.

Below are our clinical outcomes for tongue reduction surgery and the specialist speech and language therapy we provide to treat macroglossia associated with BWS.

Macroglossia clinical outcome measures

The aim of tongue reduction surgery is to reduce the length and width of the tongue so it sits comfortably within the mouth and related symptoms are resolved.

We measure immediate surgical outcomes by:

  • Start of oral feeding after surgery
  • Length of hospital stay
  • Surgical complications

1. Oral feeding start

We measure the time it takes for our patients to return to oral feeding after surgery. As patients will have their operation at different times of the day, we count as day one the first day after surgery. The table below shows the average (mean), which has improved each year for the last three years.

Year Average number of days to return to oral feeding
2015-16 4.1
2016-17 1.6
2017-18 1.3

2. Surgical complications

We measure our surgical complications using a well-established classification system called Clavien-Dindo.

Year No complications Minor complications
2015-16 13 1
2016-17 16 2
2017-18 24 0

The grading is from Type 1 to Type 5, with 1 being the most minor and requiring no intervention. There were three Type 1 complications in the last three years, which were minor wound healing delays or a length of stay of longer than six days. There have been no surgical site infections in the last three years.

3. Length of hospital stay

We measure the length of inpatient hospital stay as another measure of the quality of our care. Days are measured from the day after surgery up to and including the day of discharge. The table below shows the average (mean) length of stay, which has improved each year for the last three years.

Year

Average length of stay in days
2015-16 5.8
2016-17 3.5
2017-18 3.0

We measure the functional success of surgery and our speech and language therapy input by:

  • Resting tongue position
  • Frequency of tongue protrusion
  • Severity of drooling
  • Severity of feeding difficulties
  • Speech

The data below was collected by the specialist speech and language therapists during assessments approximately three months before and three to six months after tongue reduction surgery between April 2012 and March 2018. 70% of all the children referred to the service during this period were appropriate for tongue reduction surgery.

4. Resting tongue position before and after tongue reduction surgery

Fig 4.1 Resting tongue position before and after tongue reduction surgery, 2012/13 to 2017/18

Fig 4.1 Resting tongue position before and after tongue reduction surgery, 2012/13 to 2017/18

The above chart shows that prior to surgery the majority of children (94% total for the six years) had a resting tongue position that was on or over lip, causing a range of difficulties. After surgery, all had significant improvement and 89% had the expected resting tongue position within the dental arch.

5. Frequency of tongue protrusion before and after tongue reduction surgery

Fig 5.1 Frequency of tongue protrusion before and after tongue reduction surgery, 2012/13 to 2017/18

Fig 5.1 Frequency of tongue protrusion before and after tongue reduction surgery, 2012/13 to 2017/18

A total of 87% of the children protruded their tongue most of the time or constantly. After surgery, 100% had improvement in each of the years, with 81% in total never protruding their tongue in relation to their macroglossia condition.

6. Severity of drooling before and after tongue reduction surgery

Fig 6.1 Severity of drooling before and after tongue reduction surgery, 2012/13 to 2017/18

Fig 6.1 Severity of drooling before and after tongue reduction surgery, 2012/13 to 2017/18

Prior to surgery, 58-80% of children had severe or moderate drooling. After surgery, 83-100% had mild drooling or no drooling, with an average of 95% across all years.

7. Severity of feeding difficulties before and after tongue reduction surgery

Fig 7.1 Severity of feeding difficulties before and after tongue reduction surgery, 2012/13 to 2017/18

Fig 7.1 Severity of feeding difficulties before and after tongue reduction surgery, 2012/13 to 2017/18

Prior to surgery, 89-100% of children had oral stage feeding difficulties related to macroglossia. These included spilling of food or drink from the mouth, eating or drinking with the tongue protruded, difficulties with taking a mouthful of food or drink, difficulties preparing and manipulating food to swallow and increased feeding times. After surgery, more than 80% in each of the years had no oral stage feeding difficulties related to macroglossia, with 88% averaged across all six years.

8. Speech before and after tongue reduction surgery

Fig 8.1 Speech before and after tongue reduction surgery, 2012/13 to 2017/18

Fig 8.1 Speech before and after tongue reduction surgery, 2012/13 to 2017/18

The above chart shows that prior to surgery, in children who had started to develop speech, the large protruding tongue affected the production of the anterior sounds made with the lips and tongue (blade/lingolabial speech production). After surgery, these speech patterns related to the macroglossia were eliminated and overall the anterior speech sounds were produced in the expected way for the children’s age.  

The outcomes data for the Beckwith-Wiedemann syndrome service demonstrates significant improvement in resting tongue position, frequency of tongue protrusion, drooling and feeding difficulties, and speech development.

This information was published in January 2019.

References

Shipster, C., Oliver, B. & Morgan, A. Speech and oral motor skills in children with Beckwith Wiedemann Syndrome: Pre- and post-tongue reduction surgery. Advances in Speech Language Pathology 8, 45–55 (2006).

Shipster, C., Morgan, A. & Dunaway, D. Psychosocial, feeding, and drooling outcomes in children with Beckwith Wiedemann syndrome following tongue reduction surgery. The Cleft Palate-Craniofacial Journal 49, e25-34 (2012).

Dindo, D., Demartines, N. & Clavien P. A. Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Annals of Surgery 240, 205-213 (2004).

Prendeville, N., Sell, D. Tongue Reduction Surgery and Feeding Difficulties in Infants With Beckwith Wiedemann Syndrome: A Case Series. The Cleft Palate-Craniofacial Journal, (2018) doi: 10.1177/1055665618794070. [Epub ahead of print].