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Successful treatment of a child with Schwartz-Jampel syndrome using rapid maxillary expansion and CPAP.

ABSTRACT:

Study objectives

We reported an 8-year-old male patient with Schwartz-Jampel syndrome, severe obstructive sleep apnea, constricted maxilla and moderate tonsillar hypertrophy. The syndrome is characterized by myotonia, skeletal dysplasia, and facial dysmorphism.

Methods

CPAP was initially prescribed, but he was not able to tolerate due to a high pressure setting. Rapid maxillary expansion alone reduced AHI to 10.4 events/h.

Results

When combined with CPAP, AHI is further reduced to 2.4 events/h. The patient has a better compliance with CPAP following rapid maxillary expansion therapy as the pressure setting decreased.

Conclusions

This is the first report utilizing a combination of rapid maxillary expansion and CPAP therapy to successfully treat severe pediatric OSA.

SUBMITTER: Peanchitlertkajorn S

PROVIDER: S-EPMC7927341 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature

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Successful treatment of a child with Schwartz-Jampel syndrome using rapid maxillary expansion and CPAP.

Peanchitlertkajorn Supakit S Assawakawintip Thanate T Pibulniyom Mevadee M Srisan Panida P Pungchanchaikul Patimaporn P Jaroenying Rasintra R

Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine 20210301 3

<h4>Study objectives</h4>We reported an 8-year-old male patient with Schwartz-Jampel syndrome, severe obstructive sleep apnea, constricted maxilla and moderate tonsillar hypertrophy. The syndrome is characterized by myotonia, skeletal dysplasia, and facial dysmorphism.<h4>Methods</h4>CPAP was initially prescribed, but he was not able to tolerate due to a high pressure setting. Rapid maxillary expansion alone reduced AHI to 10.4 events/h.<h4>Results</h4>When combined with CPAP, AHI is further red ...[more]

PMID: 33231165

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