Defining Risk of Postoperative Obstructive Sleep Apnea in Patients With 22q11.2DS Undergoing Pharyngeal Flap Surgery for Velopharyngeal Dysfunction Using Polysomnographic Evaluation

Abstract

Objective: To determine pre- and postoperative prevalence of obstructive sleep apnea (OSA) in patients with 22q11.2 deletion syndrome (DS) undergoing wide posterior pharyngeal flap (PPF) surgery for velopharyngeal dysfunction (VPD). Design: Retrospective study using pre- and postoperative polysomnography (PSG) to determine prevalence of OSA. Medical records were reviewed for patients’ medical comorbidities. Parents were surveyed about snoring. Setting: Academic tertiary care pediatric hospital. Patients: Forty patients with laboratory confirmed 22q11.2DS followed over a 6-year period. Interventions: Pre- and postoperative PSG, speech evaluation, and parent surveys. Main Outcome Measure: Severity and prevalence of OSA, defined by obstructive apnea hypopnea index (OAHI), before and after PPF surgery to determine whether PPF is associated with increased risk of OSA. Results: Mean OAHI did not change significantly after PPF surgery (1.1/h vs 2.1/h, P = .330). Prevalence of clinically significant OSA (OAHI ≥ 5) was identical pre- and postoperatively (2 of 40), with both cases having severe-range OSA requiring positive airway pressure therapy. All other patients had mild-range OSA. Nasal resonance was graded as severe preoperatively in 85% of patients. None were graded as severe postoperatively. No single patient factor or parent-reported concern predicted risk of OSA (OAHI ≥ 1.5). Conclusions: Patients with 22q11.2DS are medically complex and are at increased risk of OSA at baseline. Wide PPF surgery for severe VPD does not significantly increase risk of OSA. Careful perioperative planning is essential to optimize both speech and sleep outcomes.

Tonsillectomy in Children with 22q11.2 Deletion Syndrome

“…A staged tonsillectomy with or without adenoidectomy can be performed a few months prior to speech surgery. This procedure is often recommended to make more space in the airway prior to the speech surgery in the hope of limiting the risk of postoperative OSA following speech surgery [ 13 ]. Additionally, in some cases, tonsillectomy is needed to allow access to the surgical site(s) for the speech surgery [ 13 ].…”

“…This procedure is often recommended to make more space in the airway prior to the speech surgery in the hope of limiting the risk of postoperative OSA following speech surgery [ 13 ]. Additionally, in some cases, tonsillectomy is needed to allow access to the surgical site(s) for the speech surgery [ 13 ]. In this study, airway optimization prior to speech surgery was indicated for 64% of patients.…”

“…In this study, airway optimization prior to speech surgery was indicated for 64% of patients. Similarly, tonsillectomy prior to speech surgery was also very common in a study by Lee et al, in which 77.5% of patients had tonsillectomy prior to wide pharyngeal flap to treat preoperative SDB/OSA and/or remove tonsils so as not to interfere with the pharyngeal flap surgery secondary to enlargement or positioning [ 13 ].…”

“…This study reported an overall 32% rate of positive screen for symptoms concerning for OSA; however, the subgroup with 22q11DS had the highest rate of positive scores (50%), which was a significant difference ( p = 0.042). A study by Lee et al (2020) looked at 40 patients with 22q11DS and VPD who were being evaluated for wide pharyngeal flap [ 13 ]. As part of the work-up, a preoperative sleep study was obtained, in which 37.5% of patients were diagnosed with OSA; most of these patients had mild OSA.…”

“…Tonsillectomy and adenoidectomy (T/A) are often considered the first-line treatment for pediatric SDB/OSA, because these procedures are highly effective in non-syndromic patients [ 10 ]. However, it is much less clear whether tonsillectomy has similar outcomes in syndromic patients, with current studies often highlighting lower surgical cure rates for patients with Down syndrome [ 11 ] and Prader-Willi syndrome [ 12 , 13 ]. There are currently no published reports on outcomes for SDB/OSA after tonsillectomy in patients with 22q11DS.…”