Comparative Study of Pharyngeal Flap Outcomes between Children With 22q11.2 Deletion Syndrome and Non-Syndromic Cleft Lip and Palate

Alice T Wang; Anne F Hseu; Steven J Staffa; Roseanne E Clark; John G Meara; Roger C Nuss; Ingrid M Ganske; Carolyn R Rogers-Vizena

doi:10.1097/PRS.0000000000010854

Comparative Study of Pharyngeal Flap Outcomes between Children With 22q11.2 Deletion Syndrome and Non-Syndromic Cleft Lip and Palate

Plast Reconstr Surg. 2023 Jun 20. doi: 10.1097/PRS.0000000000010854. Online ahead of print.

Authors

Alice T Wang¹, Anne F Hseu^{1

2}, Steven J Staffa¹, Roseanne E Clark², John G Meara^{1

3}, Roger C Nuss^{1

2}, Ingrid M Ganske^{1

3}, Carolyn R Rogers-Vizena^{1

3}

Affiliations

¹ Harvard Medical School; Boston, Massachusetts, USA.
² Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital; Boston, Massachusetts, USA.
³ Department of Plastic and Oral Surgery, Boston Children's Hospital; Boston, Massachusetts, USA.

PMID: 37337330
DOI: 10.1097/PRS.0000000000010854

Abstract

Background: Management of velopharyngeal insufficiency (VPI) in 22q11.2 deletion syndrome (22q) is challenging. This study compares pharyngeal flap outcomes in children with 22q to those with non-syndromic cleft lip and palate (CLP) to assess risk of poor speech outcomes and negative sequelae.

Methods: Children with 22q or CLP treated with pharyngeal flap through a multidisciplinary VPI clinic between 2009 and 2020 were retrospectively reviewed. Pre- and postoperative speech assessments, perioperative characteristics, and complications were identified.

Results: 36 children with 22q and 40 with CLP were included. Age at surgery (p=0.121), pre-operative velopharyngeal competence score (VPC) (p=0.702), and pre-operative resonance (p=0.999) were similar between groups. Pharyngeal flaps were wider (p=0.038*) and length of stay longer in the 22q group (p=0.031*). On short term follow 4 months after surgery, similar speech outcomes were seen between groups. At long term follow up >12 months after surgery, 86.7% 22q v. 100% CLP (p=0.122) had improvement in velopharyngeal function, however fewer children with 22q (60.0%) achieved a completely "competent" VPC score compared to those with CLP (92.6%) (p=0.016*). Nasal regurgitation improved for both groups, with a greater improvement in those with 22q (p=0.026*). Revision rate (p=0.609) and new onset OSA (0.999) were similar between groups.

Conclusion: Children with 22q have improved speech after pharyngeal flap, but may be less likely to reach normal velopharyngeal function over the long term than those with CLP; however, negative sequelae do not differ. Improvement in nasal regurgitation is a uniquely positive outcome in this population.