Successful Anesthetic Management of an Adult with Sotos Syndrome

Abstract

Sotos syndrome is a rare genetic disorder presenting with craniofacial abnormalities, profound hypotonia, and cardiac abnormalities, giving rise to several potential challenges and concerns for an anesthesiologist. When preparing for a Sotos syndrome patient’s case, we consulted the literature for precedents on how to plan the anesthetic, to which we were only able to find a few reports and nothing in the age group our patient fell within. We present our case of an adult in addition to examining the previous cases so as to document a precedent when encountering patients with this syndrome in the operating room. We describe a unique case of a nonverbal adult with hypotonia and severe craniofacial abnormalities who successfully underwent multiple dental extractions under general anesthesia, with no complications other than a delay of emergence attenuated by naloxone. Our case and the seven previous documented cases over the past several decades demonstrate anesthesia, including paralytics and intubation itself safe despite obvious concerns given the common features of the syndrome for both pediatric patients and the one adult we described in this report.