Anesthetic Management of a Boy with Congenital Disorder of Glycosylation (CDG) I-x


Congenital disorders of glycosylation (CDGs) are group group of genetic defects in the assembly and processing pathway of protein glycosylation, which cause a wide range of multi system dysfunction. This paper describes the anesthetic management of 6 year old boy with CDG type I-x for upper airway surgery. We used a sevoflurane-nitrous oxide-remifentanil regime with no complications and good results. As for now, the literature lacks reports of anesthetic management of children with CDG I-x, and this report may provide clinicians an option for safe anesthetic management.

Share and Cite:

A. Lehavi, H. Mandel and Y. Katz, "Anesthetic Management of a Boy with Congenital Disorder of Glycosylation (CDG) I-x," International Journal of Clinical Medicine, Vol. 2 No. 3, 2011, pp. 325-327. doi: 10.4236/ijcm.2011.23056.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] Collins AE, Ferriero DM. “The expanding spectrum of congenital disorders of glycosylation”, J Pediatr, Vol 147, 2005, pp 728-30.
[2] Leonard J, Grunewald S, Clayton P. “Diversity of congenital disorders of glycosylation”, Lancet, Vol 357, 2001 pp 1382-3.
[3] Engelhardt H, Staudt M, Hassler A et al. “Carbohydrate-deficient glycoprotein syndrome type 2”, J Inherit Metab Dis, Vol 22, 1999, pp 192-3.
[4] Marquardt T, Denecke J. “Congenital disorders of glycosylation: review of their molecular bases, clinical presentations and specific therapies”, Eur J Pediatr, Vol 162, 2003, pp 359-79.
[5] Kusa J, Pyrkosz A, Skiba A, Szkutnik M. “Cardiac manifestations of carbohydrate-deficient glycoprotein syndrome”, Pediatr Cardiol, Vol 24, 2003, pp 493-4.
[6] Meaudre E, Meyrieux V, Suprano I et al. “Anesthesia considerations in carbohydrate-deficient glycoprotein syndrome type I”, Paediatr Anaesth Vol 15, 2005, pp 905-6.
[7] Rama-Maceiras P, Ferreira TA, Molins N et al. “Less postoperative nausea and vomiting after propofol + remifentanil versus propofol + fentanyl anaesthesia during plastic surgery”, Acta Anaesthesiol Scand, Vol 49, 2005, pp 305-11.
[8] Loop T, Priebe HJ. “Recovery after anesthesia with remifentanil combined with propofol, desflurane, or sevoflurane for otorhinolaryngeal surgery”, Anesth Analg, Vol 91, 2000, pp 123-9.
[9] Thompson JP, Rowbotham DJ. “Remifentanil--an opioid for the 21st century”, Br J Anaesth Vol 76, 1996, pp 341-3.

Copyright © 2024 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.