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Anesthetic Management of a Patient With Ring 18 Syndrome.

Authors
  • Maekawa, Midori1
  • Yasuda, Makoto1
  • Sasaki, Haruka1
  • Tachinami, Yasuharu1, 2
  • Mizuta, Kentaro1
  • 1 Division of Dento-oral Anesthesiology, Tohoku University Graduate School of Dentistry, Sendai, Japan. , (Japan)
  • 2 Tachinami Dental office, Imizu, Japan. , (China)
Type
Published Article
Journal
Anesthesia progress
Publication Date
Oct 01, 2021
Volume
68
Issue
3
Pages
178–179
Identifiers
DOI: 10.2344/anpr-68-03-01
PMID: 34606568
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Ring 18 syndrome or ring chromosome 18 is an extremely rare genetic disorder involving the fusion of the 18th chromosomal ends to form a ring, often with genetic material loss of varying degrees. Although clinical presentation can be extremely variable, characteristic features usually include craniofacial malformations, delayed development, hypotonia, and other skeletal and congenital heart defects. We report the management of a 20-year-old male with ring chromosome 18 who underwent general anesthesia for dental treatment. Clinical manifestations for this patient included intellectual disability, short stature, hypertelorism, flat nasal bridge, micrognathia, a "carp-shaped" mouth, and aortic and pulmonary valve regurgitation. Although mask ventilation and oral intubation were easily performed, nasal intubation was difficult because of rhinostenosis. When providing general anesthesia for a patient with ring chromosome 18, anesthesiologists should evaluate the patient preoperatively for congenital heart defects and prepare for a potential difficult airway. © 2021 by the American Dental Society of Anesthesiology.

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