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Patient-Reported Speech Outcomes in Patients with Beckwith-Wiedemann Syndrome.
Allison C Hu1, Andrew M George2, Aravind Viswanathan2
1Division of Plastic, Reconstructive, and Oral Surgery, Children's Hospital of Philadelphia, Pennsylvania, PA.
Plastic and Reconstructive Surgery
|March 10, 2026
Summary
This study on Beckwith-Wiedemann Syndrome (BWS) found that while many patients report good outcomes after tongue reduction surgery (TRS), speech function scores indicate persistent challenges. Individualized management is key for optimizing speech development in BWS patients.
Area of Science:
- Pediatric Surgery
- Genetics
- Speech Pathology
Background:
- Macroglossia is a primary feature of Beckwith-Wiedemann Syndrome (BWS), impacting speech and feeding.
- Tongue reduction surgery (TRS) is common for BWS, but its effect on patient-reported speech outcomes is not well understood.
Purpose of the Study:
- To evaluate patient-reported speech outcomes in individuals with BWS.
- To assess the impact of TRS and other factors on speech function in BWS.
Main Methods:
- Prospective study of 36 BWS patients (ages 4-18) using the FACE-Q Craniofacial measures.
- Collected data on clinical characteristics, BWS Index of Macroglossia (BIG), and Intelligibility in Context Scale (ICS).
Main Results:
- Overall favorable outcomes reported, but speech function scores were lower (75.8±19.5).
- Moderate correlation found between ICS and speech function (r=0.697, p=0.001).
- Patients receiving speech therapy showed lower speech function (p=0.014); no significant difference between surgical/non-surgical groups.
Conclusions:
- Speech function remains a challenge for BWS patients despite overall favorable outcomes.
- Multifactorial nature of speech development in BWS requires individualized, long-term management.
- Further research needed to optimize speech outcomes in BWS.
