Anesthesia management of pediatric dentistry patients with cardiofaciocutaneous syndrome: a case report
Cuidado antestésico em pacientes de odontopediatria com síndrome cardiofaciocutânea: relato de caso
Gian Luigi Gonnella, Pietro Paolo Giuri, Bruno Antonio Zanfini, Matteo Biancone, Luciano Frassanito, Cristina Olivieri, Gaetano Draisci
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
References
1 KA Rauen Cardiofaciocutaneous syndrome RA Pagon, MP Adam, HH Ardinger, TD Bird, C Dolan, CT Fong, RJH Smith, K Stephens, A Amemiya (Eds.), GeneReviews® [Internet], University of Washington, Seattle, Seattle, WA (2007), pp. 1993-2020 Jan 18 [Updated 2012 Sep 6].
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
Facebook
Google+
Twitter
LinkedIn
Mendeley
StumbleUpon
CiteULike
Reddit
Email