Beckwith-Wiedemann syndrome clinical outcomes

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 can 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 surgical outcomes for tongue reduction surgery and functional clinical outcomes for children following tongue reduction surgery. The surgical outcomes are collected by the surgical team. The functional clinical outcomes are collected by the specialist speech and language therapists who assess the children with macroglossia associated with BWS both before and after surgery.

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
• sength of hospital stay
• surgical complications

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 to below two days from 2016/17 to 2021/22.

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

The grading is from Type 1 to Type 5, with 1 being the most minor and requiring no intervention. There were five Type 1 complications in the four years 2015/16 to 2018/19. These were minor wound healing delays or a length of stay of longer than six days. There were no surgical site infections in these seven years.

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 decreased to three days or below in the two years from 2020/21 to 2021/22.

We measure the functional success of surgery by looking at outcomes in the following areas:

• 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 2022. 202 children underwent tongue reduction surgery during this 10 year period.

80% of all the children referred to the service during this period were appropriate for tongue reduction surgery.

Figure 4.1 Resting tongue position before and after tongue reduction surgery, 2012/13 to 2021/22

The above chart shows that prior to surgery, 200 of the 202 children (99%) had a resting tongue position that was either over the lower lip; resting on the lower lip; or resting over the lower teeth and in the lower lip. This atypical resting tongue position caused a range of difficulties. After surgery, almost all the children had significant improvement and 183 of the 202 children (91%) had the expected resting tongue position within the dental arch.

Figure 5.1 Frequency of tongue protrusion before and after tongue reduction surgery, 2012/13 to 2021/22

A total of 177 (88%) children protruded their tongue most of the time or constantly before surgery. After surgery, almost all children showed improvement with 147 (73%) children never protruding their tongue, and a further 49 (24%) children protruding their tongue only occasionally.

Figure 6.1 Severity of drooling before and after tongue reduction surgery, 2012/13 to 2021/22

Prior to surgery, 131 (65%) of children had severe or moderate drooling. After surgery 128 (63%) had no drooling, and 61(30%) had mild drooling.

Figure 7.1 Severity of feeding difficulties before and after tongue reduction surgery, 2012/13 to 2021/22

Prior to surgery, 192 (95%) of children in total 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, 178(88%) of children had no oral stage feeding difficulties related to macroglossia, and there was a reduction in severity and quantity of oral stage feeding difficulties for 24 (12%) children who still had some oral stage feeding difficulties.

Figure 8.1 Speech before and after tongue reduction surgery, 2012/13 to 2021/22

The above chart shows that prior to surgery, 18 children (11%) had not started to develop speech. In 184 out of 202 children (91%) who had started to produce speech; the large protruding tongue affected the production of the anterior sounds made with the lips and tongue causing anterior placement articulation errors (blade/lingolabial, interdental and dental speech production). After surgery, the speech patterns related to the macroglossia were either eliminated or reduced in severity for all. Post-surgically, for 110 children out of 202 (54%) 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 production in the 3 to 6 months following tongue reduction surgery.

Our team is committed to achieving good outcomes for the children who undergo tongue reduction surgery. The data we gather enables us to conduct an evidence based practice and make changes where indicated. It also enables us to give our families accurate information about what improvements can be expected 3 to 6 months following the surgery.

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, (2019) May;56(5):679-689.