Journal of The Korean Dental Society of Anesthesiology (대한치과마취과학회지)

The Korean Dental Society of Anesthsiology (대한치과마취과학회)
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Ambulatory Anesthesia Management of the Patient with Adrenoleukodystrophy for Dental Treatment -A Case Report-

부신백질이영양증 환자의 치과치료를 위한 외래전신마취 -증례 보고-

  • Seo, Kwang-Suk (Department of Dental Anesthesiology, Seoul National University, School of Dentistry) ;
  • Bak, So-Yeon (Department of Pediatric Dentistry, Seoul National University, School of Dentistry) ;
  • Shin, Teo-Jeon (Department of Dental Anesthesiology, Seoul National University, School of Dentistry) ;
  • Kim, Hyun-Jeong (Department of Dental Anesthesiology, Seoul National University, School of Dentistry)
  • 서광석 (서울대학교 치의학대학원 치과마취과학교실) ;
  • 박소연 (서울대학교 치의학대학원 소아치과학교실) ;
  • 신터전 (서울대학교 치의학대학원 치과마취과학교실) ;
  • 김현정 (서울대학교 치의학대학원 치과마취과학교실)
  • Received : 2010.06.08
  • Accepted : 2010.06.20
  • Published : 2010.06.27
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Abstract

Adrenoleukodystrophy (ALD) is a rare X-linked genetic disorder associated with various central nervous system problems and adrenal insufficiency. It is common in childhood and symptoms include loss of previously acquired neurological abilities, seizures, ataxia, Addison's disease as well as degeneration of visual and auditory function. These children frequently require anesthesia during imagining procedure such as MRI or during some surgical procedures like gastrostomy. There is special need for careful management because of numerous anesthetic challenges like difficulty in cooperation, seizure disorders, life-threatening airway obstruction, copious oral secretion and possibility of aspiration. In addition, adrenal involvement and hypofunction must be considered for safe anesthesia management. We report a successful anesthetic management in a patient with ALD for dental procedures.

Keywords

References

  1. Dobson G, Lyons J: Anaesthesia for a life-limited child with adrenoleucodystrophy. Eur J Anaesthesiol 2004; 21(1): 78-9.
  2. Hamdiye CT, Yavuz G, Kamil T, Mine S: Anesthesiamanagement of a child with adrenoleukodystrophy. Paediatr Anaesth 2006; 16(2): 221-2. https://doi.org/10.1111/j.1460-9592.2005.01781.x
  3. Kindopp AS, Ashbury T: Anaesthetic management of an adult patient with X-linked adrenoleukodystrophy. Can J Anaesth 1998; 45(10): 990-2. https://doi.org/10.1007/BF03012307
  4. Krivit W, Peters C, Shapiro EG: Bone marrow transplantation as effective treatment of central nervous system disease in globoid cell leukodystrophy, metachromatic leukodystrophy, adrenoleukodystrophy, mannosidosis, fucosidosis, aspartylglucosaminuria, Hurler, Maroteaux-Lamy, and Sly syndromes, and Gaucher disease type III. Curr Opin Neurol 1999; 12(2): 167-76. https://doi.org/10.1097/00019052-199904000-00007
  5. Moser H, Dubey P, Fatemi A: Progress in X-linked adrenoleukodystrophy. Curr Opin Neurol 2004a; 17(3): 263-9. https://doi.org/10.1097/00019052-200406000-00005
  6. Moser HW, Fatemi A, Zackowski K, Smith S, Golay X, Muenz L, et al. Evaluation of therapy of X-linked adrenoleukodystrophy. Neurochem Res 2004b; 29(5): 1003-16. https://doi.org/10.1023/B:NERE.0000021245.12181.90
  7. Rizzo WB, Leshner RT, Odone A, Dammann AL, Craft DA, Jensen ME, et al. Dietary erucic acid therapy for X-linked adrenoleukodystrophy. Neurology 1989; 39(11): 1415-22. https://doi.org/10.1212/WNL.39.11.1415
  8. Schwartz RE, Stayer SA, Pasquariello CA, Lowe DA, Foster CJ. Anaesthesia for the patient with neonatal adrenoleukodystrophy. Can J Anaesth 1994; 41(1): 56-8. https://doi.org/10.1007/BF03009663
  9. van Geel BM, Assies J, Haverkort EB, Koelman JH, Verbeeten B, Jr., Wanders RJ, et al. Progression of abnormalities in adrenomyeloneuropathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with "Lorenzo's oil". J Neurol Neurosurg Psychiatry 1999; 67(3): 290-9. https://doi.org/10.1136/jnnp.67.3.290