Abstract

GONZALEZ LUMBRERAS, Aniza Surinam et al. Perioperative considerations in an infant with Noonan syndrome. Acta méd. Grupo Ángeles [online]. 2021, vol.19, n.4, pp.535-538.  Epub Aug 01, 2022. ISSN 1870-7203.  https://doi.org/10.35366/102541.

Noonan syndrome is a congenital disease belonging to the rasopathy family, caused by mutations in the RAS-MAPK pathway. It is a multisystemic syndrome commonly associated with structural heart disease, coagulopathies, cryptorchidism, cerebrovascular anomalies, and cognitive impairment, as well as renal and lymphoid alterations. Patients usually show typical facial anomalies and a difficult airway, which might portend a challenge for the anesthesiologist. We present the case of an infant with Noonan syndrome and bilateral cryptorchidism, treated with laparoscopic orchidopexy under general anesthesia. Anesthesiology approach was selected based on the history of congenital heart disease, difficult airway, and difficult vascular access secondary to peripheral lymphedema.

Keywords : Noonan syndrome; general anesthesia; difficult airway; congenital heart disease.