The Journal of Korea Assosiation for Disability and Oral Health (대한장애인치과학회지)

Korean Association for Disability and Oral Health (대한장애인치과학회)
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DENTAL TREATMENT FOR A PATIENT WITH NOONAN SYNDROME UNDER GENERAL ANESTHESIA: CASE REPORT

누난 증후군(Noonan syndrome) 환자의 전신마취 하 치과치료 : 증례보고

  • Seo, Meekyung (Dept. of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Song, Ji-Soo (Dept. of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Shin, Teo Jeon (Dept. of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Hyun, Hong-Keun (Dept. of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Jung-Wook (Dept. of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Jang, Ki-Taeg (Dept. of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Lee, Sang-Hoon (Dept. of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Young-Jae (Dept. of Pediatric Dentistry, School of Dentistry, Seoul National University)
  • 서미경 (서울대학교 치의학대학원 소아치과학교실) ;
  • 송지수 (서울대학교 치의학대학원 소아치과학교실) ;
  • 신터전 (서울대학교 치의학대학원 소아치과학교실) ;
  • 현홍근 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김정욱 (서울대학교 치의학대학원 소아치과학교실) ;
  • 장기택 (서울대학교 치의학대학원 소아치과학교실) ;
  • 이상훈 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김영재 (서울대학교 치의학대학원 소아치과학교실)
  • Received : 2017.06.01
  • Accepted : 2017.06.21
  • Published : 2017.06.30
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Abstract

Noonan syndrome is a relatively common genetic disorder which is autosomal dominant. Prevalence of Noonan syndrome is varying from 0.04% to 0.1%. It is characterized by distinctive facial features, chest deformity, short stature and congenital heart disease. Oral findings in patient with Noonan syndrome include high arched palate, dental malocclusion, articulation difficulties, and micrognathia. The purpose of this case report is to describe dental treatment of a children with Noonan syndrome. 5 year old boy with Noonan syndrome visited to the Seoul National University Dental Hospital for dental treatment. Due to need for close monitoring, concern about seizure and poor cooperation, we planned to perform the dental treatment under general anesthesia. Under general anesthesia, caries treatment was successfully performed and there was no postoperative complications related to general anesthesia. High arched palate was observed which is characteristic in Noonan syndrome.

본 증례는 유구치부의 치아우식증을 주소로 내원한 누난 증후군 환자의 전신마취 하 치과치료에 대한 보고이다. 누난 증후군 환자의 특징적인 안모가 관찰되었으며, 높은 아치형의 구개와 작은 턱이 관찰되었다. 성장 지연, 발달 지연, 가슴 기형, 언어 지연을 보였다. 누난 증후군 환자는 다양한 전신질환을 동반할 수 있으므로, 치과 치료 시 전신적인 상태에 대한 고려가 필요하며 이와 더불어 행동 문제를 보이는 경우가 많아 행동 조절에 대해 고려해야 한다. 누난 증후군 환자의 전신마취 시 기도 문제, 심폐 기능의 손상 등이 문제가 될 수 있으므로, 이에 대한 평가가 필요하다.

Keywords

References

  1. Allanson JE : Noonan Syndrome. Journal of Medical Genetics, 24:9, 1987. https://doi.org/10.1136/jmg.24.1.9
  2. Verhoeven W, Wingbermuhle E, Egger J, et al. : Noonan Syndrome: Psychological and Psychiatric Aspects. Am J Med Genet A, 146:191-196, 2008.
  3. Noonan JA : Noonan Syndrome an Update and Review for the Primary Pediatrician. Clin Pediatr (Phila), 33:548-555, 1994. https://doi.org/10.1177/000992289403300907
  4. Van der Burgt I : Noonan Syndrome. Orphanet J Rare Dis, 2:4, 2007. https://doi.org/10.1186/1750-1172-2-4
  5. Van Der Burgt I, Berends E, Lommen E, et al. : Clinical and Molecular Studies in a Large Dutch Family with Noonan Syndrome. Am J Med Genet, 53:187-191, 1994. https://doi.org/10.1002/ajmg.1320530213
  6. Romano AA, Allanson JE, Dahlgren J, et al. : Noonan Syndrome: Clinical Features, Diagnosis, and Management Guidelines. Pediatrics, 126:746-759, 2010. https://doi.org/10.1542/peds.2009-3207
  7. Tartaglia M, Zampino G, Gelb B : Noonan Syndrome: Clinical Aspects and Molecular Pathogenesis. Mol Syndromol, 1:2-26, 2010. https://doi.org/10.1159/000276766
  8. Roberts AE, Allanson JE, Tartaglia M, et al. : Noonan Syndrome. Lancet, 381:333-342, 2013. https://doi.org/10.1016/S0140-6736(12)61023-X
  9. Schubbert S, Zenker M, Rowe SL, et al. : Germline Kras Mutations Cause Noonan Syndrome. Nat Genet, 38:331-336, 2006. https://doi.org/10.1038/ng1748
  10. Tartaglia M, Mehler EL, Goldberg R, et al. : Mutations in Ptpn11, Encoding the Protein Tyrosine Phosphatase Shp-2, Cause Noonan Syndrome. Nat Genet, 29:465, 2001. https://doi.org/10.1038/ng772
  11. Zenker M, Buheitel G, Rauch R, et al. : Genotype-Phenotype Correlations in Noonan Syndrome. J Pediatr, 144:368-374, 2004. https://doi.org/10.1016/j.jpeds.2003.11.032
  12. Tartaglia M, Gelb BD, and Zenker M : Noonan Syndrome and Clinically Related Disorders. Best Pract Res Clin Endocrinol Metab, 25:161-179, 2011. https://doi.org/10.1016/j.beem.2010.09.002
  13. Sharland M, Burch M, McKenna W, et al. : A Clinical Study of Noonan Syndrome. Arch Dis Child, 67:178-183, 1992. https://doi.org/10.1136/adc.67.2.178
  14. 2007년 소아 청소년 표준 성장도표(해설). Available from URL: http://cdc.go.kr/CDC/notice/CdcKrInfo0201.jsp?menuIds=HOME001-MNU1154-MNU0005-MNU1889&cid=1235 (Assessed on May 29, 2017).
  15. Horowitz SL, Morishima A : Palatal Abnormalities in the Syndrome of Gonadal Dysgenesis and Its Variants and in Noonan's Syndrome. Oral Surg Oral Med Oral Pathol, 38:839-844, 1974. https://doi.org/10.1016/0030-4220(74)90334-X
  16. Lee DA, Portnoy S, Hill P, et al. : Psychological Profile of Children with Noonan Syndrome. Dev Med Child Neurol, 47:35-38, 2005.
  17. van der Burgt I, Thoonen G, Roosenboom N, et al. : Patterns of Cognitive Functioning in School-Aged Children with Noonan Syndrome Associated with Variability in Phenotypic Expression. J Pediatr, 135:707-713, 1999. https://doi.org/10.1016/S0022-3476(99)70089-2
  18. Schwartz N, Eisenkraft JB : Anesthetic Management of a Child with Noonan's Syndrome and Idiopathic Hypertrophic Subaortic Stenosis. Anesth Analg, 74:464-466, 1992.