Ten cases of adenoidectomy and tonsillectomy for children with achondroplasia and sleep apnea

Sleep apnea in childhood is mostly cured by upper airway surgery (adenoidectomy and/or tonsillectomy). However, sleep apnea with achondroplasia may not be cured even after upper airway surgery due to structural nasopharyngeal stenosis and central sleep apnea. Forty-seven cases of achondroplasia were...

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Published inStomato-pharyngology Vol. 34; no. 1; pp. 53 - 60
Main Authors Morimoto, Noriko, Yamaguchi, Sota, Yoshikawa, Mamoru
Format Journal Article
LanguageJapanese
Published Japan Society of Stomato-pharyngology 2021
日本口腔・咽頭科学会
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ISSN0917-5105
1884-4316
DOI10.14821/stomatopharyngology.34.53

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Abstract Sleep apnea in childhood is mostly cured by upper airway surgery (adenoidectomy and/or tonsillectomy). However, sleep apnea with achondroplasia may not be cured even after upper airway surgery due to structural nasopharyngeal stenosis and central sleep apnea. Forty-seven cases of achondroplasia were followed in our hospital, of which we examined 10 cases who underwent upper airway surgery due to suspicion of sleep apnea. Diagnosis of sleep apnea was performed by a sleep test or overnight pulse oxymetry during hospitalization. Symptoms of sleep apnea improved in 7 of the 10 cases (70.0%) including reoperation cases due to upper airway surgery and who did not relapse, but 3 of the 10 cases (30.0%) needed conservative treatment such as CPAP. It was considered that sleep apnea with achondroplasia not only affects obstructive sleep apnea but also involves multiple factors such as central sleep apnea, lower respiratory tract disease and GH replacement therapy. In the perioperative management, it is necessary to manage patients in the PICU to secure the airway with a nasal airway or positive pressure breathing in cases where stenosis of the postoperative upper airway is temporarily exacerbated. When snoring or depressed breathing remains after surgery, CPAP therapy, continuous nasal airway and home oxygen therapy should be considered.
AbstractList Sleep apnea in childhood is mostly cured by upper airway surgery (adenoidectomy and/or tonsillectomy). However, sleep apnea with achondroplasia may not be cured even after upper airway surgery due to structural nasopharyngeal stenosis and central sleep apnea. Forty-seven cases of achondroplasia were followed in our hospital, of which we examined 10 cases who underwent upper airway surgery due to suspicion of sleep apnea. Diagnosis of sleep apnea was performed by a sleep test or overnight pulse oxymetry during hospitalization. Symptoms of sleep apnea improved in 7 of the 10 cases (70.0%) including reoperation cases due to upper airway surgery and who did not relapse, but 3 of the 10 cases (30.0%) needed conservative treatment such as CPAP. It was considered that sleep apnea with achondroplasia not only affects obstructive sleep apnea but also involves multiple factors such as central sleep apnea, lower respiratory tract disease and GH replacement therapy. In the perioperative management, it is necessary to manage patients in the PICU to secure the airway with a nasal airway or positive pressure breathing in cases where stenosis of the postoperative upper airway is temporarily exacerbated. When snoring or depressed breathing remains after surgery, CPAP therapy, continuous nasal airway and home oxygen therapy should be considered. 軟骨無形成症は構造的な鼻咽腔狭窄に加え,中枢性の睡眠時無呼吸も合併しやすいことから上気道手術を行っても睡眠時無呼吸症が改善されない例がある.今回,われわれは国立成育医療研究センターに軟骨無形成症で受診した47例のうち,睡眠時無呼吸症の疑いで耳鼻咽喉科に受診し上気道手術(アデノイド切除術・口蓋扁桃摘出術)を施行した10症例を対象に検討を行った.再手術例を併せると10例中7例(70.0%)は上気道手術により睡眠時無呼吸症状は改善し再燃もしなかったが,10例中3例(30.0%)はCPAPなどの保存的治療が必要であった.軟骨無形成症に伴う睡眠時無呼吸には閉塞性のみではなく,中枢性の睡眠時無呼吸や下気道疾患,低身長に対して行う成長ホルモン補充療法が複数の因子として影響していることが示唆された.
Sleep apnea in childhood is mostly cured by upper airway surgery (adenoidectomy and/or tonsillectomy). However, sleep apnea with achondroplasia may not be cured even after upper airway surgery due to structural nasopharyngeal stenosis and central sleep apnea. Forty-seven cases of achondroplasia were followed in our hospital, of which we examined 10 cases who underwent upper airway surgery due to suspicion of sleep apnea. Diagnosis of sleep apnea was performed by a sleep test or overnight pulse oxymetry during hospitalization. Symptoms of sleep apnea improved in 7 of the 10 cases (70.0%) including reoperation cases due to upper airway surgery and who did not relapse, but 3 of the 10 cases (30.0%) needed conservative treatment such as CPAP. It was considered that sleep apnea with achondroplasia not only affects obstructive sleep apnea but also involves multiple factors such as central sleep apnea, lower respiratory tract disease and GH replacement therapy. In the perioperative management, it is necessary to manage patients in the PICU to secure the airway with a nasal airway or positive pressure breathing in cases where stenosis of the postoperative upper airway is temporarily exacerbated. When snoring or depressed breathing remains after surgery, CPAP therapy, continuous nasal airway and home oxygen therapy should be considered.
Author Yamaguchi, Sota
Morimoto, Noriko
Yoshikawa, Mamoru
Author_FL 守本 倫子
Yamaguchi Sota
吉川 衛
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  organization: Department of Otorhinolaryngology, Toho University Ohashi Medical Center
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References 12) 酒井あや,木下裕子,三輪高喜:当科における小児睡眠時無呼吸症についての検討.頭頸部外科 2016;26(1):65-70
3) 田中弘之:軟骨無形成症における睡眠の障害.小児内科 2008;40(1):101-103
9) Capdevila OS: Pediatric obstructive sleep apnea: complications, management, and long-term Outcomes. Proc Am Thorac Soc 2008;5(2):274-282.
5) The AASM manual for the Scoring of Sleep and Associated Events. Rules, Terminology, and Technical Specifications. Westchester, Illinois, American Academy of Sleep Medicine; 2007.
14) Afsharpaiman S, Sillence DO, Sheikhvatan M, et al: Respiratory events and obstructive sleep apnea in children with achondroplasia: investigation and treatment outcomes. Sleep Breath 2011;15(4):755-761.
6) International Classification of Sleep Disorders, 3rd: Diagnostic and coding manual. Westchester, Illinois, American Academy of Sleep Medicine; 2014, p. 63-68
8) Schechter MS: Technical report: Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics 2002;109(4):e69.
10) Nieminen P, Tolonen U, Löppönen H: Snoring and obstructive sleep apnea in children: a 6-month follow-up study. Arch Otolaryngol Head Neck Surg 2000;126(4):481-486
13) 馬場洋徳,相澤直孝,高橋奈央,他:小児睡眠呼吸障害に対するアデノイド口蓋扁桃摘出術の成績:手術前後のPSGによる検討.口咽科 2015;28(2):205-209.
16) 水野貴基,和田友香,守本倫子:基礎疾患を有する新生児・乳児への経鼻エアウェイ使用に関する検討.小児耳鼻咽喉科 2018;39(3):333-338
11) Ameli F, Brocchetti F, Semino L, et al: Ad­e­no­ton­sil­lec­to­my in obstructive sleep apnea syndrome. Propasal of a surgical decision-taking algorithm. Int J Pediatr Otorhinolaryngol 2007;71(5):729-734.
4) 菅原大輔,田中裕之,田中康子,他:軟骨無形成症及び軟骨低形成症に対する治療効果についての検討.日本内分泌学会雑誌 2015;91:27-29
17) 軟骨無形成症診療ガイドライン作成委員会:軟骨無形成症診療ガイドライン-UMIN.2019,http://jspe.umin.jp/medical/files/guide2_20190111.pdf(参照 2020-12-1
19) 国立成育医療研究センター,小児慢性特定疾病情報室 編,日本小児科学会 監:小児慢性特定疾病—診断の手引き.東京,日本,診断と治療社;2016,p. 433-434
18) 粕田承吾,田中一郎,高橋幸博,他:軟骨無形成症に合併する睡眠時無呼吸に対するアデノイド切除術及び大後頭孔減圧術前後の呼吸モニタリング.日本小児科学会雑誌 2003;107(6):928-931
7) Onodera K, Sakata H, Niikuni N, et al: Survey of the present status of sleep-disordered breathing in children with achondroplasia. Int J Pediatr Otorhinolaryngol 2005;69(4):457-461
2) Foldynova-Trantirkova S, Wilcox WR, Krejci P: Sixteen years and counting: the current understanding of fibroblast growth factor receptor 3(FGFR3) signaling in skeletal dysplasias. Hum Mutat 2012;33(1):29-41
21) 衞藤義勝 監:ネルソン小児科学 原著第19版.東京,日本,エルゼビア・ジャパン株式会社;2015,p. 1768-1769
15) Tenconi R, Khirani S, Amaddeo A, et al: Sleep-disordered breathing and its management in children with achondroplasia. Am J Med Genet A 2017;173(4):868-878
1) Vajo Z, Francomano CA, Wilkin DJ: The molecular and genetic basis of fibroblast growth factor receptor 3 disorders: the achondroplasia family of skeletal dysplasias, Muenke craniosynostosis, and Crouzon syndrome with acanthosis nigricans. Endocr Rev 2000;21(1):23-39
20) Tasker RC, Dundas I, Laverty A, et al: Distinct patterns of respiratory difficulty in young children with achondroplasia: a clinical, sleep, and lung function study. Arch Dis Child 1998;79(2):99-108
References_xml – reference: 5) The AASM manual for the Scoring of Sleep and Associated Events. Rules, Terminology, and Technical Specifications. Westchester, Illinois, American Academy of Sleep Medicine; 2007.
– reference: 17) 軟骨無形成症診療ガイドライン作成委員会:軟骨無形成症診療ガイドライン-UMIN.2019,http://jspe.umin.jp/medical/files/guide2_20190111.pdf(参照 2020-12-1)
– reference: 16) 水野貴基,和田友香,守本倫子:基礎疾患を有する新生児・乳児への経鼻エアウェイ使用に関する検討.小児耳鼻咽喉科 2018;39(3):333-338.
– reference: 9) Capdevila OS: Pediatric obstructive sleep apnea: complications, management, and long-term Outcomes. Proc Am Thorac Soc 2008;5(2):274-282.
– reference: 10) Nieminen P, Tolonen U, Löppönen H: Snoring and obstructive sleep apnea in children: a 6-month follow-up study. Arch Otolaryngol Head Neck Surg 2000;126(4):481-486.
– reference: 2) Foldynova-Trantirkova S, Wilcox WR, Krejci P: Sixteen years and counting: the current understanding of fibroblast growth factor receptor 3(FGFR3) signaling in skeletal dysplasias. Hum Mutat 2012;33(1):29-41.
– reference: 4) 菅原大輔,田中裕之,田中康子,他:軟骨無形成症及び軟骨低形成症に対する治療効果についての検討.日本内分泌学会雑誌 2015;91:27-29.
– reference: 6) International Classification of Sleep Disorders, 3rd: Diagnostic and coding manual. Westchester, Illinois, American Academy of Sleep Medicine; 2014, p. 63-68.
– reference: 7) Onodera K, Sakata H, Niikuni N, et al: Survey of the present status of sleep-disordered breathing in children with achondroplasia. Int J Pediatr Otorhinolaryngol 2005;69(4):457-461.
– reference: 18) 粕田承吾,田中一郎,高橋幸博,他:軟骨無形成症に合併する睡眠時無呼吸に対するアデノイド切除術及び大後頭孔減圧術前後の呼吸モニタリング.日本小児科学会雑誌 2003;107(6):928-931.
– reference: 3) 田中弘之:軟骨無形成症における睡眠の障害.小児内科 2008;40(1):101-103.
– reference: 20) Tasker RC, Dundas I, Laverty A, et al: Distinct patterns of respiratory difficulty in young children with achondroplasia: a clinical, sleep, and lung function study. Arch Dis Child 1998;79(2):99-108.
– reference: 8) Schechter MS: Technical report: Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics 2002;109(4):e69.
– reference: 11) Ameli F, Brocchetti F, Semino L, et al: Ad­e­no­ton­sil­lec­to­my in obstructive sleep apnea syndrome. Propasal of a surgical decision-taking algorithm. Int J Pediatr Otorhinolaryngol 2007;71(5):729-734.
– reference: 15) Tenconi R, Khirani S, Amaddeo A, et al: Sleep-disordered breathing and its management in children with achondroplasia. Am J Med Genet A 2017;173(4):868-878.
– reference: 21) 衞藤義勝 監:ネルソン小児科学 原著第19版.東京,日本,エルゼビア・ジャパン株式会社;2015,p. 1768-1769.
– reference: 1) Vajo Z, Francomano CA, Wilkin DJ: The molecular and genetic basis of fibroblast growth factor receptor 3 disorders: the achondroplasia family of skeletal dysplasias, Muenke craniosynostosis, and Crouzon syndrome with acanthosis nigricans. Endocr Rev 2000;21(1):23-39.
– reference: 19) 国立成育医療研究センター,小児慢性特定疾病情報室 編,日本小児科学会 監:小児慢性特定疾病—診断の手引き.東京,日本,診断と治療社;2016,p. 433-434.
– reference: 12) 酒井あや,木下裕子,三輪高喜:当科における小児睡眠時無呼吸症についての検討.頭頸部外科 2016;26(1):65-70.
– reference: 13) 馬場洋徳,相澤直孝,高橋奈央,他:小児睡眠呼吸障害に対するアデノイド口蓋扁桃摘出術の成績:手術前後のPSGによる検討.口咽科 2015;28(2):205-209.
– reference: 14) Afsharpaiman S, Sillence DO, Sheikhvatan M, et al: Respiratory events and obstructive sleep apnea in children with achondroplasia: investigation and treatment outcomes. Sleep Breath 2011;15(4):755-761.
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Snippet Sleep apnea in childhood is mostly cured by upper airway surgery (adenoidectomy and/or tonsillectomy). However, sleep apnea with achondroplasia may not be...
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StartPage 53
SubjectTerms achondroplasia
adenoidectomy
central sleep apnea
obstructive sleep apnea
tonsillectomy
アデノイド切除術
中枢性睡眠時無呼吸
口蓋扁桃摘出術
軟骨無形成症
閉塞性睡眠時無呼吸
Title Ten cases of adenoidectomy and tonsillectomy for children with achondroplasia and sleep apnea
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