Anesthetic management of a child with Noonan syndrome
DOI:
https://doi.org/10.18004/Keywords:
Noonan syndrome, anesthesia, malignant hyperthermia, ParaguayAbstract
Noonan syndrome is a non-chromosomal syndrome with autosomal dominant heritance and sporadic occurrence. Affected children have short stature, chest deformity and usually cryptorchidism together with a structural cardiac abnormality. Pulmonary stenosis and hypertrophic obstructive cardiomyopathy are the most common cardiac anomalies found in combination with other anomalies which determines a special perioperative management of such patients. We describe the anesthetic management of a child with Noonan syndrome scheduled for extraction of several infected teeth under general anesthesia to eliminate sources of bacteremia, at the Service of Pediatric Cardiology of the School of Medicine of the Universidad Nacional de Asunción. The surgical procedure was done under general anesthesia with a favorable outcome.
References
1. Noonan J, O'Connor' W. Noonan syndrome: A clinical description emphasizing the cardiac findings. Acta Paediatr Jpn 1996;38:76-83.
2. Kobylinski 0. Ueber Eine Flughoutahnbiche Ausbreitung. Am Halse Arch Anthropol 1883;14:342-8.
3. Noonan JA, Ehmke DA. Associated noncardiac malformation in children with congenital heart disease. J Pediatr 1963;63:468-9.
4. Noonan JA. Noonan syndrome and related disorders. Prog Pediatr Cardiol 2005;20:177-185.
5. Mckenzie IM, Weintraub RG. Cardiomyopathies. In: Lake CL, Booker PD editors. Pediatric Cardiac Anaesthesia. 4 th Ed. Philadelphia: Lipincott Williams and Williams; 2005;Pg 530-6.
6. Pettersen MD, Du W, Skeens ME, Humes RA. Regression equations for calculation of z scores of cardiac structures in a large cohort of healthy infants, children, and adolescents: An echocardiographic study. J Am Soc Echocardiogr 2008;21:922-34.
7. Marik PE. Propofol: therapeutic indications and side-effects. Curr Pharm Des. 2004; 10:3639-49
8. Yu EH, Omran AS, Wigle ED, Williams WG, Siu SC, Rakowski H. Mitral Regurgitation in Hypertrophic Obstructive Cardiomyopathy: Relationship to obstruction and Relief with Myectomy. J Am Coll Cardiol 2000;36:2219-25.
9. Goens MB, Campbell D, Wiggins JW. Spontaneous chylothorax in Noonan syndrome. Treatment with prednisone. Am J Dis Child 1992;146:1453-6.
10. Carpenter D, Robinson RL, Quinnell RJ et al. – Genetic variation in RYR1 and malignant hyperthermia phenotypes. Br J Anaesth, 2009;1-
