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

Supakit Peanchitlertkajorn; Thanate Assawakawintip; Mevadee Pibulniyom; Panida Srisan; Patimaporn Pungchanchaikul; Rasintra Jaroenying

doi:10.5664/jcsm.9028

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

J Clin Sleep Med. 2021 Mar 1;17(3):601-604. doi: 10.5664/jcsm.9028.

Authors

Supakit Peanchitlertkajorn¹, Thanate Assawakawintip¹, Mevadee Pibulniyom², Panida Srisan³, Patimaporn Pungchanchaikul⁴, Rasintra Jaroenying⁵

Affiliations

¹ Department of Orthodontics, Faculty of Dentistry, Mahidol University, Bangkok, Thailand.
² Panyananthaphikkhu Chonprathan Medical Center, Srinakharinwirot University, Nonthaburi, Thailand.
³ Division of Pulmonary and Critical Care, Department of Pediatrics, Queen Sirikit National Institute of Child Health, Bangkok, Thailand.
⁴ Biofilm Research Group, Faculty of Dentistry, Khon Kaen University, Khon Kaen, Thailand.
⁵ Division of Pediatric Pulmonary and Critical Care, Department of Pediatrics, Phramongkutklao Hospital and College of Medicine, Bangkok, Thailand.

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.

Keywords: Schwartz-Jampel syndrome; orthodontic treatment; pediatric OSA; rapid maxillary expansion; rapid palatal expansion.

Publication types

Case Reports

MeSH terms

Child
Continuous Positive Airway Pressure
Humans
Male
Maxilla
Osteochondrodysplasias*
Palatal Expansion Technique
Patient Compliance
Sleep Apnea, Obstructive*