Dataset Information

Sleep-Disordered Breathing in Children with Rare Skeletal Disorders: A Survey of Clinical Records.

ABSTRACT: OBJECTIVE:Craniofacial disharmony in skeletal diseases is strongly associated with sleep-disordered breathing. This study was aimed at studying the sleep respiratory patterns in young children with rare skeletal disorders. DESIGN:This retrospective study included children with achondroplasia (ACH), osteogenesis imperfecta (OI) and Ellis van Creveld Syndrome. Our subjects underwent an in-laboratory overnight respiratory polygraph between January 2012 and April 2016. All medical records were reviewed and brain Magnetic Resonance Imaging was conducted on patients with ACH, nasopharynx, oropharynx and laryngopharynx spaces. PATIENTS:Twenty-four children were enrolled, 13 with ACH, 2 with spondyloepiphyseal dysplasia, 1 with odontochondrodysplasia, 6 with OI and 2 with Ellis van Creveld Syndrome. RESULTS:Children with ACH, who had adenotonsillectomy, showed fewer sleep respiratory involvement than untreated children. Among 13 patients with ACH, brain magnetic resonance imaging was available in 10 subjects and significant negative correlation was found between sleep respiratory patterns, nasopharynx and oropharynx space (p < 0.05). In 2 patients with spondyloepiphyseal dysplasia, mild-to-moderate sleep respiratory involvement was found. Both subjects had a history of adenotonsillectomy. Mild sleep respiratory involvement was also observed in 4 out of 6 patients with OI. One patient with Ellis van Creveld syndrome had mild sleep respiratory disturbance. CONCLUSIONS:Sleep respiratory disturbances were detected in children with ACH, and with less severity also in OI and Ellis van Creveld syndrome. Adenotonsillectomy was successful in ACH in reducing symptoms. In light of our findings, multicenter studies are needed to obtain further information on these rare skeletal diseases.

SUBMITTER: Zaffanello M

PROVIDER: S-EPMC6244108 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature

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Sleep-Disordered Breathing in Children with Rare Skeletal Disorders: A Survey of Clinical Records.

Zaffanello Marco M Piacentini Giorgio G Sacchetto Luca L Pietrobelli Angelo A Gasperi Emma E Barillari Marco M Cardobi Nicolò N Nosetti Luana L Ramaroli Diego D Antoniazzi Franco F

Medical principles and practice : international journal of the Kuwait University, Health Science Centre 20180621 5

<h4>Objective</h4>Craniofacial disharmony in skeletal diseases is strongly associated with sleep-disordered breathing. This study was aimed at studying the sleep respiratory patterns in young children with rare skeletal disorders.<h4>Design</h4>This retrospective study included children with achondroplasia (ACH), osteogenesis imperfecta (OI) and Ellis van Creveld Syndrome. Our subjects underwent an in-laboratory overnight respiratory polygraph between January 2012 and April 2016. All medical rec ...[more]

PMID: 29929197

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Project description:OBJECTIVES:Although adenotonsillar hypertrophy is the main cause of sleep-disordered breathing in children, surrounding anatomic factors, such as the width of the nasopharynx, can affect upper airway patency. However, there have been no reports of the association of nasopharyngeal width with sleep-disordered breathing in children. This study was undertaken to measure nasopharyngeal width in children undergoing adenotonsillectomy for sleep-disordered breathing and to investigate the clinical implications of this factor. METHODS:This was a retrospective study with a follow-up period of 1 year, performed at a tertiary referral center. We reviewed the operative records of children who underwent adenotonsillectomy at our center for symptoms of sleepdisordered breathing, such as snoring, apnea, and mouth breathing. The nasopharyngeal width was measured immediately before adenotonsillectomy, which was performed under general anesthesia with a microscopy-assisted mirror view. Adenotonsillar hypertrophy was graded on a four-point scale, and symptoms of sleep-disordered breathing were evaluated by using the Korean version of the Obstructive Sleep Apnea-18 questionnaire before and after surgery. The relationships between the average nasopharyngeal width and patient age and sex, adenotonsillar hypertrophy, and the Korean version of the Obstructive Sleep Apnea-18 score were analyzed. RESULTS:The study included 549 children (343 boys) with a mean age of 6.0 years (range, 2 to 11 years). The average nasopharyngeal width was 11.9 mm (range, 7.0 to 18.0 mm) and increased with age (range, 11.2 to 13.3; β=0.264; P< 0.001). At 1 year after surgery, children with a greater nasopharyngeal width at the time of surgery exhibited additional improvements in symptoms of obstruction relative to those at 1 month after surgery. CONCLUSION:The average nasopharyngeal width in children is approximately 11.9 mm and exhibits a slight increase with age. The width of the nasopharynx may be a factor associated with the degree of improvement in symptoms of sleepdisordered breathing after adenotonsillectomy.

Dataset Information

Sleep-Disordered Breathing in Children with Rare Skeletal Disorders: A Survey of Clinical Records.

Publications

Sleep-Disordered Breathing in Children with Rare Skeletal Disorders: A Survey of Clinical Records.

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