{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T20:18:39Z","timestamp":1778703519436,"version":"3.51.4"},"reference-count":34,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2022,11,23]],"date-time":"2022-11-23T00:00:00Z","timestamp":1669161600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Genes"],"abstract":"<jats:p>Tonsillectomy is one of the most common procedures performed in children, however there are currently no published studies evaluating tonsillectomy in children with 22q11.2 deletion syndrome (22q11DS). With this study, our goal was to investigate the indications, efficacy, and complications of tonsillectomy in a pediatric cohort of patients with 22q11DS. This is a retrospective chart review of patients in our 22q Center\u2019s repository. Inclusion criteria were a diagnosis of 22q11DS and a history of tonsillectomy or adenotonsillectomy. Data collected included: indications for tonsillectomy, preoperative and postoperative polysomnography (PSG) results, and surgical complications. In total, 33 patients were included. Most common indications for tonsillectomy were facilitation with speech surgery (n = 21) and sleep-disordered breathing (SDB)\/obstructive sleep apnea (OSA) (n = 16). Average length of stay was 1.15 days. Most patients (69%) had some degree of persistent OSA on postoperative PSG. Complications occurred in 18% of patients and included respiratory distress, hemorrhage, and hypocalcemia. This study demonstrates tonsillectomy was a commonly performed procedure in this cohort of patients with 22q11DS. These data highlight the potential need for close postoperative calcium and respiratory monitoring. The data were limited with respect to PSG outcomes, and future studies are needed to better characterize OSA outcomes and complications in this patient population.<\/jats:p>","DOI":"10.3390\/genes13122187","type":"journal-article","created":{"date-parts":[[2022,11,23]],"date-time":"2022-11-23T02:55:51Z","timestamp":1669172151000},"page":"2187","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Tonsillectomy in Children with 22q11.2 Deletion Syndrome"],"prefix":"10.3390","volume":"13","author":[{"given":"Jill M.","family":"Arganbright","sequence":"first","affiliation":[{"name":"Division of Otolaryngology, Children\u2019s Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108, USA"},{"name":"School of Medicine, University of Missouri-Kansas City, 2411 Holmes St., Kansas City, MO 64108, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3863-5928","authenticated-orcid":false,"given":"Paul Bryan","family":"Hankey","sequence":"additional","affiliation":[{"name":"College of Osteopathic Medicine, Kansas City University, 1750 Independence Ave, Kansas City, MO 64106, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0588-6889","authenticated-orcid":false,"given":"Meghan","family":"Tracy","sequence":"additional","affiliation":[{"name":"Division of Otolaryngology, Children\u2019s Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6460-7579","authenticated-orcid":false,"given":"Srivats","family":"Narayanan","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Missouri-Kansas City, 2411 Holmes St., Kansas City, MO 64108, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8171-6056","authenticated-orcid":false,"given":"Janelle","family":"Noel-MacDonnell","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Missouri-Kansas City, 2411 Holmes St., Kansas City, MO 64108, USA"},{"name":"Department of Health Services and Outcomes Research, Children\u2019s Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Ingram","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Missouri-Kansas City, 2411 Holmes St., Kansas City, MO 64108, USA"},{"name":"Department of Sleep Medicine, Children\u2019s Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"879","DOI":"10.1002\/ajmg.a.38199","article-title":"22q11.2 deletion syndrome in diverse populations","volume":"173","author":"Kruszka","year":"2017","journal-title":"Am. J. Med. Genet. A"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1016\/j.jpeds.2011.02.039","article-title":"Practical guidelines for managing patients with 22q11.2 deletion syndrome","volume":"159","author":"Bassett","year":"2011","journal-title":"J. Pediatr."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/j.ijporl.2017.02.012","article-title":"Association of airway abnormalities with 22q11.2 deletion syndrome","volume":"96","author":"Sacca","year":"2017","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1177\/0003489418771711","article-title":"Airway Anomalies in Patients With 22q11.2 Deletion Syndrome: A 5-Year Review","volume":"127","author":"Jones","year":"2018","journal-title":"Ann. Otol. Rhinol. Laryngol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2082","DOI":"10.1002\/mds.21103","article-title":"Neuromotor deficits in children with the 22q11 deletion syndrome","volume":"21","author":"Sobin","year":"2006","journal-title":"Mov. Disord."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1038\/gim.2016.98","article-title":"Obesity in adults with 22q11.2 deletion syndrome","volume":"19","author":"Voll","year":"2017","journal-title":"Genet. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1360","DOI":"10.1016\/j.ijporl.2014.05.031","article-title":"22q11.2 Deletion syndrome and obstructive sleep apnea","volume":"78","author":"Kennedy","year":"2014","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1475","DOI":"10.1016\/j.bjps.2014.07.026","article-title":"Screening for obstructive sleep apnea in children with syndromic cleft lip and\/or palate","volume":"67","author":"Silvestre","year":"2014","journal-title":"J. Plast. Reconstr. Aesthet. Surg."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1177\/0194599818807917","article-title":"Clinical Practice Guideline: Tonsillectomy in Children (Update)-Executive Summary","volume":"160","author":"Mitchell","year":"2019","journal-title":"Otolaryngol. Head Neck Surg."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1002\/lio2.118","article-title":"An updated review of pediatric drug-induced sleep endoscopy","volume":"2","author":"Wilcox","year":"2017","journal-title":"Laryngoscope Investig. Otolaryngol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"975","DOI":"10.5664\/jcsm.6698","article-title":"Success of Tonsillectomy for Obstructive Sleep Apnea in Children With Down Syndrome","volume":"13","author":"Ingram","year":"2017","journal-title":"J. Clin. Sleep Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"898","DOI":"10.1002\/lary.28922","article-title":"Outcomes of Adenotonsillectomy for Obstructive Sleep Apnea in Prader-Willi Syndrome: Systematic Review and Meta-analysis","volume":"131","author":"Clements","year":"2021","journal-title":"Laryngoscope"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1177\/1055665619900871","article-title":"Defining Risk of Postoperative Obstructive Sleep Apnea in Patients With 22q11.2DS Undergoing Pharyngeal Flap Surgery for Velopharyngeal Dysfunction Using Polysomnographic Evaluation","volume":"57","author":"Lee","year":"2020","journal-title":"Cleft Palate Craniofac. J."},{"key":"ref_14","first-page":"224","article-title":"Update on paediatric obstructive sleep apnoea","volume":"8","author":"Dehlink","year":"2016","journal-title":"J. Thorac. Dis."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1016\/j.jsmc.2009.04.007","article-title":"Pediatric Polysomnography","volume":"4","author":"Beck","year":"2009","journal-title":"Sleep Med. Clin."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"105","DOI":"10.2147\/NSS.S127816","article-title":"The effect of tonsillectomy on obstructive sleep apnea: An overview of systematic reviews","volume":"10","author":"Reckley","year":"2018","journal-title":"Nat. Sci. Sleep"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"e1153","DOI":"10.1002\/mgg3.1153","article-title":"Sleep patterns and problems among children with 22q11 deletion syndrome","volume":"8","author":"Arganbright","year":"2020","journal-title":"Mol. Genet. Genomic. Med."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"764","DOI":"10.1017\/S002221511800155X","article-title":"Wound healing after tonsillectomy\u2014A review of the literature","volume":"132","author":"Davidoss","year":"2018","journal-title":"J. Laryngol. Otol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/0165-5876(96)01364-X","article-title":"Post-tonsillectomy hemorrhage: An assessment of risk factors","volume":"37","author":"Myssiorek","year":"1996","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1007\/s00405-011-1535-9","article-title":"Hemorrhage rate after coblation tonsillectomy: A meta-analysis of published trials","volume":"268","author":"Hellmich","year":"2011","journal-title":"Eur. Arch. Otorhinolaryngol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"151","DOI":"10.5581\/1516-8484.20110037","article-title":"Hematological abnormalities and 22q11.2 deletion syndrome","volume":"33","author":"Rosa","year":"2011","journal-title":"Rev. Bras. Hematol. Hemoter."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1298","DOI":"10.1160\/TH07-05-0350","article-title":"Heterozygous loss of platelet glycoprotein (GP) Ib-V-IX variably affects platelet function in velocardiofacial syndrome (VCFS) patients","volume":"98","author":"Liang","year":"2007","journal-title":"Thromb. Haemost."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1111\/ped.12105","article-title":"Bernard-Soulier syndrome caused by a hemizygous GPIb\u03b2 mutation and 22q11.2 deletion","volume":"55","author":"Kunishima","year":"2013","journal-title":"Pediatr. Int."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1055\/s-0040-1701239","article-title":"Platelet Dysfunction in Noonan and 22q11.2 Deletion Syndromes in Childhood","volume":"120","author":"Isola","year":"2020","journal-title":"Thromb. Haemost."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1097\/GIM.0b013e3181634edf","article-title":"Parathyroid hormone reserve in 22q11.2 deletion syndrome","volume":"10","author":"Kapadia","year":"2008","journal-title":"Genet. Med."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1184","DOI":"10.1002\/ajmg.a.61152","article-title":"Palatal evaluation and treatment in 22q11.2 deletion syndrome","volume":"179","author":"Jackson","year":"2019","journal-title":"Am. J. Med. Genet. A"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/S0165-5876(00)00350-5","article-title":"Velopharyngeal insufficiency and articulation impairment in velo-cardio-facial syndrome: The influence of adenoids on phonemic development","volume":"54","author":"Havkin","year":"2000","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1001\/archotol.126.5.645","article-title":"Presence of 22q11 deletion in postadenoidectomy velopharyngeal insufficiency","volume":"126","author":"Perkins","year":"2000","journal-title":"Arch. Otolaryngol. Head Neck Surg."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1016\/j.ijporl.2010.03.006","article-title":"Cervical vascular and upper airway asymmetry in Velo-cardio-facial syndrome: Correlation of nasopharyngoscopy with MRA","volume":"74","author":"Oppenheimer","year":"2010","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"110236","DOI":"10.1016\/j.ijporl.2020.110236","article-title":"Magnetic resonance angiography (MRA) in preoperative planning for patients with 22q11.2 deletion syndrome undergoing craniofacial and otorhinolaryngologic procedures","volume":"138","author":"Kimia","year":"2020","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1177\/0003489414554943","article-title":"Use of imaging to evaluate course of the carotid artery in surgery for velopharyngeal insufficiency","volume":"124","author":"Raol","year":"2015","journal-title":"Ann. Otol. Rhinol. Laryngol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1184","DOI":"10.1097\/00006534-199804010-00002","article-title":"Limited value of preoperative cervical vascular imaging in patients with velocardiofacial syndrome","volume":"101","author":"Witt","year":"1998","journal-title":"Plast. Reconstr. Surg."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"e674","DOI":"10.1097\/BPO.0000000000000994","article-title":"The Clinical Utility of Flexion-extension Cervical Spine MRI in 22q11.2 Deletion Syndrome","volume":"39","author":"Kolman","year":"2019","journal-title":"J. Pediatr. Orthop."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"2104","DOI":"10.1002\/ajmg.a.38545","article-title":"Orthopaedic manifestations within the 22q11.2 Deletion syndrome: A systematic review","volume":"176","author":"Homans","year":"2018","journal-title":"Am. J. Med. Genet. A"}],"container-title":["Genes"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4425\/13\/12\/2187\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:24:43Z","timestamp":1760145883000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4425\/13\/12\/2187"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,23]]},"references-count":34,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2022,12]]}},"alternative-id":["genes13122187"],"URL":"https:\/\/doi.org\/10.3390\/genes13122187","relation":{},"ISSN":["2073-4425"],"issn-type":[{"value":"2073-4425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,11,23]]}}}