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

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. CPAP was initially prescribed, but he was not able to t...

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Published in:Journal of clinical sleep medicine Vol. 17; no. 3; pp. 601 - 604
Main Authors: Peanchitlertkajorn, Supakit, Assawakawintip, Thanate, Pibulniyom, Mevadee, Srisan, Panida, Pungchanchaikul, Patimaporn, Jaroenying, Rasintra
Format: Journal Article
Language:English
Published: United States American Academy of Sleep Medicine 01-03-2021
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Abstract 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. 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. 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. This is the first report utilizing a combination of rapid maxillary expansion and CPAP therapy to successfully treat severe pediatric OSA.
AbstractList 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. 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. 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. This is the first report utilizing a combination of rapid maxillary expansion and CPAP therapy to successfully treat severe pediatric OSA.
Author Pungchanchaikul, Patimaporn
Jaroenying, Rasintra
Pibulniyom, Mevadee
Srisan, Panida
Peanchitlertkajorn, Supakit
Assawakawintip, Thanate
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  organization: Division of Pediatric Pulmonary and Critical Care, Department of Pediatrics, Phramongkutklao Hospital and College of Medicine, Bangkok, Thailand
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10.1186/1471-2377-3-3
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10.1136/jmg.29.1.58
10.1002/lary.26352
10.1001/archotol.1997.01900120098017
10.1016/j.prrv.2017.06.003
10.1016/j.sleep.2008.04.003
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Keywords orthodontic treatment
Schwartz-Jampel syndrome
pediatric OSA
rapid palatal expansion
rapid maxillary expansion
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  doi: 10.1038/82638
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  article-title: Obstructive sleep apnoea syndrome (OSAS) and rhino-tubaric disfunction in children: therapeutic effects of RME therapy
  publication-title: Prog Orthod.
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    fullname: Pirelli P
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  doi: 10.1186/1471-2377-3-3
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Snippet We reported an 8-year-old male patient with Schwartz-Jampel syndrome, severe obstructive sleep apnea, constricted maxilla and moderate tonsillar hypertrophy....
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SubjectTerms Case Reports
Child
Continuous Positive Airway Pressure
Humans
Male
Maxilla
Osteochondrodysplasias
Palatal Expansion Technique
Patient Compliance
Sleep Apnea, Obstructive
Title Successful treatment of a child with Schwartz-Jampel syndrome using rapid maxillary expansion and CPAP
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