Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2020.10.013
Brazilian Journal of Anesthesiology
Case Report

Anesthesia management of pediatric dentistry patients with cardiofaciocutaneous syndrome: a case report

Gian Luigi Gonnella, Pietro Paolo Giuri, Bruno Antonio Zanfini, Matteo Biancone, Luciano Frassanito, Cristina Olivieri, Gaetano Draisci

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Abstract

Cardiofaciocutaneous syndrome is a rare syndrome characterized by particular craniofacial features, cardiac abnormalities, and multiple organ diseases. Patients present with pulmonary stenosis, hypertrophic cardiomyopathy, short neck, micrognathia, laryngomalacia, and tracheomalacia. These conditions may strongly influence patient perioperative outcomes. We describe a 15-year-old child with cardiofaciocutaneous syndrome presenting for a dentistry procedure. She had an uneventful perioperative and postoperative course except for difficult airway management.

Keywords

Cardiofaciocutaneous syndrome;  Airway management;  Congenital heart defects

References

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2 CM Armour, JE Allanson Further delineation of cardiofaciocutaneous syndrome: clinical features of 38 individuals with proven mutations J Med Genet., 45 (2008), pp. 249-254

3 K. Katcher, M. Bothwell, J.D. Tobias Anaesthetic implications of Costello syndrome Paediatr Anaesth., 13 (2003), pp. 257-262

4 A.F. Goodwin, S. Oberoi, M. Landan, et al. Craniofacial and dental development in cardiofaciocutaneous syndrome: the importance of Ras signaling homeostasis Clin Genet., 83 (2013), pp. 539-544

5 W.E. Tidyman, S.L. Han, K.A. Rauen Skeletal muscle pathology in Costello and cardiofaciocutaneous syndromes: developmental consequences of germline Ras/MAPK activation on myogenesis Am J Med Genet C Semin in Med Genet., 157C (2011), pp. 104-114
 

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