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 |
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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. |
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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 |
Author_xml | – sequence: 1 givenname: Supakit surname: Peanchitlertkajorn fullname: Peanchitlertkajorn, Supakit organization: Department of Orthodontics, Faculty of Dentistry, Mahidol University, Bangkok, Thailand – sequence: 2 givenname: Thanate surname: Assawakawintip fullname: Assawakawintip, Thanate organization: Department of Orthodontics, Faculty of Dentistry, Mahidol University, Bangkok, Thailand – sequence: 3 givenname: Mevadee surname: Pibulniyom fullname: Pibulniyom, Mevadee organization: Panyananthaphikkhu Chonprathan Medical Center, Srinakharinwirot University, Nonthaburi, Thailand – sequence: 4 givenname: Panida surname: Srisan fullname: Srisan, Panida organization: Division of Pulmonary and Critical Care, Department of Pediatrics, Queen Sirikit National Institute of Child Health, Bangkok, Thailand – sequence: 5 givenname: Patimaporn surname: Pungchanchaikul fullname: Pungchanchaikul, Patimaporn organization: Biofilm Research Group, Faculty of Dentistry, Khon Kaen University, Khon Kaen, Thailand – sequence: 6 givenname: Rasintra surname: Jaroenying fullname: Jaroenying, Rasintra organization: Division of Pediatric Pulmonary and Critical Care, Department of Pediatrics, Phramongkutklao Hospital and College of Medicine, Bangkok, Thailand |
BackLink | https://www.ncbi.nlm.nih.gov/pubmed/33231165$$D View this record in MEDLINE/PubMed |
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Cites_doi | 10.1038/82638 10.1186/1471-2377-3-3 10.1016/j.ajodo.2009.02.019 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 10.1542/peds.2005-1634 |
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Keywords | orthodontic treatment Schwartz-Jampel syndrome pediatric OSA rapid palatal expansion rapid maxillary expansion |
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References | e_1_3_2_8_2 Pirelli P (e_1_3_2_9_2) 2005; 6 e_1_3_2_7_2 e_1_3_2_6_2 e_1_3_2_10_2 e_1_3_2_5_2 e_1_3_2_11_2 e_1_3_2_4_2 e_1_3_2_12_2 e_1_3_2_3_2 e_1_3_2_2_2 |
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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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