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Introduction: 22q11.2 deletion syndrome (22qDS) is a common genetic disorder with an estimated risk of obstructive airway symptoms in 10-37% of affected children. While tonsillectomy is commonly pursued in an effort to ameliorate airway obstruction this population, there are currently no published studies evaluating tonsillectomy outcomes in children with 22q11DS. Our goal was to investigate the indications, efficacy, and complications of tonsillectomy in a large cohort of patients with 22qDS.

Methods: We performed a retrospective chart review of 168 consecutive patients with 22qDS from the institution’s 22q repository from 1/1/2010-6/1/2021. Data was collected for tonsillectomy indications, length of hospital stay, complications, and pre- and post-op sleep study results.

Results: Thirty-three of the 168 (20%) patients with 22qDS in our sample underwent tonsillectomy. Indications for tonsillectomy included: to facilitate with speech surgery (n=21), sleep disordered breathing/OSA (n=16), dysphagia (n=3), and airway management prior to trach decannulation (n=2). Average length of stay was 1.15 days (range 0-4 days). Six patients had a pre-op sleep study, 10 patients had post-op sleep study, and 3 patients had both a pre- and post-op sleep studies. Of those children who had post-op sleep studies, 3 were normal, 5 had mild OSA, 1 had moderate OSA, and 1 had severe OSA. Three patients presented with post-op tonsil bleeds, of whom 1 required surgical cauterization and 2 recovered with observation alone. In the 8 patients who had post-op calcium monitoring, 4 (50%) had hypocalcemia and endocrine was consulted; 1 patient was treated with oral supplementation, and 3 were observed with repeat labs. One patient experienced postoperative respiratory failure requiring overnight intubation.

Conclusion: Tonsillectomy was a commonly performed procedure in this large cohort of patients with 22qDS. Facilitation of speech surgery and sleep disordered breathing were the most common indications. Most patients spent one night in the hospital prior to discharge. These data highlight the potential need for close post-op calcium and respiratory monitoring. Our data were limited with respect to polysomnographic outcomes, where future studies are still needed to better characterize sleep disordered breathing outcomes following tonsillectomy for patients with 22qDS.

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Tonsillectomy In Children With 22q11.2 Deletion Syndrome