title: Sleep-disordered breathing and its management in children with achondroplasia.
abstract: Sleep-disordered breathing is a common feature in children with achondroplasia. The aim of our study was to review the poly(somno)graphic (P(S)G) findings and consequent treatments in children with achondroplasia followed in the national reference center for skeletal dysplasia. A retrospective review of the clinical charts and P(S)G of 43 consecutive children (mean age 3.9 ± 3.5 years) with achondroplasia seen over a period of 2 years was performed. Twenty four (59%) children had obstructive sleep apnea (OSA). Thirteen children had an obstructive apnea-hypopnea index (OAHI) < 5/hr, four had an OAHI between 5 and 10/hr, and seven had an OAHI ≥ 10/hr. Ten of the 15 children who had previous upper airway surgery still had an abnormal P(S)G. All the patients with an AHI ≥ 10/hr were under 7 years of age and none had a prior tonsillectomy. The children who underwent adeno-tonsillectomy, coupled in most cases with turbinectomy, were significantly older (mean age 7.5 ± 3.5 vs. 3.5 ± 1.7 years old, P = 0.015) and had significantly better P(S)G results than those who underwent only adeno-turbinectomy. No correlation was observed between the mean AHI value at the baseline P(S)G and the type of academic course (standard, supported or specialized). In conclusion, OSA is common in children with achondroplasia. The observation of a reduced prevalence of OSA after (adeno-)tonsillectomy is in favor of this type of surgery when possible.
authors.affiliation: Pediatric Highly Intensive Care Unit Department of Pathophysiology and Transplantation, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Università degli Studi di Milano, Milan, Italy.|AP-HP, Hôpital Necker-Enfants malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France.
authors.forename: Rossana
authors.identifier: http://orcid.org/0000-0003-3137-5756
authors.initials: R
authors.lastname: Tenconi
authors.affiliation: AP-HP, Hôpital Necker-Enfants malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France.|ASV Santé, Gennevilliers, France.
authors.forename: Sonia
authors.initials: S
authors.lastname: Khirani
authors.affiliation: AP-HP, Hôpital Necker-Enfants malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France.|Paris Descartes University, Paris, France.|Inserm U955, Equipe 13, Créteil, France.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Amaddeo
authors.affiliation: AP-HP, Hôpital Necker-Enfants malades, Genetics Departement, Centre of Reference for Skeletal Dysplasia, INSERM UMR 1163, Institut Imagine, University Paris Descartes-Sorbonne Paris Cité, Paris, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Michot
authors.affiliation: AP-HP, Hôpital Necker-Enfants malades, Genetics Departement, Centre of Reference for Skeletal Dysplasia, INSERM UMR 1163, Institut Imagine, University Paris Descartes-Sorbonne Paris Cité, Paris, France.
authors.forename: Geneviève
authors.initials: G
authors.lastname: Baujat
authors.affiliation: Head and Neck Surgery and Otorhinolaryngology Department, AP-HP, Hôpital Necker-Enfants malades, Paris, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Couloigner
authors.affiliation: AP-HP, Hôpital Necker-Enfants malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France.
authors.forename: Livio
authors.initials: L
authors.lastname: De Sanctis
authors.affiliation: Pediatric Neurosurgery Department, AP-HP, Hôpital Necker-Enfants malades, Paris, France.
authors.forename: Syril
authors.initials: S
authors.lastname: James
authors.affiliation: Paris Descartes University, Paris, France.|Pediatric Neurosurgery Department, AP-HP, Hôpital Necker-Enfants malades, Paris, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Zerah
authors.affiliation: AP-HP, Hôpital Necker-Enfants malades, Genetics Departement, Centre of Reference for Skeletal Dysplasia, INSERM UMR 1163, Institut Imagine, University Paris Descartes-Sorbonne Paris Cité, Paris, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Cormier-Daire
authors.affiliation: AP-HP, Hôpital Necker-Enfants malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France.|Paris Descartes University, Paris, France.|Inserm U955, Equipe 13, Créteil, France.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Fauroux
mesh_terms: D000130:Achondroplasia / Q000150:complications / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D000233:Adenoidectomy / Q000706:statistics & numerical data*; D002648:Child; D002675:Child, Preschool; D019468:Disease Management; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D017286:Polysomnography; D012189:Retrospective Studies; D020182:Sleep Apnea, Central / Q000150:complications / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D020181:Sleep Apnea, Obstructive / Q000150:complications / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D014068:Tonsillectomy / Q000706:statistics & numerical data*
keywords: achondroplasia; neurosurgery; polysomnography; sleep apnea; tonsillectomy