Presenter Status
Fellow
Abstract Type
Research
Primary Mentor
Jill Arganbright, MD
Start Date
16-5-2025 11:30 AM
End Date
16-5-2025 1:30 PM
Presentation Type
Poster Presentation
Description
Introduction (including objectives): Velopharyngeal dysfunction (VPD) is one of the most common clinical features of 22q11.2 deletion syndrome (22q11.2DS) and often requires surgical intervention; due to the multifactorial nature of VPD, these patients present unique and often complex surgical challenges. Prior studies have generally demonstrated poorer speech outcomes postoperatively in children with 22q11.2DS. The posterior pharyngeal flap (PPF) is often considered first-line surgical choice for correction of VPD in children with 22q11.2DS. The aim of this study is to compare PPF outcomes for children with 22q11.2DS to those without.
Methods: Retrospective review for pediatric patients diagnosed with VPI who underwent PPF surgery between July 2014 and July 2024. Data was collected from a single pediatric institution and included demographics, past medical history, surgical and postoperative details, complications, and resonance speech outcomes.
Results: 77 pediatric patients underwent PPF for management of VPI, of which 32 had 22q11.2DS and 45 did not (non-22q). Congenital heart disease and developmental delay were more common in the 22q11.2DS group. No difference was found for age at time of surgery between 22q11.2DS (7.7 years) and non-22q (7.4 years). Length of stay was significantly longer for those with 22q11.2DS compared to those without, averaging 4 and 3 days respectively (p=0.002). Complication rates were similar; each group had 1 patient with postoperative bleeding and 1 patient with an unplanned PICU admission for airway obstruction. Neither group had hospital readmissions. Postoperative hypocalcemia was seen in 18/32 (56%) of 22q11.2DS patients. Review of the postoperative speech outcomes showed improvement in resonance for 87% in the 22q11.2DS group and 81% in the non-22q group. One (3%) patient with 22q11.2 DS required secondary speech surgery compared to six (13%) of those in the the non-22q group.
Discussion: Children with 22q11.2DS who underwent PPF for VPI had overall similar outcomes to those without the syndrome. Length of stay was significantly longer for children with 22q11.2DS, which may be useful in preoperative family counseling. Postoperative hypocalcemia was common for patients with 22q11.2DS and providers should consider checking calcium on postoperative day one. Larger prospective studies are needed to further investigate the impact of 22q11.2DS on PPF outcomes.
Included in
Higher Education and Teaching Commons, Medical Education Commons, Pediatrics Commons, Science and Mathematics Education Commons
Posterior pharyngeal flap for the management of velopharyngeal dysfunction: comparing outcomes for children with 22q11.2 deletion syndrome to those without
Introduction (including objectives): Velopharyngeal dysfunction (VPD) is one of the most common clinical features of 22q11.2 deletion syndrome (22q11.2DS) and often requires surgical intervention; due to the multifactorial nature of VPD, these patients present unique and often complex surgical challenges. Prior studies have generally demonstrated poorer speech outcomes postoperatively in children with 22q11.2DS. The posterior pharyngeal flap (PPF) is often considered first-line surgical choice for correction of VPD in children with 22q11.2DS. The aim of this study is to compare PPF outcomes for children with 22q11.2DS to those without.
Methods: Retrospective review for pediatric patients diagnosed with VPI who underwent PPF surgery between July 2014 and July 2024. Data was collected from a single pediatric institution and included demographics, past medical history, surgical and postoperative details, complications, and resonance speech outcomes.
Results: 77 pediatric patients underwent PPF for management of VPI, of which 32 had 22q11.2DS and 45 did not (non-22q). Congenital heart disease and developmental delay were more common in the 22q11.2DS group. No difference was found for age at time of surgery between 22q11.2DS (7.7 years) and non-22q (7.4 years). Length of stay was significantly longer for those with 22q11.2DS compared to those without, averaging 4 and 3 days respectively (p=0.002). Complication rates were similar; each group had 1 patient with postoperative bleeding and 1 patient with an unplanned PICU admission for airway obstruction. Neither group had hospital readmissions. Postoperative hypocalcemia was seen in 18/32 (56%) of 22q11.2DS patients. Review of the postoperative speech outcomes showed improvement in resonance for 87% in the 22q11.2DS group and 81% in the non-22q group. One (3%) patient with 22q11.2 DS required secondary speech surgery compared to six (13%) of those in the the non-22q group.
Discussion: Children with 22q11.2DS who underwent PPF for VPI had overall similar outcomes to those without the syndrome. Length of stay was significantly longer for children with 22q11.2DS, which may be useful in preoperative family counseling. Postoperative hypocalcemia was common for patients with 22q11.2DS and providers should consider checking calcium on postoperative day one. Larger prospective studies are needed to further investigate the impact of 22q11.2DS on PPF outcomes.
