Considerations for Anesthesiologists Treating Patients with Cardiofaciocutaneous Syndrome

Anesthesiologists treating patients with cardiofaciocutaneous (CFC) syndrome may need to adjust care for this patient population, according to a study published in Anesthesia Progress.

CFC syndrome is a characterized by congenital heart disease, craniofacial dysmorphology, and dermatological abnormalities and is a part of the RASopathies family of disorders, which have underlying gene mutations involving the Ras/mitogen-activated protein kinase pathways.

When caring for patients with CFC syndrome, anesthesiologists need to:

-check for possible cardiac defects

-anticipate difficult airway management

-take into account potential respiratory muscle weakness

The use of desflurane and remifentanil may reduce the risk of respiratory complications in patients who experience respiratory muscle weakness following general anesthesia.

Reference

Shionoya Y, Yamamoto M, Sunada K, et al. Anesthetic management of a pediatric patient with cardiofaciocutaneous syndrome. Anesth Prog. 2020;67(1):45-47 Spring.