Sleep-disordered breathing in children with achondroplasia

Title

Sleep-disordered breathing in children with achondroplasia

Creator

Mogayzel P J; Carroll J L; Loughlin G M; Hurko O; Francomano C A; Marcus C L

Publisher

Journal of Pediatrics

Date

1998

Subject

Pediatrics; death; unexpected; apnea; cervicomedullary compression; decompression; infants; obstruction; respiratory complications; breathing difficulties; achondroplasia; trajectory; characteristics; obstructive sleep apnea; central apnea

Description

Objective: Our objective was to characterize sleep-disordered breathing in 88 children with achondroplasia aged 1 month to 12.6 years. Results: At the time of their initial polysomnography, five children had previously undergone tracheostomy, and seven children required supplemental oxygen. Initial polysomnography demonstrated a median obstructive apnea index of 0 (range, 0 to 19.2 apneas/hr). The median number of central apneas with desaturation per study was 0.5 (0 to 49), the median oxygen saturation nadir was 91% (50% to 99%), and the median peak end-tidal pCO(2) was 47 mm Hg (36 to 87 mm Hg). Forty-two children (47.7%) had abnormal initial study results, usually caused by hypoxemia. Two children with severe obstructive sleep apnea eventually required continuous positive airway pressure therapy, and three additional children required tracheostomies. Conclusions: (1) Children with achondroplasia often have sleep-related respiratory disturbances, primarily hypoxemia. (2) The majority do not have significant obstructive or central apnea; however, a substantial minority are severely affected. (3) Tonsillectomy and adenoidectomy decreases the degree of upper airway obstruction in most but not all children with achondroplasia and obstructive sleep apnea. (4) Restrictive lung disease can present at a young age in children with achondroplasia.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation

Mogayzel P J; Carroll J L; Loughlin G M; Hurko O; Francomano C A; Marcus C L, “Sleep-disordered breathing in children with achondroplasia,” Pediatric Palliative Care Library, accessed August 2, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16747.

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