González LAS, Díaz FLA, Asz SJ, Fernández SJR, Medina RE, Ramírez MK

Language: Spanish
References: 10
Page: 535-538
PDF size: 174.5 Kb.

ABSTRACT

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.

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